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Source book
PREGNANCY AND CARDIOVASCULAR DISEASE: Risk Assessment, Management, and Prevention
Editors: Prof. Dr. Ali ACAR · Assoc. Prof. Dr. Jule ERIC HORASANLI Associate editor: Dr. Beyza GÜZELKARA ERGENE

Cardiovascular Disease in Pregnancy

Clinical decision algorithm

⚕️ Information: This algorithm is for healthcare professionals only. It is a reference for clinical decision support; It does not replace the clinician's patient-specific assessment.

1Disease
2Second criterion
3Third criterion

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Congenital heart diseases

Atrial septal defect (ASD)
Repair status and pulmonary pressure
Successfully repaired, no pulmonary hypertension

Approach

  1. Use mWHO I classification for a successfully repaired simple septal defect without significant residual lesion, ventricular dysfunction, or clinically significant arrhythmia; If there is one of these findings, reclassify the risk and make an expert assessment. Explain that pregnancy is generally safe.
  2. Perform routine obstetric care; Cardiology evaluation 1-2 times during pregnancy may be sufficient depending on the symptoms.
  3. Keep in mind during follow-up that arrhythmia may develop after septal surgery. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  4. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  5. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  6. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • No specific drug treatment is required.

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
Unrepaired, no pulmonary hypertension

Approach

  1. Risk class: mWHO II (unrepaired ASD); Pregnancy is generally well tolerated, give individual counselling.
  2. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy. Get a cardiology evaluation at least once each trimester. Plan pre-pregnancy closure of large or hemodynamically significant ASD at your adult congenital heart disease center. Consider the risk of paradoxical embolism (2–5%) and arrhythmia (4%) in unrepaired ASD.
  3. Evaluate right heart dimensions and pulmonary pressure by ECHO; Approximately 10% of unrepaired ASDs develop pulmonary hypertension.
  4. Consider that symptoms previously associated only with exertion may occur at rest and tachyarrhythmias may increase due to increased pulmonary flow; Functional deterioration is more common in the third trimester. If there is right atrial enlargement, monitor for atrial arrhythmia. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  5. In case of signs of heart failure (increased jugular venous pressure, rales, resting dyspnea, orthopnea, marked edema, hepatomegaly, desaturation), perform urgent cardiology evaluation and refer to the Pregnancy Heart Team.
  6. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely. If expert counseling and referral to a higher center are provided, birth can take place at the local hospital.
  8. Initiate contraception counseling before need arises; avoid combined oral contraceptives if heart chambers are enlarged or atrial fibrillation; The intrauterine device is an excellent option.

Treatment

  • If symptoms or tachyarrhythmia develop at rest, for rate control in the hemodynamically stable patient — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If diuresis is required in the symptomatic patient during the early puerperal period — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • By individual decision if paradoxical embolism prevention is deemed necessary in unrepaired ASD — Aspirin (low dose): 75–100 mg orally once daily.
  • By individual decision instead of aspirin for paradoxical embolism prevention in unrepaired ASD — Enoxaparin (prophylactic): 40 mg SC once daily under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).

Note

  • Avoid atenolol in your choice of β-blocker.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
Significant pulmonary arterial hypertension or Eisenmenger syndrome

Approach

  1. Risk class: mWHO IV (significant pulmonary arterial hypertension of any cause or Eisenmenger syndrome); pregnancy is contraindicated. If pulmonary hypertension is non-PAH or of uncertain type, do not assign mWHO IV based solely on elevated pressure; determine risk with an expert team according to the underlying disease, ventricular function, and functional status.
  2. Recommend avoiding pregnancy if pregnancy is planned; individualize counseling at a specialist Pregnancy Heart Team or pulmonary hypertension centre; Record the decision, its rationale, and the patient's preferences.
  3. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the option of terminating the pregnancy if it is legally and ethically appropriate. If pregnancy continues, centralize care at a specialist center with experience in pulmonary hypertension, right heart failure, targeted pulmonary vasodilator therapy, intensive care and mechanical circulatory support.
  5. Plan close monitoring during late pregnancy, birth, and early puerperium when risk is highest. Do not delay hospitalization in Eisenmenger syndrome; Late diagnosis and delayed hospitalization significantly increase maternal mortality.
  6. Record basal oxygen saturation; If pulmonary pressure decreases and saturation increases with oxygen, the resistance is not constant and the prognosis is better. Be aware that the physiological decrease in systemic vascular resistance during pregnancy may initiate right-to-left shunting in a patient without previous cyanosis.
  7. If Eisenmenger syndrome is present, monitor for heart failure, hemoptysis, sudden arrhythmic death, cerebrovascular accident and hyperviscosity syndrome. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  8. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  9. Plan the mode and time of birth in a timely manner with the Pregnancy Heart Team attended by a pulmonary hypertension specialist; Cesarean section may be preferred to vaginal birth as it provides a more controlled birth. Regional anesthesia is generally preferred to general anesthesia. In cases of progressive heart failure refractory to treatment and to reduce the risk of unplanned birth outside a specialist center, plan for premature birth by monitoring fetal growth.
  10. Create the birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  11. Perform meticulous fluid balance monitoring with ECG, pulse oximetry, cardiac output, and central venous pressure during and after birth in the intensive care unit; Most maternal deaths occur in the postpartum period, the risk persists for months.
  12. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • Apply activity restriction in symptomatic PAH or Eisenmenger syndrome; give bed rest if necessary; Plan thromboprophylaxis together for long periods of immobility.
  • In Eisenmenger syndrome — Oxygen: maintain supplemental oxygen if indicated; adjust flow based on saturation and symptom response; Give supplemental oxygen during labor.
  • If hypoxemia is present in non-Eisenmenger significant PAH — Oxygen: SpO₂ is maintained at least 90%, preferably 95% and above.
  • If targeted pulmonary vasodilator therapy is required — Sildenafil (oral): 20 mg 3 times daily; If it cannot be taken orally, 10 mg IV bolus 3 times a day.
  • Significant right ventricular dysfunction, progressive disease in functional class III, class IV symptoms, or clinical worsening despite oral therapy without delay in a specialized center — Epoprostenol (IV): start at 1–2 ng/kg/min by continuous infusion through a central venous catheter into a separate lumen with no other medication administered; titrate in increments of 1–2 ng/kg/min at intervals sufficient to assess clinical response (as little as 15 minutes, up to 2–4 weeks); It is not stopped suddenly, avoiding infusion interruption and large sudden dose reduction. Option if parenteral therapy cannot be applied: inhaled iloprost, 2.5 µg per dose, 5 µg if tolerated, 6–9 times a day (at least 2 hours apart), up to 45 µg daily.
  • If acute pulmonary vasodilator required — Inhaled nitric oxide: 20 ppm (range 5–20 ppm); No additional vasodilator response is expected between 20 and 40 ppm; not stopped suddenly: the dose is reduced in several steps, waiting several hours at each step and monitoring hypoxemia; It is usually safe to cut down to 10 ppm (temporary FiO2 increase may be required); If oxygenation or hemodynamics are impaired, it is reduced to 5 or 1 ppm before discontinuation.
  • Mechanical circulatory support (including ECMO): perform in specialist center if necessary.
  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy. Discontinue endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat, and selexipag due to possible teratogenicity; do not use during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • In women with severe PAH or complications during pregnancy/birth, optimization of PAH treatment should be prioritized over breastfeeding.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • In Eisenmenger syndrome, SpO₂ below 95% alone is not an indication for noninvasive or invasive ventilation; The decision on ventilation is made by the intensive care and pulmonary hypertension team.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Sildenafil (oral): Not for use with organic nitrates (continuous or intermittent) and riociguat; Sudden discontinuation may worsen PAH.
  • Sildenafil (oral): The risk of premature birth and small for gestational age (SGA) has been reported during pregnancy.
  • Epoprostenol (IV): Sudden discontinuation or large abrupt dose reduction may cause rebound pulmonary hypertension; Spare pump, infusion set and medication are kept ready. Prostanoids may disrupt platelet aggregation and increase bleeding.
  • Inhaled nitric oxide: Sudden discontinuation may cause rebound pulmonary hypertension and hemodynamic collapse even after short-term use; In this case, it starts over immediately. The risk of methemoglobinemia increases at doses above 40 ppm; Methemoglobin is measured 4–8 hours after the start and periodically, and inspired NO2 is monitored.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Ventricular septal defect (VSD)
Repair status and pulmonary pressure
Successfully repaired, no pulmonary hypertension

Approach

  1. Use mWHO I classification for a successfully repaired simple septal defect without significant residual lesion, ventricular dysfunction, or clinically significant arrhythmia; If there is one of these findings, reclassify the risk and make an expert assessment. Explain that pregnancy is generally safe.
  2. Perform routine obstetric care; Cardiology evaluation 1-2 times during pregnancy may be sufficient depending on the symptoms.
  3. Keep in mind during follow-up that arrhythmia may develop after septal surgery. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  4. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  5. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  6. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • No specific drug treatment is required.

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
Unrepaired, no pulmonary hypertension

Approach

  1. Risk class: mWHO II (unrepaired VSD); Pregnancy is generally well tolerated, give individual counselling. VSD rarely complicates pregnancy if there is no accompanying lesion or pulmonary hypertension; The small defect is hemodynamically insignificant but carries the risk of endocarditis.
  2. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy. Get a cardiology evaluation at least once each trimester.
  3. Evaluate the defect, accompanying lesions, and pulmonary pressure by ECHO; high-velocity interventricular jet suggests large pressure difference and low right ventricular pressure; Calculate right ventricular systolic pressure along with systemic blood pressure and confirm pulmonary hypertension with other ECHO findings.
  4. Consider that symptoms previously only associated with exertion may occur at rest and tachyarrhythmias may increase due to increased pulmonary flow. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  5. In case of signs of heart failure (increased jugular venous pressure, rales, resting dyspnea, orthopnea, marked edema, hepatomegaly, desaturation), perform urgent cardiology evaluation and refer to the Pregnancy Heart Team.
  6. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely. If expert counseling and referral to a higher center are provided, birth can take place at the local hospital.
  8. Initiate contraception counseling before need arises; avoid combined oral contraceptives if heart chambers are enlarged or atrial fibrillation; The intrauterine device is an excellent option.

Treatment

  • If symptoms or tachyarrhythmia develop at rest, for rate control in the hemodynamically stable patient — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If diuresis is required in the symptomatic patient during the early puerperal period — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).

Note

  • Avoid atenolol in your choice of β-blocker.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Significant pulmonary arterial hypertension or Eisenmenger syndrome

Approach

  1. Risk class: mWHO IV (significant pulmonary arterial hypertension of any cause or Eisenmenger syndrome); pregnancy is contraindicated. If pulmonary hypertension is non-PAH or of uncertain type, do not assign mWHO IV based solely on elevated pressure; determine risk with an expert team according to the underlying disease, ventricular function, and functional status.
  2. Recommend avoiding pregnancy if pregnancy is planned; individualize counseling at a specialist Pregnancy Heart Team or pulmonary hypertension centre; Record the decision, its rationale, and the patient's preferences.
  3. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the option of terminating the pregnancy if it is legally and ethically appropriate. If pregnancy continues, centralize care at a specialist center with experience in pulmonary hypertension, right heart failure, targeted pulmonary vasodilator therapy, intensive care and mechanical circulatory support.
  5. Plan close monitoring during late pregnancy, birth, and early puerperium when risk is highest. Do not delay hospitalization in Eisenmenger syndrome; Late diagnosis and delayed hospitalization significantly increase maternal mortality.
  6. Record basal oxygen saturation; If pulmonary pressure decreases and saturation increases with oxygen, the resistance is not constant and the prognosis is better. Be aware that the physiological decrease in systemic vascular resistance during pregnancy may initiate right-to-left shunting in a patient without previous cyanosis.
  7. If Eisenmenger syndrome is present, monitor for heart failure, hemoptysis, sudden arrhythmic death, cerebrovascular accident and hyperviscosity syndrome. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  8. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  9. Plan the mode and time of birth in a timely manner with the Pregnancy Heart Team attended by a pulmonary hypertension specialist; Cesarean section may be preferred to vaginal birth as it provides a more controlled birth. Regional anesthesia is generally preferred to general anesthesia. In cases of progressive heart failure refractory to treatment and to reduce the risk of unplanned birth outside a specialist center, plan for premature birth by monitoring fetal growth.
  10. Create the birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  11. Perform meticulous fluid balance monitoring with ECG, pulse oximetry, cardiac output, and central venous pressure during and after birth in the intensive care unit; Most maternal deaths occur in the postpartum period, the risk persists for months.
  12. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • Apply activity restriction in symptomatic PAH or Eisenmenger syndrome; give bed rest if necessary; Plan thromboprophylaxis together for long periods of immobility.
  • In Eisenmenger syndrome — Oxygen: maintain supplemental oxygen if indicated; adjust flow based on saturation and symptom response; Give supplemental oxygen during labor.
  • If hypoxemia is present in non-Eisenmenger significant PAH — Oxygen: SpO₂ is maintained at least 90%, preferably 95% and above.
  • If targeted pulmonary vasodilator therapy is required — Sildenafil (oral): 20 mg 3 times daily; If it cannot be taken orally, 10 mg IV bolus 3 times a day.
  • Significant right ventricular dysfunction, progressive disease in functional class III, class IV symptoms, or clinical worsening despite oral therapy without delay in a specialized center — Epoprostenol (IV): start at 1–2 ng/kg/min by continuous infusion through a central venous catheter into a separate lumen with no other medication administered; titrate in increments of 1–2 ng/kg/min at intervals sufficient to assess clinical response (as little as 15 minutes, up to 2–4 weeks); It is not stopped suddenly, avoiding infusion interruption and large sudden dose reduction. Option if parenteral therapy cannot be applied: inhaled iloprost, 2.5 µg per dose, 5 µg if tolerated, 6–9 times a day (at least 2 hours apart), up to 45 µg daily.
  • If acute pulmonary vasodilator required — Inhaled nitric oxide: 20 ppm (range 5–20 ppm); No additional vasodilator response is expected between 20 and 40 ppm; not stopped suddenly: the dose is reduced in several steps, waiting several hours at each step and monitoring hypoxemia; It is usually safe to cut down to 10 ppm (temporary FiO2 increase may be required); If oxygenation or hemodynamics are impaired, it is reduced to 5 or 1 ppm before discontinuation.
  • Mechanical circulatory support (including ECMO): perform in specialist center if necessary.
  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy. Discontinue endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat, and selexipag due to possible teratogenicity; do not use during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • In women with severe PAH or complications during pregnancy/birth, optimization of PAH treatment should be prioritized over breastfeeding.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • In Eisenmenger syndrome, SpO₂ below 95% alone is not an indication for noninvasive or invasive ventilation; The decision on ventilation is made by the intensive care and pulmonary hypertension team.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Sildenafil (oral): Not for use with organic nitrates (continuous or intermittent) and riociguat; Sudden discontinuation may worsen PAH.
  • Sildenafil (oral): The risk of premature birth and small for gestational age (SGA) has been reported during pregnancy.
  • Epoprostenol (IV): Sudden discontinuation or large abrupt dose reduction may cause rebound pulmonary hypertension; Spare pump, infusion set and medication are kept ready. Prostanoids may disrupt platelet aggregation and increase bleeding.
  • Inhaled nitric oxide: Sudden discontinuation may cause rebound pulmonary hypertension and hemodynamic collapse even after short-term use; In this case, it starts over immediately. The risk of methemoglobinemia increases at doses above 40 ppm; Methemoglobin is measured 4–8 hours after the start and periodically, and inspired NO2 is monitored.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Patent ductus arteriosus (PDA)
Duct and pulmonary vascular disease
Small or obstructed ductus; No Eisenmenger physiology

Approach

  1. Risk class: Use mWHO I only for a small or successfully closed PDA without pulmonary hypertension, left ventricular dysfunction, or a significant residual shunt; if any of these are present, reclassify the risk and perform a specialist evaluation. There is no detectable increase in maternal mortality, and morbidity increase is absent or mild.
  2. Perform routine obstetric care; Cardiology evaluation 1-2 times during pregnancy may be sufficient depending on the symptoms.
  3. Explain that if there is no Eisenmenger physiology, the pregnancy usually proceeds without any problems; increased pulmonary flow may cause shortness of breath. Keep in mind that the main risk in the small ductus is endocarditis.
  4. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  5. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  6. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  7. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • No specific drug treatment is required.

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
Small or non-sealed PDA; no significant pulmonary arterial hypertension and Eisenmenger physiology

Approach

  1. Evaluate the hemodynamic effect of the shunt and ventricular function by ECHO; Individualize the risk with an expert team.
  2. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  3. Explain that if there is no Eisenmenger physiology, the pregnancy usually proceeds without any problems. Monitor for dyspnea due to increased pulmonary flow.
  4. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  5. In case of signs of heart failure (increased jugular venous pressure, rales, resting dyspnea, orthopnea, marked edema, hepatomegaly, desaturation), perform urgent cardiology evaluation and refer to the Pregnancy Heart Team.
  6. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  8. Initiate contraception counseling before need arises; avoid combined oral contraceptives if heart chambers are enlarged or atrial fibrillation; The intrauterine device is an excellent option.

Treatment

  • No specific drug treatment is required in a patient who is asymptomatic, has preserved ventricular function, and has no congestion.
  • If pulmonary congestion or volume overload develops — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Significant pulmonary arterial hypertension or Eisenmenger syndrome

Approach

  1. Risk class: mWHO IV (significant pulmonary arterial hypertension of any cause or Eisenmenger syndrome); pregnancy is contraindicated. If pulmonary hypertension is non-PAH or of uncertain type, do not assign mWHO IV based solely on elevated pressure; determine risk with an expert team according to the underlying disease, ventricular function, and functional status.
  2. Recommend avoiding pregnancy if pregnancy is planned; individualize counseling at a specialist Pregnancy Heart Team or pulmonary hypertension centre; Record the decision, its rationale, and the patient's preferences.
  3. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the option of terminating the pregnancy if it is legally and ethically appropriate. If pregnancy continues, centralize care at a specialist center with experience in pulmonary hypertension, right heart failure, targeted pulmonary vasodilator therapy, intensive care and mechanical circulatory support.
  5. Plan close monitoring during late pregnancy, birth, and early puerperium when risk is highest. Do not delay hospitalization in Eisenmenger syndrome; Late diagnosis and delayed hospitalization significantly increase maternal mortality.
  6. Record basal oxygen saturation; If pulmonary pressure decreases and saturation increases with oxygen, the resistance is not constant and the prognosis is better. Be aware that the physiological decrease in systemic vascular resistance during pregnancy may initiate right-to-left shunting in a patient without previous cyanosis.
  7. If Eisenmenger syndrome is present, monitor for heart failure, hemoptysis, sudden arrhythmic death, cerebrovascular accident and hyperviscosity syndrome. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  8. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  9. Plan the mode and time of birth in a timely manner with the Pregnancy Heart Team attended by a pulmonary hypertension specialist; Cesarean section may be preferred to vaginal birth as it provides a more controlled birth. Regional anesthesia is generally preferred to general anesthesia. In cases of progressive heart failure refractory to treatment and to reduce the risk of unplanned birth outside a specialist center, plan for premature birth by monitoring fetal growth.
  10. Create the birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  11. Perform meticulous fluid balance monitoring with ECG, pulse oximetry, cardiac output, and central venous pressure during and after birth in the intensive care unit; Most maternal deaths occur in the postpartum period, the risk persists for months.
  12. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • Apply activity restriction in symptomatic PAH or Eisenmenger syndrome; give bed rest if necessary; Plan thromboprophylaxis together for long periods of immobility.
  • In Eisenmenger syndrome — Oxygen: maintain supplemental oxygen if indicated; adjust flow based on saturation and symptom response; Give supplemental oxygen during labor.
  • If hypoxemia is present in non-Eisenmenger significant PAH — Oxygen: SpO₂ is maintained at least 90%, preferably 95% and above.
  • If targeted pulmonary vasodilator therapy is required — Sildenafil (oral): 20 mg 3 times daily; If it cannot be taken orally, 10 mg IV bolus 3 times a day.
  • Significant right ventricular dysfunction, progressive disease in functional class III, class IV symptoms, or clinical worsening despite oral therapy without delay in a specialized center — Epoprostenol (IV): start at 1–2 ng/kg/min by continuous infusion through a central venous catheter into a separate lumen with no other medication administered; titrate in increments of 1–2 ng/kg/min at intervals sufficient to assess clinical response (as little as 15 minutes, up to 2–4 weeks); It is not stopped suddenly, avoiding infusion interruption and large sudden dose reduction. Option if parenteral therapy cannot be applied: inhaled iloprost, 2.5 µg per dose, 5 µg if tolerated, 6–9 times a day (at least 2 hours apart), up to 45 µg daily.
  • If acute pulmonary vasodilator required — Inhaled nitric oxide: 20 ppm (range 5–20 ppm); No additional vasodilator response is expected between 20 and 40 ppm; not stopped suddenly: the dose is reduced in several steps, waiting several hours at each step and monitoring hypoxemia; It is usually safe to cut down to 10 ppm (temporary FiO2 increase may be required); If oxygenation or hemodynamics are impaired, it is reduced to 5 or 1 ppm before discontinuation.
  • Mechanical circulatory support (including ECMO): perform in specialist center if necessary.
  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy. Discontinue endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat, and selexipag due to possible teratogenicity; do not use during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • In women with severe PAH or complications during pregnancy/birth, optimization of PAH treatment should be prioritized over breastfeeding.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • In Eisenmenger syndrome, SpO₂ below 95% alone is not an indication for noninvasive or invasive ventilation; The decision on ventilation is made by the intensive care and pulmonary hypertension team.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Sildenafil (oral): Not for use with organic nitrates (continuous or intermittent) and riociguat; Sudden discontinuation may worsen PAH.
  • Sildenafil (oral): The risk of premature birth and small for gestational age (SGA) has been reported during pregnancy.
  • Epoprostenol (IV): Sudden discontinuation or large abrupt dose reduction may cause rebound pulmonary hypertension; Spare pump, infusion set and medication are kept ready. Prostanoids may disrupt platelet aggregation and increase bleeding.
  • Inhaled nitric oxide: Sudden discontinuation may cause rebound pulmonary hypertension and hemodynamic collapse even after short-term use; In this case, it starts over immediately. The risk of methemoglobinemia increases at doses above 40 ppm; Methemoglobin is measured 4–8 hours after the start and periodically, and inspired NO2 is monitored.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Tetralogy of Fallot
Repair status and residual lesion
Repaired, with good ventricular function; no right ventricular dilatation and no significant residual lesion

Approach

  1. Risk class: mWHO II (well-functioning repaired tetralogy of Fallot); Pregnancy is generally well tolerated, give individual counselling. Use the ZAHARA score in addition to the mWHO class to estimate risk; Cardiac drug use before pregnancy predicts adverse cardiac events.
  2. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy. Get a cardiology evaluation at least once each trimester.
  3. Evaluate the size and function of both ventricles and the severity of pulmonary insufficiency or stenosis with ECHO before pregnancy.
  4. Provide genetic counseling; it is particularly valuable in conotruncal anomalies (22q11.2 deletion is inherited in an autosomal dominant manner). Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  5. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  6. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely. If expert counseling and referral to a higher center are provided, birth can take place at the local hospital.
  7. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • No specific drug treatment is required.

Note

  • Before measuring blood pressure, ask the patient if an arm is unreliable or look for a thoracotomy scar; After the classic Blalock–Thomas–Taussig shunt, the pressure in that arm may not reflect the aortic pressure.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
Repaired, severe pulmonary insufficiency or stenosis or right ventricular dilatation/dysfunction

Approach

  1. Obtain expert evaluation before pregnancy; Use mWHO, CARPREG II and ZAHARA scores together in risk classification. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  2. Explain that severe pulmonary insufficiency and right ventricular dysfunction predict cardiac complications (supraventricular tachycardia, heart failure, thromboembolism) during pregnancy, and right heart dilatation is a sign of a complicated course. Monitor for right heart failure and atrial and ventricular tachyarrhythmias during pregnancy. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  3. Evaluate the size and function of both ventricles and the severity of pulmonary insufficiency or stenosis with ECHO before pregnancy.
  4. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities.
  5. Provide genetic counseling; it is particularly valuable in conotruncal anomalies (22q11.2 deletion is inherited in an autosomal dominant manner). Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  6. Prefer vaginal birth; Consider cesarean section in severe right ventricular outflow tract stenosis or severe pulmonary valve stenosis; Caesarean section is preferred in acute refractory heart failure.
  7. Initiate contraception counseling before need arises; avoid combined oral contraceptives if heart chambers are enlarged or atrial fibrillation; The intrauterine device is an excellent option.

Treatment

  • Severe pulmonary insufficiency repair: evaluate before pregnancy if pregnancy is planned.
  • Consider intervention before pregnancy in cases of severe right ventricular outflow tract stenosis (Doppler peak gradient >64 mmHg) or evidence of right heart failure.
  • Consider transcatheter balloon valvotomy for severe symptomatic pulmonary valve stenosis that does not respond to bed rest and conservative treatment during pregnancy.
  • Bed rest and diuretics in right ventricular failure during pregnancy — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).

Note

  • Before measuring blood pressure, ask the patient if an arm is unreliable or look for a thoracotomy scar; After the classic Blalock–Thomas–Taussig shunt, the pressure in that arm may not reflect the aortic pressure.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
With unrepaired or only palliative shunt (cyanotic)

Approach

  1. Note that cyanosis is the primary determinant of maternal risk in an unrepaired cyanotic lesion; Get an expert evaluation before pregnancy. Use CARPREG II (NYHA III–IV or cyanosis 3 points) and ZAHARA (cyanotic heart disease) scores for risk stratification. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  2. Record basal oxygen saturation; Evaluate ventricular function and pulmonary hypertension by echocardiography.
  3. Explain in counseling that there is an increased risk of miscarriage, premature birth, and fetal growth restriction. Perform fetal growth monitoring; Plan your birth in a center with neonatal intensive care facilities.
  4. Provide genetic counseling; it is particularly valuable in conotruncal anomalies (22q11.2 deletion is inherited in an autosomal dominant manner). Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  5. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  6. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.
  7. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.
  8. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.

Treatment

  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Review teratogenic or fetotoxic drugs; discontinue ACE inhibitors and ARBs before pregnancy, and do not use them during pregnancy. Do not use DOACs during pregnancy and breastfeeding; warfarin crosses the placenta and is teratogenic, so avoid it during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Before measuring blood pressure, ask the patient if an arm is unreliable or look for a thoracotomy scar; After the classic Blalock–Thomas–Taussig shunt, the pressure in that arm may not reflect the aortic pressure.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Transposition of the great arteries (TGA)
Type of repair
Arterial switch (anatomical repair)

Approach

  1. Obtain expert evaluation before pregnancy as a complex repaired lesion; Use mWHO, CARPREG II and ZAHARA scores together in risk classification. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  2. Explain that pregnancy data after arterial switch are limited and that the prognosis is expected to be more positive since the repair is anatomical.
  3. Evaluate ventricular and valve function, residual lesions, and pulmonary pressure by ECHO before pregnancy; Record basal oxygen saturation.
  4. Provide genetic counseling; it is particularly valuable in conotruncal anomalies (22q11.2 deletion is inherited in an autosomal dominant manner). Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  5. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  6. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  7. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • No specific drug treatment is required.

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
Atrial switch (Mustard/Senning) or congenital corrected TGA (systemic right ventricle)
Systemic right ventricular status
Function preserved or slightly reduced, NYHA I–II; No more than moderate tricuspid regurgitation and treated heart failure

Approach

  1. Risk class: mWHO III (systemic right ventricle with preserved or slightly reduced function); The risk of maternal mortality or severe morbidity is significantly increased. Explain that pregnancy is not absolutely contraindicated, but complications are common and intensive monitoring is required during antenatal, delivery and postpartum periods.
  2. Follow up from pre-pregnancy to puerperium in a specialist center with an experienced team and a Pregnancy Heart Team. Perform cardiology follow-up usually every 1-2 months.
  3. Discuss the risk of premature failure of the systemic right ventricle; A more rapid decline in right ventricular systolic function has been reported in pregnancy after atrial switch. Monitor functional status and cyanosis as the most reliable risk markers; Entering pregnancy with NYHA III–IV is associated with a severe course. Consider the systemic right ventricle 'at risk' during the puerperium and monitor closely for failure. Plan extended monitoring and early follow-up for 48–72 hours after birth.
  4. Record baseline ECG, ECHO, oxygen saturation and BNP/NT-proBNP; additionally use the ZAHARA score in risk estimation.
  5. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  6. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  8. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • If there is tachyarrhythmia requiring rate control during puerperium — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If there are signs of systemic right ventricular failure or ventricular dysfunction during puerperium, for afterload reduction by expert cardiology decision — Enalapril: 2.5 mg twice a day is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • If there is congestion or fluid overload in the puerperium — Furosemide (oral): 20–40 mg once a day is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).

Note

  • If pregnancy is planned, discontinue ACE inhibitor and ARB before pregnancy or switch to pregnancy-compatible alternatives; Assess maternal stability with changed therapy. If the first application is during pregnancy, do not delay medication adjustment. Discontinue the ACE inhibitor at the beginning of the first trimester to prevent fetal renal complications.
  • Evaluate the safety of each medication separately during breastfeeding in the breastfeeding patient before discharge.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Do not routinely start metoprolol, enalapril or furosemide solely because a patient is postpartum when function is preserved and there are no symptoms or congestion.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
NYHA III–IV, more than mild ventricular dysfunction, more than moderate tricuspid regurgitation, or treated heart failure

Approach

  1. Risk class: mWHO IV (NYHA III–IV, systemic right ventricle with more than mild ventricular dysfunction, more than moderate tricuspid regurgitation, or treated heart failure); The risk of maternal mortality or severe morbidity is extremely high, pregnancy is contraindicated.
  2. Record NYHA functional class, rhythm, and history of previous cardiac events at initial evaluation; Evaluate ventricular and valve function, pulmonary pressure with ECG and ECHO; Document baseline oxygen saturation.
  3. Recommend avoiding pregnancy if pregnancy is planned; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  4. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  5. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If pregnancy persists, centralize care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care, and neonatal expertise.
  6. If pregnancy persists, closely monitor the systemic right ventricle with an experienced team; Consider the patient 'at risk' during the puerperium and continue monitoring for insufficiency. Plan extended monitoring and early follow-up for 48–72 hours after birth. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  7. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. If pregnancy continues, discuss the risk of recurrence of congenital heart disease in the child and recommend fetal echocardiography at 18–22 weeks.
  8. Prefer vaginal birth; Perform cesarean section in obstetric indication or acute refractory heart failure. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.

Treatment

  • If there is tachyarrhythmia requiring rate control during puerperium — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If there are signs of systemic right ventricular failure or ventricular dysfunction during puerperium, for afterload reduction by expert cardiology decision — Enalapril: 2.5 mg twice a day is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • If there is congestion or fluid overload in the puerperium — Furosemide (oral): 20–40 mg once a day is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).

Note

  • If pregnancy is planned, discontinue ACE inhibitor and ARB before pregnancy or switch to pregnancy-compatible alternatives; Assess maternal stability with changed therapy. If the first application is during pregnancy, do not delay medication adjustment. Discontinue the ACE inhibitor at the beginning of the first trimester to prevent fetal renal complications.
  • Evaluate the safety of each medication separately during breastfeeding in the breastfeeding patient before discharge.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Do not routinely start metoprolol, enalapril or furosemide solely because a patient is postpartum when function is preserved and there are no symptoms or congestion.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Fontan circulation
Clinical situation
Uncomplicated: NYHA I–II; No cyanosis, ventricular dysfunction, significant atrioventricular valve regurgitation, severe or refractory arrhythmia, and protein-losing enteropathy

Approach

  1. Risk class: mWHO III (uncomplicated Fontan circulation); The risk of maternal mortality or severe morbidity is significantly increased. Explain that pregnancy is not automatically contraindicated, but complications are common and intensive monitoring is required during antenatal, delivery and postpartum periods.
  2. Manage from pre-pregnancy to postpartum in a center with adult congenital heart disease experience and a Pregnancy Heart Team. Perform cardiology follow-up usually every 1-2 months.
  3. Explain in counseling that there is a high risk of pregnancy loss, premature birth and small babies for gestational age, and that the main maternal complications are arrhythmia and heart failure. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  4. Record baseline ECG, echocardiography, oxygen saturation and BNP/NT-proBNP before pregnancy or at first admission; Compare if symptoms develop.
  5. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  6. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely. Take into account the intolerance of the Fontan circulation to increased intrathoracic and central venous pressure in your delivery and anesthesia plan; Provide appropriate analgesia and minimize Valsalva during vaginal birth. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  7. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an assisted reproductive technique is planned, make the decision with the fertility specialist and the Pregnancy Heart Team before the treatment; discuss ovarian hyperstimulation syndrome and the risk of multiple pregnancy; If possible, apply a single embryo transfer.
  8. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Use an air filter on IV lines if fenestration or significant venovenous collaterals are present.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
With major complications (ventricular dysfunction, severe/refractory arrhythmia, cyanosis, NYHA III–IV, significant atrioventricular valve regurgitation or protein-losing enteropathy)

Approach

  1. Risk class: mWHO IV (Fontan circulation with major complications); The risk of maternal mortality or severe morbidity is extremely high, pregnancy is contraindicated.
  2. Record NYHA functional class, rhythm, and history of previous cardiac events at initial evaluation; Evaluate ventricular and valve function, pulmonary pressure with ECG and ECHO; Document baseline oxygen saturation. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  3. Recommend avoiding pregnancy if pregnancy is planned; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  4. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  5. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If the pregnancy continues, those with adult congenital heart disease experience; Centralize in a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care and neonatal specialties.
  6. Explain that cyanosis before pregnancy increases the risk of miscarriage, preterm birth, and fetal growth restriction; monitor fetal growth. Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Prefer vaginal birth; Perform cesarean section in obstetric indication or acute refractory heart failure. Take into account the intolerance of the Fontan circulation to increased intrathoracic and central venous pressure in your delivery and anesthesia plan; Provide appropriate analgesia and minimize Valsalva during vaginal birth. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  8. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Use an air filter on IV lines if fenestration or significant venovenous collaterals are present.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Eisenmenger syndrome
Pregnancy status
Pregnancy is planned or not pregnant

Approach

  1. Risk class: mWHO IV; Pregnancy is contraindicated.
  2. Recommend avoiding pregnancy; Explain that maternal mortality is very high and most deaths occur during childbirth and early puerperium. Individualize counseling at a specialist Pregnancy Heart Team or pulmonary hypertension center without relying on a single fixed mortality rate; Talk about fetal complications being also high.
  3. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and patient understanding and preferences.
  4. Record basal oxygen saturation; Evaluate ventricular function and pulmonary pressure by echocardiography.
  5. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  6. Discuss the risk of recurrence of congenital heart disease in the child and the need for fetal echocardiography at 18–22 weeks.
  7. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  8. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • Targeted PAH treatment: if pregnancy is planned, switch to pregnancy-compatible treatment (sildenafil; prostacyclin analogue if necessary) before pregnancy in a specialist center with endothelin receptor antagonists, riociguat and selexipag; Do not discontinue effective treatment in a patient who is not planning a pregnancy just because she is not pregnant.

Note

  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Do not use endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat and selexipag during pregnancy; provide reliable contraception in women of childbearing age using these medications—add a barrier method because endothelin receptor antagonists may reduce the effectiveness of hormonal contraceptives; If pregnancy is detected, discontinue the medication and refer urgently to a specialist center.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
Pregnancy present

Approach

  1. Risk class: mWHO IV; Maternal mortality is very high and deaths occur most at birth and in the first week of puerperium.
  2. Refer urgently to Specialist Pregnancy Heart Team or pulmonary hypertension centre. In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If the pregnancy continues, centralize care in a center with experience in pulmonary hypertension, right heart failure, targeted pulmonary vasodilator therapy, intensive care and mechanical circulatory support.
  3. Record NYHA functional class, rhythm, and history of previous cardiac events at initial evaluation; Evaluate ventricular and valve function, pulmonary pressure with ECG and ECHO; Document baseline oxygen saturation.
  4. Do not delay hospitalization; Late diagnosis and delayed hospitalization significantly increase maternal mortality.
  5. Monitor for heart failure, hemoptysis, sudden arrhythmic death, cerebrovascular accident and hyperviscosity syndrome. Monitor oxygen saturation; If pulmonary pressure decreases and saturation increases with oxygen, the resistance is not constant and the prognosis is better. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  6. Monitor fetal growth and recommend fetal echocardiography at 18–22 weeks. Plan delivery at a center with neonatal intensive care facilities.
  7. Plan the mode and time of birth in a timely manner with the Pregnancy Heart Team attended by a pulmonary hypertension specialist; Cesarean section may be preferred to vaginal birth as it provides a more controlled birth. Regional anesthesia is generally preferred to general anesthesia. In cases of progressive heart failure refractory to treatment and to reduce the risk of unplanned birth outside a specialist center, plan for premature birth by monitoring fetal growth.
  8. Create the birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  9. Perform meticulous fluid balance monitoring with ECG, pulse oximetry, cardiac output, and central venous pressure during and after birth in the intensive care unit; Most maternal deaths occur in the postpartum period, the risk persists for months.
  10. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. Avoid combined hormonal contraception that contains estrogen. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%.
  11. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • Apply activity restriction.
  • Oxygen: maintain supplemental oxygen if indicated; adjust flow based on saturation and symptom response; Give supplemental oxygen during labor.
  • If targeted pulmonary vasodilator therapy is required — Sildenafil (oral): 20 mg 3 times daily; If it cannot be taken orally, 10 mg IV bolus 3 times a day.
  • If acute pulmonary vasodilator required — Inhaled nitric oxide: 20 ppm (range 5–20 ppm); No additional vasodilator response is expected between 20 and 40 ppm; not stopped suddenly: the dose is reduced in several steps, waiting several hours at each step and monitoring hypoxemia; It is usually safe to cut down to 10 ppm (temporary FiO2 increase may be required); If oxygenation or hemodynamics are impaired, it is reduced to 5 or 1 ppm before discontinuation.
  • Significant right ventricular dysfunction, progressive disease in functional class III, class IV symptoms, or clinical worsening despite oral therapy without delay in a specialized center — Epoprostenol (IV): start at 1–2 ng/kg/min by continuous infusion through a central venous catheter into a separate lumen with no other medication administered; titrate in increments of 1–2 ng/kg/min at intervals sufficient to assess clinical response (as little as 15 minutes, up to 2–4 weeks); It is not stopped suddenly, avoiding infusion interruption and large sudden dose reduction. Option if parenteral therapy cannot be applied: inhaled iloprost, 2.5 µg per dose, 5 µg if tolerated, 6–9 times a day (at least 2 hours apart), up to 45 µg daily.
  • Mechanical circulatory support (including ECMO): perform in specialist center if necessary.
  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy. Discontinue endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat, and selexipag due to possible teratogenicity; do not use during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • In women with severe PAH or complications during pregnancy/birth, optimization of PAH treatment should be prioritized over breastfeeding.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • In Eisenmenger syndrome, SpO₂ below 95% alone is not an indication for noninvasive or invasive ventilation; The decision on ventilation is made by the intensive care and pulmonary hypertension team.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Sildenafil (oral): Not for use with organic nitrates (continuous or intermittent) and riociguat; Sudden discontinuation may worsen PAH.
  • Sildenafil (oral): The risk of premature birth and small for gestational age (SGA) has been reported during pregnancy.
  • Inhaled nitric oxide: Sudden discontinuation may cause rebound pulmonary hypertension and hemodynamic collapse even after short-term use; In this case, it starts over immediately. The risk of methemoglobinemia increases at doses above 40 ppm; Methemoglobin is measured 4–8 hours after the start and periodically, and inspired NO2 is monitored.
  • Epoprostenol (IV): Sudden discontinuation or large abrupt dose reduction may cause rebound pulmonary hypertension; Spare pump, infusion set and medication are kept ready. Prostanoids may disrupt platelet aggregation and increase bleeding.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Cyanotic congenital heart disease (unrepaired or palliative)
Pulmonary hypertension
No pulmonary hypertension

Approach

  1. Note that cyanosis is the primary determinant of maternal risk in an unrepaired cyanotic lesion; Get an expert evaluation before pregnancy. Use CARPREG II (NYHA III–IV or cyanosis 3 points) and ZAHARA (cyanotic heart disease) scores for risk stratification. Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  2. Record basal oxygen saturation; Evaluate ventricular function and pulmonary hypertension by echocardiography.
  3. Explain in counseling that there is an increased risk of miscarriage, premature birth, and fetal growth restriction. Perform fetal growth monitoring; Plan your birth in a center with neonatal intensive care facilities.
  4. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  5. Determine mode of delivery according to obstetric indication; Perform cesarean section only for obstetric indication, not routinely.
  6. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.
  7. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.
  8. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.

Treatment

  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Significant pulmonary arterial hypertension or Eisenmenger syndrome

Approach

  1. Risk class: mWHO IV (significant pulmonary arterial hypertension of any cause or Eisenmenger syndrome); pregnancy is contraindicated. If pulmonary hypertension is non-PAH or of uncertain type, do not assign mWHO IV based solely on elevated pressure; determine risk with an expert team according to the underlying disease, ventricular function, and functional status.
  2. Recommend avoiding pregnancy if pregnancy is planned; individualize counseling at a specialist Pregnancy Heart Team or pulmonary hypertension centre; Record the decision, its rationale, and the patient's preferences.
  3. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If an intrauterine device is to be chosen, choose an intrauterine device that releases levonorgestrel because it reduces menstrual bleeding; Avoid copper intrauterine devices due to the risk of excessive menstrual bleeding, especially in the cyanotic patient with a hematocrit greater than 55%.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the option of terminating the pregnancy if it is legally and ethically appropriate. If pregnancy continues, centralize care at a specialist center with experience in pulmonary hypertension, right heart failure, targeted pulmonary vasodilator therapy, intensive care and mechanical circulatory support.
  5. Plan close monitoring during late pregnancy, birth, and early puerperium when risk is highest. Do not delay hospitalization in Eisenmenger syndrome; Late diagnosis and delayed hospitalization significantly increase maternal mortality.
  6. Record basal oxygen saturation; If pulmonary pressure decreases and saturation increases with oxygen, the resistance is not constant and the prognosis is better. Be aware that the physiological decrease in systemic vascular resistance during pregnancy may initiate right-to-left shunting in a patient without previous cyanosis.
  7. If Eisenmenger syndrome is present, monitor for heart failure, hemoptysis, sudden arrhythmic death, cerebrovascular accident and hyperviscosity syndrome. Treat chronic, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  8. Monitor fetal growth; Plan delivery at a center with neonatal intensive care facilities. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  9. Plan the mode and time of birth in a timely manner with the Pregnancy Heart Team attended by a pulmonary hypertension specialist; Cesarean section may be preferred to vaginal birth as it provides a more controlled birth. Regional anesthesia is generally preferred to general anesthesia. In cases of progressive heart failure refractory to treatment and to reduce the risk of unplanned birth outside a specialist center, plan for premature birth by monitoring fetal growth.
  10. Create the birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  11. Perform meticulous fluid balance monitoring with ECG, pulse oximetry, cardiac output, and central venous pressure during and after birth in the intensive care unit; Most maternal deaths occur in the postpartum period, the risk persists for months.
  12. Increased thromboembolic risk; individualize the decision for anticoagulation or thromboprophylaxis based on the lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Make the plan before pregnancy if possible.

Treatment

  • Apply activity restriction in symptomatic PAH or Eisenmenger syndrome; give bed rest if necessary; Plan thromboprophylaxis together for long periods of immobility.
  • In Eisenmenger syndrome — Oxygen: maintain supplemental oxygen if indicated; adjust flow based on saturation and symptom response; Give supplemental oxygen during labor.
  • If hypoxemia is present in non-Eisenmenger significant PAH — Oxygen: SpO₂ is maintained at least 90%, preferably 95% and above.
  • If targeted pulmonary vasodilator therapy is required — Sildenafil (oral): 20 mg 3 times daily; If it cannot be taken orally, 10 mg IV bolus 3 times a day.
  • Significant right ventricular dysfunction, progressive disease in functional class III, class IV symptoms, or clinical worsening despite oral therapy without delay in a specialized center — Epoprostenol (IV): start at 1–2 ng/kg/min by continuous infusion through a central venous catheter into a separate lumen with no other medication administered; titrate in increments of 1–2 ng/kg/min at intervals sufficient to assess clinical response (as little as 15 minutes, up to 2–4 weeks); It is not stopped suddenly, avoiding infusion interruption and large sudden dose reduction. Option if parenteral therapy cannot be applied: inhaled iloprost, 2.5 µg per dose, 5 µg if tolerated, 6–9 times a day (at least 2 hours apart), up to 45 µg daily.
  • If acute pulmonary vasodilator required — Inhaled nitric oxide: 20 ppm (range 5–20 ppm); No additional vasodilator response is expected between 20 and 40 ppm; not stopped suddenly: the dose is reduced in several steps, waiting several hours at each step and monitoring hypoxemia; It is usually safe to cut down to 10 ppm (temporary FiO2 increase may be required); If oxygenation or hemodynamics are impaired, it is reduced to 5 or 1 ppm before discontinuation.
  • Mechanical circulatory support (including ECMO): perform in specialist center if necessary.
  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • In unrepaired cyanotic congenital heart disease (highest endocarditis risk), prophylaxis for vaginal delivery may be considered in selected patients by specialist team decision — Endocarditis prophylaxis: 30–60 minutes before anticipated vaginal delivery using the same regimen as for cesarean prophylaxis (cefazolin 2 g IV; 3 g for body weight ≥120 kg) or a single dose of ampicillin 2 g IV; for cesarean delivery, routine cesarean prophylaxis is sufficient; in patients with a history of anaphylaxis, angioedema, or urticaria to penicillin or ampicillin, give vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of a cephalosporin, infused over at least 60 minutes at a maximum rate of 10 mg/min (a 2 g dose takes approximately 200 minutes).

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy. Discontinue endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat, and selexipag due to possible teratogenicity; do not use during pregnancy.
  • Routine endocarditis prophylaxis is not required for vaginal delivery; Routine antibiotic prophylaxis before cesarean section also protects against endocarditis.
  • In women with severe PAH or complications during pregnancy/birth, optimization of PAH treatment should be prioritized over breastfeeding.
  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • In Eisenmenger syndrome, SpO₂ below 95% alone is not an indication for noninvasive or invasive ventilation; The decision on ventilation is made by the intensive care and pulmonary hypertension team.
  • If there is a right-to-left shunt, use an air filter on all IV lines and carefully remove air from all connections; Administer drugs with caution that may cause sudden systemic vasodilatation.
  • Sildenafil (oral): Not for use with organic nitrates (continuous or intermittent) and riociguat; Sudden discontinuation may worsen PAH.
  • Sildenafil (oral): The risk of premature birth and small for gestational age (SGA) has been reported during pregnancy.
  • Epoprostenol (IV): Sudden discontinuation or large abrupt dose reduction may cause rebound pulmonary hypertension; Spare pump, infusion set and medication are kept ready. Prostanoids may disrupt platelet aggregation and increase bleeding.
  • Inhaled nitric oxide: Sudden discontinuation may cause rebound pulmonary hypertension and hemodynamic collapse even after short-term use; In this case, it starts over immediately. The risk of methemoglobinemia increases at doses above 40 ppm; Methemoglobin is measured 4–8 hours after the start and periodically, and inspired NO2 is monitored.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
Aortic coarctation
Repair status
Repaired

Approach

  1. Risk class: repaired coarctation without residual stenosis mWHO II–III; severe coarctation or recoarctation mWHO IV. Do not accept repair history alone as a low risk indicator; Residual stenosis (gradient >20 mmHg, aortic lumen <12 mm), signs of heart failure, LVEF <40% or NYHA class >I increase the risk of complications; If there is an aneurysm, the risk of dissection increases.
  2. Obtain expert evaluation before pregnancy; Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  3. Note that systemic hypertension may persist after repair; Monitor blood pressure throughout pregnancy. Explain in counseling that hypertension is common during pregnancy and the risk of dissection in the coarctation area; Fatal dissection has been reported at 36 weeks.
  4. Consider during follow-up the complications of cerebral berry aneurysm rupture, heart failure, and ischemic heart disease due to cephalic hypertension. If Turner syndrome accompanies it, keep in mind that the risk of pregnancy and hypertensive complications is higher.
  5. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  6. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Prefer vaginal birth; If there is aortic aneurysm, heart failure or severe hypertension, determine the delivery method with the expert team and consider cesarean section.
  8. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • In systemic hypertension that persists after repair — Labetalol (oral): Start at 100 mg twice a day, increase by 100 mg (twice a day) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • In systemic hypertension that persists after repair — Nifedipine (long-acting, oral): 30–60 mg is started once a day and increased at intervals of 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In systemic hypertension that persists after repair — Methyldopa (oral): 250 mg 2–3 times a day is started, increased every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Unrepaired

Approach

  1. Risk class: repaired coarctation without residual stenosis mWHO II–III; severe coarctation or recoarctation mWHO IV. Do not accept repair history alone as a low risk indicator; Residual stenosis (gradient >20 mmHg, aortic lumen <12 mm), signs of heart failure, LVEF <40% or NYHA class >I increase the risk of complications; If there is an aneurysm, the risk of dissection increases.
  2. Evaluate pre-pregnancy interventional treatment of significant coarctation at an adult congenital heart center.
  3. Obtain expert evaluation before pregnancy; Plan multidisciplinary follow-up with high-risk obstetrics and cardiology, starting before pregnancy.
  4. Explain that unrepaired coarctation has historically been associated with high rates of obstetric and cardiac complications; current data is limited. Explain in counseling that hypertension is common during pregnancy and the risk of dissection in the coarctation area; Fatal dissection has been reported at 36 weeks.
  5. Monitor blood pressure throughout pregnancy; In the evaluation, take into account the pressure difference between the right arm and lower extremities.
  6. Consider during follow-up the complications of cerebral berry aneurysm rupture, heart failure, and ischemic heart disease due to cephalic hypertension. If Turner syndrome accompanies it, keep in mind that the risk of pregnancy and hypertensive complications is higher.
  7. Treat persistent, symptomatic or decompensating arrhythmia without delay by evaluating its type and hemodynamic effect.
  8. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  9. Prefer vaginal birth; If there is aortic aneurysm, heart failure or severe hypertension, determine the delivery method with the expert team and consider cesarean section.
  10. Initiate contraception counseling before need arises; The intrauterine device is an excellent option.

Treatment

  • If systemic hypertension — Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If not appropriate or as an adjunct — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Administer bed rest and hospitalization for resistant hypertension, maternal or fetal deterioration, or severe symptomatic (re)coarctation; Evaluate stent intervention at a specialist center.

Note

  • Do not automatically start aggressive pharmacological treatment for isolated benign premature beats.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
  • Avoid placental hypoperfusion when lowering blood pressure in residual or native stenosis; monitor treatment using right-arm blood pressure.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.

Aortopathies

Marfan syndrome
Aortic diameter
No significant aortic dilatation; no previous dissection with progressive dilatation

Approach

  1. Risk class: mWHO II–III (Marfan syndrome without significant aortic dilatation); There is a moderate increase in maternal mortality or a moderate to severe increase in morbidity. Explain that the risk is closely related to aortic root diameter and that pregnancy and puerperium may increase the risk of progressive dilatation and dissection; Provide detailed counseling regarding the possibility of worsening and the need for structured monitoring. In Marfan syndrome with previous aortic root replacement or previous aortic dissection with a stable diameter, the risk class is mWHO III; If the diameter increases after dissection, use mWHO IV. Be aware that the risk of peripartum dissection of the distal aortic segments persists after aortic root replacement.
  2. Evaluate and manage from pre-pregnancy to postpartum with the Pregnancy Heart Team. Individualize monitoring frequency based on lesion, ventricular function, arrhythmia burden, and symptoms.
  3. Visualize the entire aorta with CT or cardiac MRI before pregnancy; Include diagnosis, aortic diameter, growth rate, genotype, family history, and previous vascular events in the risk assessment. In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. In low-risk aortic disease (mWHO II and II–III), perform echocardiography once between weeks 20 and 30 and imaging at 6 months postpartum; For intermediate-high risk (mWHO III and IV), perform ECHO every 4-12 weeks throughout pregnancy and up to the 6th month postpartum.
  4. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities.
  5. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Perform an obstetric anesthesia evaluation for dural ectasia and scoliosis before birth. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  6. Provide genetic counseling; if an autosomal dominant pathogenic variant is present, the risk to the child is approximately 50%; discuss genetic testing or preimplantation genetic testing if the familial variant is known. Include fetal echocardiography at 18–22 weeks in prenatal diagnostic counseling.
  7. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.

Treatment

  • During pregnancy and puerperium — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Switch from teratogenic or pregnancy-incompatible medications to safe alternatives before pregnancy; Stop ACE inhibitor and ARB.
  • Avoid atenolol in your choice of β-blocker.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
There is moderate aortic dilatation, diameter ≤ 45 mm; no previous dissection with progressive dilatation

Approach

  1. Risk class: mWHO III (Marfan syndrome with moderate aortic dilatation); The risk of maternal mortality or severe morbidity is significantly increased. Provide individual counseling for intermediate aortic diameters.
  2. Manage from pre-pregnancy to postpartum in a specialist center with a Pregnancy Heart Team. Perform cardiology follow-up usually every 1-2 months.
  3. Visualize the entire aorta with CT or cardiac MRI before pregnancy; Include aortic diameter, growth rate, genotype, family history, and previous vascular events in risk assessment. In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. Perform echocardiography every 4–12 weeks throughout pregnancy and up to the 6th month after birth.
  4. Explain that pregnancy and puerperium may increase the risk of progressive aortic dilatation and dissection; Watch puerperium as a risk period.
  5. If pregnancy occurs, plan the birth at a specialist center; Perform fetal growth monitoring. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  6. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Perform an obstetric anesthesia evaluation for dural ectasia and scoliosis before birth. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  7. Provide genetic counseling; if an autosomal dominant pathogenic variant is present, the risk to the child is approximately 50%; discuss genetic testing or preimplantation genetic testing if the familial variant is known. Include fetal echocardiography at 18–22 weeks in prenatal diagnostic counseling.
  8. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.

Treatment

  • During pregnancy and puerperium — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Prophylactic aortic surgery: consider pre-pregnancy surgery if the aortic root is 40–45 mm and annual growth is greater than 3 mm or there is a family history of aortic dissection.

Note

  • Avoid atenolol in your choice of β-blocker.
  • Switch from teratogenic or pregnancy-incompatible medications to safe alternatives before pregnancy; Stop ACE inhibitor and ARB.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Aortic diameter > 45 mm or previous aortic dissection with progressive dilatation

Approach

  1. Risk class: mWHO IV (Marfan syndrome with aortic diameter > 45 mm or previous aortic dissection with progressive dilatation); The risk of maternal mortality or severe morbidity is extremely high, pregnancy is contraindicated.
  2. Recommend avoiding pregnancy if pregnancy is planned; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  3. Provide effective contraception; Choose long-acting reversible methods such as intrauterine device or progestin implant. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If pregnancy persists, centralize care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care, and neonatal expertise.
  5. Visualize the entire aorta with CT or cardiac MRI before pregnancy. If the pregnancy continues, perform an ECHO every 4–12 weeks throughout pregnancy and until the 6th month after birth; If pre-pregnancy cross-sectional imaging is not available, evaluate the entire aorta with gadolinium-free MRI; Observe puerperium as a risk period for dissection.
  6. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  7. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Perform an obstetric anesthesia evaluation for dural ectasia and scoliosis before birth. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  8. Provide genetic counseling; if an autosomal dominant pathogenic variant is present, the risk to the child is approximately 50%; discuss genetic testing or preimplantation genetic testing if the familial variant is known. Include fetal echocardiography at 18–22 weeks in prenatal diagnostic counseling.

Treatment

  • Prophylactic aortic surgery: if the aortic root diameter is over 45 mm, recommend surgery before pregnancy and complete it before pregnancy.
  • During pregnancy and puerperium — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Avoid atenolol in your choice of β-blocker.
  • Switch from teratogenic or pregnancy-incompatible medications to safe alternatives before pregnancy; Stop ACE inhibitor and ARB.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Aortopathy due to bicuspid aortic valve
Ascending aortic diameter
Ascending aorta < 50 mm; no previous dissection with progressive dilatation

Approach

  1. Evaluate with the Pregnancy Heart Team from before pregnancy. Risk class: mWHO II–III if the ascending aorta is <45 mm, mWHO III if it is 45–50 mm; Follow up at a specialist center with a Pregnancy Heart Team.
  2. Include both valve function (aortic stenosis and regurgitation) and ascending aortic diameter in the risk assessment. Before pregnancy, image the entire aorta with CT or cardiac MRI; In addition to the aortic root and ascending aorta, also evaluate the descending aorta (with ECHO) to exclude coarctation; Include diagnosis, aortic diameter, growth rate, genotype, family history, and previous vascular events in the risk assessment. In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. In low-risk aortic disease (mWHO II and II–III), perform an echocardiogram once between 20–30 weeks and perform imaging at 6 months postpartum; For intermediate-high risk (mWHO III and IV), perform ECHO every 4-12 weeks throughout pregnancy and up to the 6th month postpartum.
  3. Explain that pregnancy and puerperium may increase the risk of progressive aortic dilatation and dissection; Watch puerperium as a risk period.
  4. Plan delivery at the reference center for a medium or high risk patient. Perform fetal growth monitoring at moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities.
  5. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  6. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.
  7. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.

Treatment

  • Pre-pregnancy aortic surgery: evaluate at diameter ≥45 mm if there is a root phenotype or a family history of aortic aneurysm/dissection; If cardiac surgery is required for another reason, evaluate the 45 mm threshold on an individual basis.

Note

  • Discontinue or change teratogenic or fetotoxic cardiac medications (ACE inhibitor, ARB) before pregnancy.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
≥ 50 mm or previous aortic dissection with progressive dilatation

Approach

  1. Risk class: mWHO IV (avoid pregnancy) applies to diameter > 50 mm or previous dissection with progressive dilatation; Individualize the risk at exactly 50 mm with the expert team.
  2. Evaluate valve function (aortic stenosis and regurgitation) with ECHO. Before pregnancy, image the entire aorta with CT or cardiac MRI; In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. If the pregnancy continues, perform an ECHO every 4-12 weeks throughout pregnancy and until the 6th month after birth.
  3. Recommend avoiding pregnancy if pregnancy is planned; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  4. Provide effective contraception; Choose long-acting reversible methods such as intrauterine device or progestin implant. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  5. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If pregnancy persists, centralize care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care, and neonatal expertise.
  6. If pregnancy continues, monitor considering that the risk of progressive dilatation and dissection increases during pregnancy and puerperium.
  7. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  8. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  9. Discuss the risk of recurrence of congenital heart disease in the child (including the possible type of lesion if a first-degree relative is affected) and recommend fetal echocardiography at 18–22 weeks.

Treatment

  • Aortic surgery: perform before pregnancy when the diameter of the ascending aorta reaches 50 mm.

Note

  • Discontinue or change teratogenic or fetotoxic cardiac medications (ACE inhibitor, ARB) before pregnancy.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
Turner syndrome
Aortic size index (ASI)
ASI ≤ 25 mm/m²; No previous aortic dissection with progressive dilatation

Approach

  1. Risk class: mWHO II if there are no cardiovascular features (BAV, coarctation, hypertension, aortic dilatation); If ASI is 20–25 mm/m², mWHO III. ASI ≤25 mm/m² alone does not indicate low risk. Evaluate with the Pregnancy Heart Team from before pregnancy.
  2. Before pregnancy, image the entire aorta with CT or cardiac MRI and determine the aortic size index (ASI); ASI > 25 mm/m² is in mWHO IV class. In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. In low-risk aortic disease (mWHO II and II–III), perform echocardiography once between weeks 20 and 30 and imaging at 6 months postpartum; For intermediate-high risk (mWHO III and IV), perform ECHO every 4-12 weeks throughout pregnancy and up to the 6th month postpartum. If ASI ≥25 mm/m², aortic height index ≥25 mm/m or Z score >4, discuss the increased risk of dissection and the option of pre-pregnancy surgery with the Pregnancy Heart Team with additional risk factors such as hypertension, BAV and coarctation. Complete a cardiovascular evaluation before fertility treatment. Keep in mind that the risk of hypertensive disease, gestational diabetes and small babies for gestational age increases during pregnancy.
  3. Explain that pregnancy and puerperium may increase the risk of progressive aortic dilatation and dissection; Watch puerperium as a risk period.
  4. Consider the higher risk of pregnancy and hypertensive complications if accompanied by aortic coarctation; Monitor for hypertensive complications.
  5. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.

Treatment

  • Prophylactic aortic surgery: consider need in pre-pregnancy risk assessment; If necessary, complete before pregnancy.

Note

  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
ASI > 25 mm/m² or previous aortic dissection with progressive dilatation

Approach

  1. Risk class: mWHO IV (Turner syndrome with ASI > 25 mm/m² or previous aortic dissection with progressive dilatation); The risk of maternal mortality or severe morbidity is extremely high, pregnancy is contraindicated.
  2. Recommend avoiding pregnancy if pregnancy is planned; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  3. Provide effective contraception; Choose long-acting reversible methods such as intrauterine device or progestin implant. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If pregnancy persists, centralize care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care, and neonatal expertise.
  5. If pregnancy continues, monitor considering that the risk of progressive dilation and dissection increases during pregnancy and puerperium. If accompanied by aortic coarctation, consider that the risk of pregnancy and hypertensive complications is higher; Monitor for hypertensive complications.
  6. Visualize the entire aorta with CT or cardiac MRI before pregnancy; In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. If the pregnancy continues, perform an ECHO every 4-12 weeks throughout pregnancy and until the 6th month after birth.
  7. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.

Treatment

  • Prophylactic aortic surgery: evaluate need if pregnancy is planned; If necessary, complete before pregnancy.

Note

  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Review teratogenic or fetotoxic drugs; Stop ACE inhibitors and ARBs before pregnancy, do not use during pregnancy.
Vascular Ehlers–Danlos syndrome
Pregnancy status
Pregnancy is planned or not pregnant

Approach

  1. Risk class: mWHO IV; The risk is so high because of the possibility of spontaneous arterial rupture or dissection, pregnancy is contraindicated.
  2. Recommend avoiding pregnancy; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  3. Before pregnancy, image the entire aorta, including the supra-aortic vessels and the iliac and femoral arteries, with CT or cardiac MRI; Include diagnosis, aortic diameter, growth rate, genotype, family history, and previous vascular events in the risk assessment. If pregnancy occurs and there is no prior cross-sectional imaging, evaluate the entire aorta with gadolinium-free MRI; Perform echocardiography every 4–12 weeks throughout pregnancy and up to the 6th month after birth.
  4. Provide genetic counseling; If the autosomal dominant pathogenic variant is present, the risk in the child is approximately 50%; Discuss genetic testing or preimplantation genetic testing if the familial variant is known.
  5. Provide effective contraception; Choose long-acting reversible methods such as intrauterine device or progestin implant. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.

Treatment

  • Start before pregnancy, continue throughout pregnancy and lactation (also in normotensive patients) — Celiprolol (oral): Start at 200 mg once daily; If the response is insufficient, 400 mg once a day after 2–4 weeks; It is not stopped suddenly, it is stopped gradually over 7-10 days.
  • If celiprolol is not available, by decision of the expert center — Metoprolol (oral): immediate-release tartrate 25 mg twice a day is started, gradually increased as tolerated, to a maximum of 200 mg per day, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Avoid atenolol in your choice of β-blocker.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Celiprolol: Not used in second or third degree AV block, severe bradycardia (below 45–50/min) and hypotension (systolic <100 mmHg); Make the decision jointly at the specialist center by discussing the pregnancy and breastfeeding restrictions in the product information with the patient; Monitor the newborn for bradycardia, hypoglycemia, and respiratory distress for the first 3–5 days after birth.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Pregnancy present

Approach

  1. Risk class: mWHO IV; The risk is so high because of the possibility of spontaneous arterial rupture or dissection, pregnancy is contraindicated.
  2. Refer urgently to the Specialist Pregnancy Heart Team. In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If pregnancy persists, centralize care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care, and neonatal expertise.
  3. Monitor throughout pregnancy and puerperium, taking into account the risk of artery rupture and dissection.
  4. If pre-pregnancy cross-sectional imaging is not available, evaluate the entire aorta, including the supra-aortic vessels and iliac and femoral arteries, with gadolinium-free MRI; Perform echocardiography every 4–12 weeks throughout pregnancy and up to the 6th month after birth.
  5. Provide genetic counseling; if an autosomal dominant pathogenic variant is present, the risk to the child is approximately 50%; discuss genetic testing or preimplantation genetic testing if the familial variant is known. Include fetal echocardiography at 18–22 weeks in prenatal diagnostic counseling.
  6. Perform fetal growth monitoring; Plan your birth in a center with neonatal intensive care facilities. If earlier birth is not required, have your birth by planned cesarean section at a specialist center at 37 weeks. Perform an obstetric anesthesia evaluation for dural ectasia and scoliosis before birth; create a birth plan in advance and share it with the entire team; Provide continuous telemetry and close hemodynamic monitoring during and after labor.
  7. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks.

Treatment

  • During pregnancy and during breastfeeding (also in normotensive patients) — Celiprolol (oral): Start at 200 mg once daily; If the response is insufficient, 400 mg once a day after 2–4 weeks; It is not stopped suddenly, it is stopped gradually over 7-10 days.
  • If celiprolol is not available, by decision of the expert center — Metoprolol (oral): immediate-release tartrate 25 mg twice a day is started, gradually increased as tolerated, to a maximum of 200 mg per day, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Avoid atenolol in your choice of β-blocker.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Celiprolol: Not used in second or third degree AV block, severe bradycardia (below 45–50/min) and hypotension (systolic <100 mmHg); Make the decision jointly at the specialist center by discussing the pregnancy and breastfeeding restrictions in the product information with the patient; Monitor the newborn for bradycardia, hypoglycemia, and respiratory distress for the first 3–5 days after birth.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Loeys–Dietz syndrome and other hereditary diseases of the thoracic aorta
Aortic involvement
No severe involvement and no previous dissection with progressive dilatation

Approach

  1. Evaluate with the Pregnancy Heart Team from before pregnancy. Risk class: mWHO II–III if there is no aortic dilatation; mWHO III at 40–45 mm moderate dilatation or diameter-stable previous dissection; mWHO IV over 45 mm; Monitor at a specialist center with a moderately dilated Pregnancy Heart Team. If the diameter increases after dissection, use mWHO IV. Be aware that the risk of peripartum dissection of the distal aortic segments persists after aortic root replacement.
  2. Before pregnancy, image the entire aorta, including the supra-aortic vessels and the iliac and femoral arteries, with CT or cardiac MRI; Include diagnosis, aortic diameter, growth rate, genotype, family history, and previous vascular events in the risk assessment. In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. In low-risk aortic disease (mWHO II and II–III), perform echocardiography once between weeks 20 and 30 and imaging at 6 months postpartum; For intermediate-high risk (mWHO III and IV), perform ECHO every 4-12 weeks throughout pregnancy and up to the 6th month postpartum.
  3. Explain that pregnancy and puerperium may increase the risk of progressive aortic dilatation and dissection; Watch puerperium as a risk period.
  4. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities.
  5. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Perform an obstetric anesthesia evaluation for dural ectasia and scoliosis before birth. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  6. Provide genetic counseling; if an autosomal dominant pathogenic variant is present, the risk to the child is approximately 50%; discuss genetic testing or preimplantation genetic testing if the familial variant is known. Include fetal echocardiography at 18–22 weeks in prenatal diagnostic counseling.
  7. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. If an assisted reproductive technique is planned, make the decision with the Pregnancy Heart Team before treatment; To avoid multiple pregnancy, apply a single embryo transfer if possible.

Treatment

  • Prophylactic aortic surgery (pre-pregnancy): Recommend if the aortic root is ≥45 mm in TGFBR1 or TGFBR2 variant, can be evaluated at ≥40 mm; Consider at ≥45 mm in TGFB2, TGFB3, SMAD2 or SMAD3 variant; In MYH11, ACTA2, PRKG1 or MYLK variant, recommend at ≥45 mm, in non-syndromic disease, at 40–44 mm, evaluate according to genotype, family history and growth rate; Consider surgery if the arch, descending, or abdominal aorta is ≥50 mm.
  • During pregnancy and puerperium — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Switch from teratogenic or pregnancy-incompatible medications to safe alternatives before pregnancy; Stop ACE inhibitor and ARB.
  • Avoid atenolol in your choice of β-blocker.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Severe hereditary thoracic aortic disease or previous aortic dissection with progressive dilatation

Approach

  1. Risk class: mWHO IV (severe hereditary thoracic aortic disease or previous aortic dissection with progressive dilatation); The risk of maternal mortality or severe morbidity is extremely high, pregnancy is contraindicated. Aortic diameter >45 mm or previous dissection with increased diameter is mWHO IV.
  2. Recommend avoiding pregnancy if pregnancy is planned; Discuss maternal risk, fetal outcomes, uncertainty in the evidence, and reproductive alternatives individually. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and the patient's understanding and preferences.
  3. Provide effective contraception; Choose long-acting reversible methods such as intrauterine device or progestin implant. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  4. If pregnancy has occurred, refer urgently to the specialist Pregnancy Heart Team; In early counseling, discuss the risk to the mother, the option of continuing in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy. If pregnancy persists, centralize care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care, and neonatal expertise.
  5. If pregnancy continues, monitor considering that the risk of progressive dilatation and dissection increases during pregnancy and puerperium.
  6. Before pregnancy, image the entire aorta, including the supra-aortic vessels and the iliac and femoral arteries, with CT or cardiac MRI; In pregnant women who do not have cross-sectional imaging before pregnancy, evaluate the entire aorta with gadolinium-free MRI. If the pregnancy continues, perform an ECHO every 4-12 weeks throughout pregnancy and until the 6th month after birth.
  7. Perform fetal growth monitoring in moderate or high cardiovascular risk; In high-risk cases, plan the birth in a center with neonatal intensive care facilities. Create a birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  8. Plan the delivery method with the Pregnancy Heart Team according to aortic diameter, dissection history and genotype: recommend vaginal delivery if aorta <40 mm; At 40–45 mm, consider vaginal birth with epidural analgesia and shortened second stage, caesarean section may also be considered; Consider cesarean section at ≥45 mm or acute, subacute or chronic aortic dissection. Perform an obstetric anesthesia evaluation for dural ectasia and scoliosis before birth. Since the risk of dissection is highest around the 6th postpartum day, consider hospitalization for 1 week postpartum.
  9. Provide genetic counseling; if an autosomal dominant pathogenic variant is present, the risk to the child is approximately 50%; discuss genetic testing or preimplantation genetic testing if the familial variant is known. Include fetal echocardiography at 18–22 weeks in prenatal diagnostic counseling.

Treatment

  • Prophylactic aortic surgery (pre-pregnancy): Recommend if the aortic root is ≥45 mm in TGFBR1 or TGFBR2 variant, can be evaluated at ≥40 mm; Consider at ≥45 mm in TGFB2, TGFB3, SMAD2 or SMAD3 variant; In MYH11, ACTA2, PRKG1 or MYLK variant, recommend at ≥45 mm, in non-syndromic disease, at 40–44 mm, evaluate according to genotype, family history and growth rate; Consider surgery if the arch, descending, or abdominal aorta is ≥50 mm.
  • During pregnancy and puerperium — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Switch from teratogenic or pregnancy-incompatible medications to safe alternatives before pregnancy; Stop ACE inhibitor and ARB.
  • Avoid atenolol in your choice of β-blocker.
  • Do not use ergometrine or methylergometrine postpartum in aortopathy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.

Pulmonary hypertension

Pulmonary arterial hypertension (non-Eisenmenger)
Pregnancy status
Pregnancy is planned or not pregnant

Approach

  1. Risk class: mWHO IV (significant pulmonary arterial hypertension from any cause); Pregnancy is contraindicated.
  2. Recommend avoiding pregnancy; Explain that maternal morbidity and mortality are especially high in late pregnancy, birth and early puerperium. Discuss maternal risk, fetal outcomes (fetal growth restriction, preterm birth), uncertainty in the evidence, and reproductive alternatives individually.
  3. Document in the record that pregnancy is contraindicated, lesion-specific rationale, and patient understanding and preferences.
  4. Record basal oxygen saturation; Evaluate ventricular function and pulmonary pressure by echocardiography.
  5. Avoid estrogen-containing combined hormonal contraception; choose long-acting methods such as intrauterine device or progestin implant; In selection, consider anticoagulation, bleeding profile, risk of infection, and vasovagal response during insertion. If permanent contraception is considered, make the decision without pressure, by mutual consent; Assess procedure and anesthesia risks in advance.
  6. Increased thromboembolic risk; individualize the anticoagulation plan (before pregnancy if possible); Provide clear counseling on maternal protection, fetal safety and monitoring needs.

Treatment

  • Targeted PAH treatment: if pregnancy is planned, switch to pregnancy-compatible treatment (sildenafil; prostacyclin analogue if necessary) before pregnancy in a specialist center with endothelin receptor antagonists, riociguat and selexipag; Do not discontinue effective treatment in a patient who is not planning a pregnancy just because she is not pregnant.

Note

  • Switch from teratogenic or pregnancy-incompatible medications to safe alternatives before pregnancy; Stop ACE inhibitor and ARB.
  • Do not use endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat and selexipag during pregnancy; provide reliable contraception in women of childbearing age using these medications—add a barrier method because endothelin receptor antagonists may reduce the effectiveness of hormonal contraceptives; If pregnancy is detected, discontinue the medication and refer urgently to a specialist center.
Pregnancy present

Approach

  1. Risk class: mWHO IV (significant pulmonary arterial hypertension from any cause); Pregnancy is contraindicated.
  2. Refer urgently to the Specialist Pregnancy Heart Team. In early counseling, discuss the option of terminating the pregnancy if it is legally and ethically appropriate. If pregnancy continues, centralize care at a specialist center with experience in pulmonary hypertension, right heart failure, targeted pulmonary vasodilator therapy, intensive care and mechanical circulatory support.
  3. Record NYHA functional class, rhythm, and history of previous cardiac events at initial evaluation; Evaluate ventricular and valve function, pulmonary pressure with ECG and ECHO; Document baseline oxygen saturation.
  4. Plan close monitoring during late pregnancy, birth, and early puerperium when risk is highest. Monitor oxygen saturation.
  5. Perform fetal growth monitoring; Keep in mind that the risk of premature birth increases. Plan your birth in a center with neonatal intensive care facilities.
  6. Plan the mode and time of birth in a timely manner with the Pregnancy Heart Team attended by a pulmonary hypertension specialist; Cesarean section may be preferred to vaginal birth as it provides a more controlled birth. Regional anesthesia is generally preferred to general anesthesia. In cases of progressive heart failure refractory to treatment and to reduce the risk of unplanned birth outside a specialist center, plan for premature birth by monitoring fetal growth.
  7. Create the birth plan in advance and share it with the entire team; Perform prenatal obstetric anesthesia evaluation and provide continuous telemetry during labor.
  8. Perform meticulous fluid balance monitoring with ECG, pulse oximetry, cardiac output, and central venous pressure during and after birth in the intensive care unit; Most maternal deaths occur in the postpartum period, the risk persists for months.
  9. Discuss reproductive planning and effective contraception; Consider long-acting methods such as intrauterine devices or progestin implants based on patient preference, anticoagulation status, bleeding profile, and procedure risks. Avoid combined hormonal contraception that contains estrogen.
  10. Increased thromboembolic risk; individualize the anticoagulation plan (before pregnancy if possible); Provide clear counseling on maternal protection, fetal safety and monitoring needs.

Treatment

  • If there is hypoxemia — Oxygen: SpO₂ is maintained at least 90%, preferably 95% and above.
  • Apply activity restriction and bed rest if necessary in the symptomatic patient; Plan thromboprophylaxis together for long periods of immobility.
  • In the patient with a positive vasoreactivity test before pregnancy, well controlled with calcium channel blocker, and normal right ventricular function — Long-acting nifedipine or amlodipine: continue throughout pregnancy at the effective dose pre-pregnancy; Switch to targeted PAH therapy in case of right heart failure or clinical worsening.
  • If targeted pulmonary vasodilator therapy is required — Sildenafil (oral): 20 mg 3 times daily; If it cannot be taken orally, 10 mg IV bolus 3 times a day.
  • If acute pulmonary vasodilator required — Inhaled nitric oxide: 20 ppm (range 5–20 ppm); No additional vasodilator response is expected between 20 and 40 ppm; not stopped suddenly: the dose is reduced in several steps, waiting several hours at each step and monitoring hypoxemia; It is usually safe to cut down to 10 ppm (temporary FiO2 increase may be required); If oxygenation or hemodynamics are impaired, it is reduced to 5 or 1 ppm before discontinuation.
  • Significant right ventricular dysfunction, progressive disease in functional class III, class IV symptoms, or clinical worsening despite oral therapy without delay in a specialized center — Epoprostenol (IV): start at 1–2 ng/kg/min by continuous infusion through a central venous catheter into a separate lumen with no other medication administered; titrate in increments of 1–2 ng/kg/min at intervals sufficient to assess clinical response (as little as 15 minutes, up to 2–4 weeks); It is not stopped suddenly, avoiding infusion interruption and large sudden dose reduction. Option if parenteral therapy cannot be applied: inhaled iloprost, 2.5 µg per dose, 5 µg if tolerated, 6–9 times a day (at least 2 hours apart), up to 45 µg daily.
  • Mechanical circulatory support: apply at a specialist center if necessary.
  • In the patient who has been decided to use thromboprophylaxis and whose renal function is appropriate — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia) instead of prenatal LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • If creatinine clearance is < 30 mL/min (in the absence of heparin-induced thrombocytopenia), instead of LMWH postpartum — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours postpartum.
  • For active heparin-induced thrombocytopenia (discontinue all heparin exposure, including heparin flush solutions and heparin-containing catheters, in consultation with hematology), or for a history of heparin-induced thrombocytopenia, use instead of heparin — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • If the indication for therapeutic anticoagulation is determined by the expert team and the creatinine clearance is 30 mL/min and above — Enoxaparin (therapeutic dose): 1 mg/kg SC every 12 hours or 1.5 mg/kg SC once daily.
  • If therapeutic anticoagulation is required and creatinine clearance is below 30 mL/min, with aPTT monitoring — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • Heparin (planned antepartum): usually discontinue 12–24 hours before, taking into account the risk of thrombosis, the agent used, dose and method of delivery. If therapeutic LMWH is used, discontinue it 24 hours before each mode of delivery.
  • Anticoagulation (postpartum): restart 4–6 hours after vaginal birth, 6–12 hours after cesarean section if there is no significant bleeding and no risk of significant bleeding; individualize scheduling. In patients who have not had venous thromboembolism during pregnancy and who do not have an indication for long-term anticoagulation, thromboprophylaxis is generally continued for six weeks after birth.

Note

  • Before choosing an anticoagulant, evaluate renal function and history of heparin-induced thrombocytopenia; In thrombocytopenia due to active or previous heparin, plan non-heparin treatment with an expert team.
  • Wait at least 12 hours after prophylactic LMWH and at least 24 hours after an intermediate or therapeutic dose before neuraxial intervention. If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Do not use DOACs during pregnancy or breastfeeding; warfarin crosses the placenta and is teratogenic, avoid during pregnancy.
  • Discontinue teratogenic drugs such as ACE inhibitors and ARBs.
  • Discontinue endothelin receptor antagonists (bosentan, ambrisentan, macitentan), riociguat, and selexipag due to possible teratogenicity; do not use during pregnancy.
  • In women with severe PAH or complications during pregnancy/birth, optimization of PAH treatment should be prioritized over breastfeeding.
  • Sildenafil (oral): Not for use with organic nitrates (continuous or intermittent) and riociguat; Sudden discontinuation may worsen PAH.
  • Sildenafil (oral): The risk of premature birth and small for gestational age (SGA) has been reported during pregnancy.
  • Inhaled nitric oxide: Sudden discontinuation may cause rebound pulmonary hypertension and hemodynamic collapse even after short-term use; In this case, it starts over immediately. The risk of methemoglobinemia increases at doses above 40 ppm; Methemoglobin is measured 4–8 hours after the start and periodically, and inspired NO2 is monitored.
  • Epoprostenol (IV): Sudden discontinuation or large abrupt dose reduction may cause rebound pulmonary hypertension; Spare pump, infusion set and medication are kept ready. Prostanoids may disrupt platelet aggregation and increase bleeding.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.

Valve diseases and prosthetic valves

Mitral stenosis
Severity
Light

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Mild mitral stenosis is mWHO II–III as non-severe native valve disease; Calculate individual risk with CARPREG II.
  3. At mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team from pre-pregnancy throughout pregnancy and puerperium; Individualize monitoring frequency based on lesion type, ventricular function, arrhythmia burden, and symptoms.
  4. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of stenosis, ventricular function and pulmonary hypertension with ECHO.
  5. Monitor fetal growth if maternal cardiac risk is moderate or high; in congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks.
  6. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • In the presence of atrial fibrillation, left atrial thrombus, or previous embolism, administer full therapeutic dose anticoagulation: usually LMWH (enoxaparin based on body weight, usually 1 mg/kg SC every 12 hours) or VKA.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Medium
Period
Before pregnancy

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Moderate mitral stenosis is mWHO III; Calculate individual risk with CARPREG II.
  3. Identify before pregnancy and evaluate with the Pregnancy Heart Team; Perform the evaluation with a cardiologist experienced in pregnancy and valve disease: detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation.
  4. Determine the severity of stenosis, ventricular function, and pulmonary hypertension by ECHO; Objectively evaluate functional capacity with exercise testing.
  5. Consider preconception intervention or advice to avoid pregnancy in symptomatic and selected asymptomatic patients; If intervention is necessary, complete it before pregnancy.
  6. Explain to the woman referred for intervention the risks and benefits of each option: if replacement is required, weigh the fetal risks of thromboembolism and anticoagulation in pregnancy with a mechanical valve, limited durability of the bioprosthesis, and the need for reintervention; If appropriate, choose repair over replacement.
  7. Advise women with moderate valve disease remaining after repair not to delay pregnancy.
  8. For congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks of pregnancy.
  9. Provide effective contraception until intervention or optimization is completed; Choose long-acting reversible methods.

Treatment

  • If the mitral valve area is < 1.5 cm², perform valve intervention before pregnancy, even if there are no symptoms; The first option is percutaneous mitral balloon commissurotomy in appropriate anatomy.

Note

  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
Pregnancy, birth and puerperium

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Moderate mitral stenosis is mWHO III; Calculate individual risk with CARPREG II.
  3. Manage at a specialist center with a Pregnancy Heart Team; Have your cardiology check-up usually every 1-2 months and create your birth plan early.
  4. Perform a comprehensive risk assessment at the first antenatal visit in a woman who has not received pre-pregnancy counseling, with detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation; Monitor for atrial fibrillation and pulmonary edema during pregnancy.
  5. Consider anticoagulation in cases of significant stenosis, spontaneous echo contrast in the left atrium, left atrial volume index > 60 mL/m², or heart failure.
  6. Monitor fetal growth; in congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks.
  7. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  8. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  9. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  10. Determine frequency of postpartum check-ups based on lesion and symptoms (hemodynamic changes of pregnancy may take up to 6 months to resolve); Choose long-acting reversible methods of contraception (intrauterine device, progestin implant) and individualize the choice according to the lesion, anticoagulation status and bleeding profile.

Treatment

  • In symptomatic mitral stenosis or pulmonary hypertension, restrict physical activity and initiate beta blockers — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If congestive symptoms persist despite beta blocker — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In the presence of atrial fibrillation, left atrial thrombus, or previous embolism, administer full therapeutic dose anticoagulation: usually LMWH (enoxaparin based on body weight, usually 1 mg/kg SC every 12 hours) or VKA.
  • If NYHA III–IV symptoms or systolic pulmonary artery pressure > 50 mmHg persist despite beta blockers and diuretics, perform percutaneous mitral balloon commissurotomy in appropriate anatomy (preferably after the 20th week).
  • Only perform open valve surgery if the mother's life is in danger and other treatment methods cannot be applied or have failed.
  • If cardiopulmonary bypass is mandatory, apply high flow (2.5 L/min/m²) and mean arterial pressure greater than 70 mmHg.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Avoid atenolol as a beta blocker.
  • Consider fetal radiation exposure and risk of maternal and fetal complications during valve intervention during pregnancy; Rewarming after deep hypothermia in cardiopulmonary bypass may trigger preterm labor.
  • Left atrial thrombus or more than mild mitral regurgitation is a contraindication for percutaneous balloon commissurotomy.
  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Heavy
Period
Before pregnancy

Approach

  1. Severe mitral stenosis is mWHO IV; Pregnancy is not recommended. Write in the record that pregnancy is contraindicated, the lesion-specific rationale, and the patient's understanding and preferences.
  2. Identify before pregnancy and evaluate with the Pregnancy Heart Team; Perform the evaluation with a cardiologist experienced in pregnancy and valve disease: detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation.
  3. Determine the severity of stenosis, valve morphology, ventricular function and pulmonary hypertension by ECHO.
  4. Explain to the woman referred for intervention the risks and benefits of each option: if replacement is required, weigh the fetal risks of thromboembolism and anticoagulation in pregnancy with a mechanical valve, limited durability of the bioprosthesis, and the need for reintervention; If appropriate, choose repair over replacement.
  5. Advise women with moderate valve disease remaining after repair not to delay pregnancy.
  6. For congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks of pregnancy.
  7. Provide effective contraception until intervention or optimization is completed; Choose long-acting reversible methods.

Treatment

  • If the valve morphology is suitable, perform percutaneous mitral balloon valvotomy before pregnancy.
  • If the morphology is not suitable for balloon valvotomy, complete the necessary valve intervention before pregnancy.

Note

  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
Pregnancy, birth and puerperium

Approach

  1. Severe mitral stenosis is mWHO IV; If pregnancy has occurred, discuss the maternal risk, continuation in a specialist center, and the option of termination if legally and ethically appropriate.
  2. If pregnancy persists, concentrate care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care and neonatal facilities.
  3. Perform a comprehensive risk assessment at the first antenatal visit in a woman who has not received pre-pregnancy counseling, with detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation; Monitor for atrial fibrillation and pulmonary edema during pregnancy.
  4. Consider anticoagulation in cases of significant stenosis, spontaneous echo contrast in the left atrium, left atrial volume index > 60 mL/m², or heart failure.
  5. Monitor fetal growth; in congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks.
  6. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  7. Vaginal birth is the general preference; Cesarean section is preferred in severe mitral stenosis and resistant heart failure.
  8. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  9. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  10. Prefer long-acting reversible methods of contraception (intrauterine device, progestin implant); Individualize selection based on lesion, anticoagulation status, and bleeding profile.

Treatment

  • In symptomatic mitral stenosis or pulmonary hypertension, restrict physical activity and initiate beta blockers — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If congestive symptoms persist despite beta blocker — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In the presence of atrial fibrillation, left atrial thrombus, or previous embolism, administer full therapeutic dose anticoagulation: usually LMWH (enoxaparin based on body weight, usually 1 mg/kg SC every 12 hours) or VKA.
  • If NYHA III–IV symptoms or systolic pulmonary artery pressure > 50 mmHg persist despite beta blockers and diuretics, perform percutaneous mitral balloon commissurotomy in appropriate anatomy (preferably after the 20th week).
  • Only perform open valve surgery if the mother's life is in danger and other treatment methods cannot be applied or have failed.
  • If cardiopulmonary bypass is mandatory, apply high flow (2.5 L/min/m²) and mean arterial pressure greater than 70 mmHg.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Avoid atenolol as a beta blocker.
  • Consider fetal radiation exposure and risk of maternal and fetal complications during valve intervention during pregnancy; Rewarming after deep hypothermia in cardiopulmonary bypass may trigger preterm labor.
  • Left atrial thrombus or more than mild mitral regurgitation is a contraindication for percutaneous balloon commissurotomy.
  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Aortic stenosis
Severity and symptom
Light–medium

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Mild to moderate aortic stenosis is generally well tolerated during pregnancy; mWHO II–III as non-severe native valve disease; Calculate individual risk with CARPREG II.
  3. If symptoms are present, reassess the severity of the stenosis and other potential causes of the symptoms; if severe aortic stenosis is confirmed, switch to the severe aortic stenosis option.
  4. At mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team from pre-pregnancy throughout pregnancy and puerperium; Individualize monitoring frequency based on lesion type, ventricular function, arrhythmia burden, and symptoms.
  5. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of stenosis, ventricular function and pulmonary hypertension with ECHO.
  6. In bicuspid aortic valve, evaluate the aortic diameter regardless of the severity of stenosis; If the diameter reaches 50 mm, recommend pre-pregnancy aortic surgery (45 mm if cardiac surgery is to be performed for another reason).
  7. Monitor fetal growth if maternal cardiac risk is moderate or high; in a congenital etiology (bicuspid aortic valve), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  8. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  9. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • If symptoms develop during pregnancy, limit physical activity as the first step.
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Severe, asymptomatic

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Explain in counseling that severe aortic stenosis is not well tolerated during pregnancy and that heart failure and arrhythmia are concentrated in severe disease; Calculate individual risk with CARPREG II (high-risk left heart valve disease or left ventricular outflow tract obstruction 2 points) and assess and manage with the Pregnancy Heart Team.
  3. Assess symptom status, valve gradient, ventricular function, and concomitant aortopathy before pregnancy; Determine functional capacity, exercise-induced arrhythmia, ischemia, abnormal blood pressure response, and limited cardiac reserve with exercise testing.
  4. In bicuspid aortic valve, evaluate the aortic diameter regardless of the severity of stenosis; If the diameter reaches 50 mm, recommend pre-pregnancy aortic surgery (45 mm if cardiac surgery is to be performed for another reason).
  5. Monitor fetal growth; in a congenital etiology (bicuspid aortic valve), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  6. Plan the birth at a reference center with neonatal intensive care facilities; prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and perform continuous telemetry during labor; Intraarterial pressure monitoring may be useful.
  7. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva; Evaluate as a team that some centers advocate planned cesarean section in severe aortic stenosis.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • Perform valve intervention before pregnancy if there is left ventricular systolic dysfunction or symptoms, exercise limitation, or abnormal blood pressure response on exercise testing; In other asymptomatic severe stenosis, consider pre-pregnancy intervention after discussing the risks and benefits.
  • If symptoms develop during pregnancy, limit physical activity as the first step.
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Perform valvuloplasty, percutaneous transcatheter valve implantation, or valve replacement during pregnancy for symptoms resistant to drug therapy.
  • If cardiopulmonary bypass is mandatory, apply high flow (2.5 L/min/m²) and mean arterial pressure greater than 70 mmHg.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Consider fetal radiation exposure and risk of maternal and fetal complications during valve intervention during pregnancy; Rewarming after deep hypothermia in cardiopulmonary bypass may trigger preterm labor.
  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Severe, symptomatic
Period
Before pregnancy

Approach

  1. Severe symptomatic aortic stenosis is mWHO IV; Pregnancy is not recommended. Write in the record that pregnancy is contraindicated, the lesion-specific rationale, and the patient's understanding and preferences.
  2. Evaluate with the Pregnancy Heart Team; Perform detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine valve gradient, ventricular function, exercise capacity, and concomitant aortopathy.
  3. Explain to the woman referred for intervention the risks and benefits of each option: if replacement is required, weigh the fetal risks of thromboembolism and anticoagulation in pregnancy with a mechanical valve, limited durability of the bioprosthesis, and the need for reintervention; If appropriate, choose repair over replacement.
  4. In bicuspid aortic valve, evaluate the aortic diameter regardless of the severity of stenosis; If the diameter reaches 50 mm, recommend pre-pregnancy aortic surgery (45 mm if cardiac surgery is to be performed for another reason).
  5. For a congenital etiology (bicuspid aortic valve), discuss the risk of congenital heart disease in the child (approximately 4–10%) and fetal echocardiography at 18–22 weeks of pregnancy.
  6. Provide effective contraception until intervention or optimization is completed; Choose long-acting reversible methods.

Treatment

  • Perform valve intervention before pregnancy.

Note

  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
Pregnancy, birth and puerperium

Approach

  1. Severe symptomatic aortic stenosis is mWHO IV; If pregnancy has occurred, discuss the maternal risk, continuation in a specialist center, and the option of termination if legally and ethically appropriate.
  2. If pregnancy persists, concentrate care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care and neonatal facilities.
  3. Perform a comprehensive risk assessment at the first antenatal visit in a woman who has not received pre-pregnancy counseling, with detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation; Monitor for heart failure and arrhythmia during pregnancy.
  4. Monitor aortic diameter in bicuspid aortic valve throughout pregnancy; If the ascending aorta is > 50 mm, the risk is at mWHO IV level.
  5. Monitor fetal growth; in a congenital etiology (bicuspid aortic valve), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  6. Plan the birth at a reference center with neonatal intensive care facilities; prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and perform continuous telemetry during labor; Intraarterial pressure monitoring may be useful.
  7. Plan delivery by minimizing Valsalva with appropriate analgesia; Consider cesarean section in severe symptomatic aortic stenosis and determine the mode of delivery according to the hemodynamic status and obstetric conditions with the Pregnancy Heart Team.
  8. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  9. Determine frequency of postpartum check-ups based on lesion and symptoms (hemodynamic changes of pregnancy may take up to 6 months to resolve); Choose long-acting reversible methods of contraception (intrauterine device, progestin implant) and individualize the choice according to the lesion, anticoagulation status and bleeding profile.

Treatment

  • If symptoms develop during pregnancy, limit physical activity as the first step.
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Perform valvuloplasty, percutaneous transcatheter valve implantation, or valve replacement during pregnancy for symptoms resistant to drug therapy.
  • If cardiopulmonary bypass is mandatory, apply high flow (2.5 L/min/m²) and mean arterial pressure greater than 70 mmHg.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Consider fetal radiation exposure and risk of maternal and fetal complications during valve intervention during pregnancy; Rewarming after deep hypothermia in cardiopulmonary bypass may trigger preterm labor.
  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
Mitral regurgitation
Severity, symptoms and LV function
Light

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Mild mitral regurgitation is well tolerated in pregnancy; determine risk class: mitral valve prolapse is mWHO I, other non-severe native valve disease is mWHO II–III.
  3. In mWHO I, routine obstetric care and cardiology evaluation 1–2 times throughout pregnancy may be sufficient; At mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team, starting from pre-pregnancy and throughout pregnancy and puerperium, and individualize the frequency of monitoring.
  4. Before pregnancy (or, if not done before pregnancy, at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation, ventricular dilatation and dysfunction, and pulmonary hypertension with ECHO.
  5. Monitor fetal growth if maternal cardiac risk is moderate or high; in a congenital etiology (atrioventricular canal defect), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  6. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  8. No specific drug treatment is required.

Treatment

  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Medium–heavy; asymptomatic, no LV systolic dysfunction and pulmonary hypertension

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Moderate to severe regurgitation is usually tolerated in the absence of LV systolic dysfunction and pulmonary hypertension; However, explain in counseling that heart failure was reported in 23% of these pregnancies.
  3. Classify risk using mWHO and CARPREG II (moderate regurgitation is mWHO II–III); evaluate and manage with the Pregnancy Heart Team.
  4. Before pregnancy (or, if not done before pregnancy, at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation, ventricular dilatation and dysfunction, and pulmonary hypertension with ECHO; Monitor for signs of heart failure during pregnancy.
  5. Monitor fetal growth; in a congenital etiology (atrioventricular canal defect), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  6. Plan the birth at the reference center; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  8. Prefer long-acting reversible methods of contraception (intrauterine device, progestin implant); Individualize selection based on lesion, anticoagulation status, and bleeding profile.
  9. No specific drug treatment is required.

Treatment

  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Moderate–severe; symptoms, LV systolic dysfunction, or pulmonary hypertension are present; LVEF ≥ 30%; No significant pulmonary hypertension in severe regurgitation
Period
Before pregnancy

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Classify risk with mWHO and CARPREG II; Evaluate with the Pregnancy Heart Team; Perform the evaluation with a cardiologist experienced in pregnancy and valve disease: detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation.
  3. Determine the severity of failure, ventricular dilatation and dysfunction, and pulmonary hypertension by ECHO; Objectively evaluate functional capacity with exercise testing.
  4. Explain to the woman referred for intervention the risks and benefits of each option: if replacement is required, weigh the fetal risks of thromboembolism and anticoagulation in pregnancy with a mechanical valve, limited durability of the bioprosthesis, and the need for reintervention; If appropriate, choose repair over replacement.
  5. Advise women with moderate valve disease remaining after repair not to delay pregnancy.
  6. For a congenital etiology (atrioventricular canal defect), discuss the risk of congenital heart disease in the child (approximately 4–10%) and fetal echocardiography at 18–22 weeks of pregnancy.
  7. Provide effective contraception until intervention or optimization is completed; Choose long-acting reversible methods.

Treatment

  • Perform surgery before pregnancy if there are symptoms of severe mitral regurgitation, ventricular dysfunction, or significant ventricular dilatation; If appropriate, choose repair over replacement.

Note

  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
Pregnancy, birth and puerperium

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Classify risk with mWHO and CARPREG II; Evaluate and manage with the Pregnancy Heart Team.
  3. Perform a comprehensive risk assessment at the first antenatal visit in a woman who has not received pre-pregnancy counseling, with detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation; Assess regurgitation, LV function, and pulmonary pressure by echocardiography and monitor for signs of heart failure.
  4. Monitor fetal growth; in a congenital etiology (atrioventricular canal defect), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  5. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  6. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  9. Prefer long-acting reversible methods of contraception (intrauterine device, progestin implant); Individualize selection based on lesion, anticoagulation status, and bleeding profile.

Treatment

  • If there are signs or symptoms of congestion — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • Only perform valve surgery during pregnancy if the mother's life is in danger and other treatment methods, including medication, have failed.
  • If cardiopulmonary bypass is mandatory, apply high flow (2.5 L/min/m²) and mean arterial pressure greater than 70 mmHg.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Consider fetal radiation exposure and risk of maternal and fetal complications during valve intervention during pregnancy; Rewarming after deep hypothermia in cardiopulmonary bypass may trigger preterm labor.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Heavy; LVEF < 30% or significant pulmonary hypertension

Approach

  1. Recommend avoiding pregnancy; LVEF < 30% mWHO IV.
  2. Evaluate with the Pregnancy Heart Team; Write in the record that pregnancy is contraindicated, the lesion-specific rationale, and the patient's understanding and preferences.
  3. Provide effective contraception; prefer long-acting reversible methods, avoid combined hormonal methods containing estrogen in severe ventricular dysfunction and pulmonary hypertension; Consider permanent contraception in a woman who has completed her fertility by mutual decision, without coercion, and evaluate the risk of intervention and anesthesia in advance.
  4. If pregnancy occurs in a woman with LVEF < 30%, discuss the maternal risk, continuation in a specialist center, and the option of termination if legally and ethically appropriate, at an early stage; If pregnancy continues, concentrate care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care and neonatal facilities.
  5. If pregnancy persists, monitor fetal growth; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  6. Individualize mode of delivery according to cardiac status; Plan vaginal delivery with analgesia and minimizing Valsalva in the appropriate patient.
  7. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  8. Organize postpartum checks according to lesion and symptoms; It may take up to 6 months for hemodynamic changes to resolve.
  9. If there is significantly low LVEF, atrial arrhythmia, or documented ventricular thrombus, evaluate the need for anticoagulation according to the individual risk of thromboembolism and bleeding.

Treatment

  • Perform surgery before pregnancy if there are symptoms of severe mitral regurgitation, ventricular dysfunction, or significant ventricular dilatation; If appropriate, choose repair over replacement.
  • If pregnancy continues, in congestion — Furosemide (oral): 20–40 mg once a day is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • As a vasodilator when necessary in heart failure — Hydralazine + isosorbide dinitrate (oral): hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times a day are started; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Only perform valve surgery during pregnancy if the mother's life is in danger and other treatment methods have failed.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Evaluate the breastfeeding safety of medications in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Aortic regurgitation
Symptom and LV function
Asymptomatic; LV systolic function preserved, no significant ventricular dilatation and pulmonary hypertension

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Asymptomatic women with preserved LV systolic function tolerate pregnancy well; The frequency of maternal cardiac events in moderate to severe regurgitation is 5%.
  3. Classify risk using mWHO and CARPREG II (non-severe regurgitation is mWHO II–III); for mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team starting before pregnancy and continuing throughout pregnancy and the puerperium.
  4. Before pregnancy (if not done, at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation, ventricular dilatation and dysfunction, and pulmonary hypertension with ECHO; Monitor for the development of symptoms, systolic dysfunction, significant ventricular dilatation, and pulmonary hypertension during pregnancy.
  5. In bicuspid aortic valve, evaluate aortic diameter regardless of the severity of regurgitation; If the diameter reaches 50 mm, recommend pre-pregnancy aortic surgery (45 mm if cardiac surgery will be performed for another reason).
  6. Assess aortic root dilatation and risk of dissection in aortic regurgitation due to Marfan syndrome (risk increases with root dilatation); Consider prophylactic beta blockers to limit progression of dilatation.
  7. In Marfan syndrome, recommend pre-pregnancy aortic surgery if the aortic root diameter is > 45 mm; for a diameter of 40–45 mm, consider pre-pregnancy surgery if the growth rate is > 3 mm/year or there is a family history of aortic dissection.
  8. Monitor fetal growth if maternal cardiac risk is moderate or high; in a congenital etiology (bicuspid aortic valve), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  9. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  10. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • If symptoms develop — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • As a vasodilator when necessary in the symptomatic patient — Hydralazine + isosorbide dinitrate (oral): hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times a day are started; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Prophylactic beta blocker for aortic regurgitation due to Marfan syndrome — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Symptomatic or LV systolic dysfunction, marked dilatation or pulmonary hypertension (LVEF ≥ 30%)

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Explain in counseling that complications are concentrated in symptomatic, severe regurgitation with systolic dysfunction, marked ventricular dilatation, or pulmonary hypertension; calculate individual risk with CARPREG II (initial NYHA III–IV 3, ventricular dysfunction 2, pulmonary hypertension 2 points) and assess and manage with the Pregnancy Heart Team.
  3. Before pregnancy (or, if not done before pregnancy, at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation, ventricular dilatation and dysfunction, and pulmonary hypertension with ECHO.
  4. In bicuspid aortic valve, evaluate aortic diameter regardless of the severity of regurgitation; If the diameter reaches 50 mm, recommend pre-pregnancy aortic surgery (45 mm if cardiac surgery will be performed for another reason).
  5. Assess aortic root dilatation and risk of dissection in aortic regurgitation due to Marfan syndrome (risk increases with root dilatation); Consider prophylactic beta blockers to limit progression of dilatation.
  6. In Marfan syndrome, recommend pre-pregnancy aortic surgery if the aortic root diameter is > 45 mm; for a diameter of 40–45 mm, consider pre-pregnancy surgery if the growth rate is > 3 mm/year or there is a family history of aortic dissection.
  7. Monitor fetal growth; in a congenital etiology (bicuspid aortic valve), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  8. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  9. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  10. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  11. Determine frequency of postpartum check-ups based on lesion and symptoms (hemodynamic changes of pregnancy may take up to 6 months to resolve); Choose long-acting reversible methods of contraception (intrauterine device, progestin implant) and individualize the choice according to the lesion, anticoagulation status and bleeding profile.

Treatment

  • If there are symptoms of severe aortic regurgitation, ventricular dysfunction, or significant ventricular dilatation, perform valve surgery before pregnancy.
  • In symptomatic patient — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • As a vasodilator when necessary in the symptomatic patient — Hydralazine + isosorbide dinitrate (oral): hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times a day are started; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Prophylactic beta blocker for aortic regurgitation due to Marfan syndrome — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Only perform valve surgery during pregnancy if the mother's life is in danger and other treatment methods, including medication, have failed.
  • If cardiopulmonary bypass is mandatory, apply high flow (2.5 L/min/m²) and mean arterial pressure greater than 70 mmHg.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Consider fetal radiation exposure and risk of maternal and fetal complications during valve intervention during pregnancy; Rewarming after deep hypothermia in cardiopulmonary bypass may trigger preterm labor.
  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Severe disability and LVEF < 30%

Approach

  1. Recommend avoiding pregnancy; LVEF < 30% mWHO IV. Evaluate with the Pregnancy Heart Team; Write in the record that pregnancy is contraindicated, the lesion-specific rationale, and the patient's understanding and preferences.
  2. Provide effective contraception; prefer long-acting reversible methods, avoid combined hormonal methods containing estrogen in significant ventricular dysfunction; Consider permanent contraception in a woman who has completed her fertility by mutual decision, without coercion, and evaluate the risk of intervention and anesthesia in advance.
  3. In bicuspid aortic valve, evaluate aortic diameter regardless of the severity of regurgitation; If the diameter reaches 50 mm, recommend pre-pregnancy aortic surgery (45 mm if cardiac surgery will be performed for another reason).
  4. Assess aortic root dilatation and risk of dissection in aortic regurgitation due to Marfan syndrome (risk increases with root dilatation); Consider prophylactic beta blockers to limit progression of dilatation.
  5. In Marfan syndrome, recommend pre-pregnancy aortic surgery if the aortic root diameter is > 45 mm; for a diameter of 40–45 mm, consider pre-pregnancy surgery if the growth rate is > 3 mm/year or there is a family history of aortic dissection.
  6. If pregnancy is detected early, recommend termination and corrective surgery before trying a new pregnancy; If the patient refuses termination, concentrate care at a tertiary or quaternary center with advanced cardiac, obstetric, anesthesia, intensive care and neonatal facilities.
  7. If pregnancy persists, monitor fetal growth; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  8. Individualize mode of delivery according to cardiac status; Plan vaginal delivery with analgesia and minimizing Valsalva in the appropriate patient.
  9. Consider monitoring women at highest risk for heart failure or who develop heart failure during pregnancy or birth in intensive (cardiac) care for hemodynamic monitoring for the first 24–48 hours postpartum.
  10. Organize postpartum checks according to lesion and symptoms; It may take up to 6 months for hemodynamic changes to resolve.
  11. If there is significantly low LVEF, atrial arrhythmia, or documented ventricular thrombus, evaluate the need for anticoagulation according to the individual risk of thromboembolism and bleeding.

Treatment

  • After termination of early pregnancy, perform corrective valve surgery before attempting a new pregnancy.
  • In symptomatic patient — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • As a vasodilator when necessary in the symptomatic patient — Hydralazine + isosorbide dinitrate (oral): hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times a day are started; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Prophylactic beta blocker for aortic regurgitation due to Marfan syndrome — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Only perform valve surgery during pregnancy if the mother's life is in danger and other treatment methods have failed.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Evaluate the breastfeeding safety of medications in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Pulmonary stenosis
Severity and right ventricular function
Mild, uncomplicated (including those with little residual stenosis after repair or balloon)

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Mild pulmonary stenosis without complications is mWHO I; pregnancy is generally safe; A normal pregnancy is expected in a woman with minimal stenosis and good valve function after surgical repair or balloon dilation.
  3. Perform routine obstetric care; Cardiology evaluation 1–2 times during pregnancy may be sufficient.
  4. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease.
  5. In congenital stenosis, plan fetal echocardiography at 18–22 weeks; the child's risk of congenital heart disease is approximately 4–10%.
  6. As a low-risk lesion, plan delivery at the local center; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  8. No specific drug therapy is required; specific intervention is rarely needed.

Treatment

  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Note that outcomes are worse in cases of stenosis accompanying complex congenital lesions.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
Normal right ventricular systolic function in moderate stenosis or asymptomatic and normal right ventricular systolic function in severe stenosis

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Moderate to severe isolated pulmonary stenosis is generally well tolerated in the absence of right ventricular dysfunction; Classify the risk with mWHO, CARPREG II and ZAHARA in congenital disease (moderate stenosis mWHO II–III).
  3. At mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team from pre-pregnancy throughout pregnancy and puerperium; Individualize monitoring frequency.
  4. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of stenosis and right ventricular function by ECHO; Monitor for signs of right heart failure during pregnancy.
  5. Monitor fetal growth if maternal cardiac risk is moderate or high; in congenital stenosis, plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  6. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Consider cesarean section for severe pulmonary valve or right ventricular outflow tract stenosis; Determine the type of birth with the Pregnancy Heart Team.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  9. No specific drug treatment is required.

Treatment

  • If there is severe right ventricular outflow tract stenosis with Doppler peak gradient > 64 mmHg or evidence of right heart failure before pregnancy, perform the intervention before pregnancy; Prefer balloon valvotomy with appropriate stenosis at the valve level.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Note that outcomes are worse in cases of stenosis accompanying complex congenital lesions.
  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Symptomatic severe stenosis or right ventricular systolic dysfunction regardless of the severity of stenosis

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Symptomatic right heart failure occurs mainly in severe stenosis accompanied by right ventricular systolic dysfunction; calculate individual risk with CARPREG II and ZAHARA in congenital disease; The ventricular dysfunction score (2) in CARPREG II is for left ventricular ejection fraction < 55%, further consider isolated right ventricular dysfunction as lesion-specific risk; Evaluate and manage with the Pregnancy Heart Team.
  3. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of stenosis and right ventricular function with ECHO.
  4. Monitor fetal growth; in congenital stenosis, plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  5. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  6. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Consider cesarean section for severe pulmonary valve or right ventricular outflow tract stenosis; Determine the type of birth with the Pregnancy Heart Team.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • If there is severe right ventricular outflow tract stenosis with Doppler peak gradient > 64 mmHg or evidence of right heart failure before pregnancy, perform the intervention before pregnancy; Prefer balloon valvotomy with appropriate stenosis at the valve level.
  • If there is evidence of systemic venous congestion or right heart failure — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • Consider percutaneous balloon valvotomy for symptomatic severe pulmonary stenosis that does not respond to bed rest and conservative treatment during pregnancy.
  • Perform valve surgery or catheter intervention during pregnancy for symptoms resistant to drug therapy.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Note that outcomes are worse in cases of stenosis accompanying complex congenital lesions.
  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Consider fetal radiation exposure and the risk of maternal and fetal complications during valve intervention during pregnancy.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Pulmonary regurgitation
Right ventricular status
No right ventricular dilatation and systolic dysfunction

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Review previous intervention records (regurgitation often accompanies repaired tetralogy of Fallot or previous pulmonary stenosis valvuloplasty); Classify the risk with mWHO, CARPREG II and ZAHARA in congenital disease.
  3. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation and the size and function of both ventricles by ECHO; Monitor for right heart failure and atrial and ventricular tachyarrhythmias during pregnancy.
  4. Monitor fetal growth if maternal cardiac risk is moderate or high; in a congenital etiology (repaired tetralogy of Fallot or pulmonary stenosis), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  5. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  6. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  7. No specific drug treatment is required in an asymptomatic patient.

Treatment

  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
There is right ventricular dilatation or systolic dysfunction

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Explain in counseling that severe regurgitation accompanied by right ventricular dysfunction predicts pregnancy complications; calculate individual risk with CARPREG II and ZAHARA in congenital disease; The ventricular dysfunction score (2) in CARPREG II is for left ventricular ejection fraction < 55%, further consider isolated right ventricular dysfunction as lesion-specific risk; Evaluate and manage with the Pregnancy Heart Team.
  3. Review records of prior interventions; regurgitation frequently accompanies repaired tetralogy of Fallot or prior valvuloplasty for pulmonary stenosis.
  4. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation and the size and function of both ventricles by ECHO; Monitor for right heart failure and atrial and ventricular tachyarrhythmias during pregnancy.
  5. Monitor fetal growth; in a congenital etiology (repaired tetralogy of Fallot or pulmonary stenosis), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  6. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  7. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • If there is evidence of systemic venous congestion or right heart failure — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • Consider preconception repair of severe regurgitation in repaired tetralogy of Fallot.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Tricuspid regurgitation and Ebstein anomaly
Severity
Light

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Mild tricuspid regurgitation is well tolerated; mWHO II–III as non-severe native valve disease.
  3. At mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team from pre-pregnancy throughout pregnancy and puerperium; Individualize monitoring frequency based on lesion type, ventricular function, arrhythmia burden, and symptoms.
  4. Before pregnancy (or, if not done before pregnancy, at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation and ventricular function with ECHO.
  5. Evaluate the risk of congenital cause (Ebstein anomaly, atrioventricular canal defect) with ZAHARA; Record baseline oxygen saturation, cyanosis, residual shunts, and rhythm history and determine risk class based on these findings and ventricular function, along with degree of failure.
  6. Monitor fetal growth if maternal cardiac risk is moderate or high; in a congenital etiology (Ebstein anomaly or atrioventricular canal defect), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  7. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  9. No specific drug treatment is required.

Treatment

  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Medium–heavy

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. If ventricular function is normal and there is no history of cardiac event before pregnancy, pregnancy with moderate to severe regurgitation may proceed well; record ventricular dysfunction and pre-pregnancy history of cardiac events (prior cardiac event or arrhythmia in CARPREG II 3 points; ventricular dysfunction score (2) for left ventricular ejection fraction < 55%, separately consider isolated right ventricular dysfunction as lesion-specific risk).
  3. Add ZAHARA score for congenital cause (Ebstein anomaly, atrioventricular canal defect) (moderate–severe subpulmonary atrioventricular valve regurgitation is ZAHARA variable); Record baseline oxygen saturation, cyanosis, residual shunts, and rhythm history and determine risk class based on these findings and ventricular function, along with degree of failure.
  4. Classify risk using mWHO (moderate regurgitation is mWHO II–III); for mWHO II–III or higher risk, conduct evaluation and management with the Pregnancy Heart Team starting before pregnancy and continuing throughout pregnancy and the puerperium.
  5. Before pregnancy (or, if not done before pregnancy, at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of regurgitation and ventricular function with ECHO.
  6. Monitor fetal growth; in a congenital etiology (Ebstein anomaly or atrioventricular canal defect), plan fetal echocardiography at 18–22 weeks (the risk of congenital heart disease in the child is approximately 4–10%).
  7. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  9. No specific drug treatment is required in an asymptomatic patient.

Treatment

  • If there is evidence of systemic venous congestion or right heart failure — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Insufficiency may not change, increase or decrease during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Tricuspid stenosis
Clinical situation
No refractory systemic venous congestion

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Look for accompanying mitral and aortic valve involvement in rheumatic tricuspid involvement.
  3. Note that symptoms may first appear during pregnancy as cardiac output increases; Monitor for systemic venous congestion.
  4. Classify risk with mWHO and CARPREG II; Determine the frequency of monitoring and the need for a special cardio-obstetric center according to risk class.
  5. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of stenosis and accompanying valve lesions with ECHO.
  6. Monitor fetal growth if maternal cardiac risk is moderate or high; in congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks.
  7. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  9. No specific drug treatment is required in an asymptomatic patient.

Treatment

  • If there is evidence of systemic venous congestion or right heart failure — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Refractory systemic venous congestion

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Look for accompanying mitral and aortic valve involvement in rheumatic tricuspid involvement.
  3. Classify risk with mWHO and CARPREG II; Evaluate and manage with the Pregnancy Heart Team.
  4. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of stenosis and accompanying valve lesions with ECHO.
  5. Monitor fetal growth; in congenital stenosis, address the fetal recurrence risk during counseling and plan fetal echocardiography at 18–22 weeks.
  6. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team, perform prenatal obstetric anesthesia evaluation and apply continuous telemetry during labor.
  7. Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • If there is evidence of systemic venous congestion or right heart failure — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • Balloon valvotomy: evaluate by a specialist team in pregnancy in resistant systemic venous congestion.
  • In cases of accompanying mitral involvement, isolated tricuspid or simultaneous mitral–tricuspid valvotomy can be performed.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Stenosis gradients increase during pregnancy; Interpret ECHO findings accordingly.
  • Experience with tricuspid balloon valvotomy in pregnancy is limited but appears effective.
  • Consider fetal radiation exposure and risk of maternal and fetal complications during catheterization.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Multiple valve lesion
Status
Multiple valve lesions

Approach

  1. Apply the mWHO IV approach if LVEF < 30% or NYHA III–IV heart failure, regardless of the severity of the valve lesion: recommend avoiding pregnancy; If pregnancy has occurred, discuss the option of terminating it if it is legally and ethically appropriate.
  2. Identify the hemodynamically dominant lesion; The dominant lesion determines risk and treatment; Apply principles other than pregnancy in management.
  3. Explain in counseling that the frequency of maternal cardiac events is high (27%) in moderate to severe multiple regurgitant lesions.
  4. Classify risk with mWHO and CARPREG II; Determine the frequency of monitoring and the need for a special cardio-obstetric center according to risk class.
  5. Before pregnancy (if not done at the first antenatal visit), perform a detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation with a cardiologist experienced in pregnancy and valve disease; Determine the severity of each lesion, ventricular function, and pulmonary hypertension by ECHO.
  6. Monitor fetal growth if moderate or high maternal cardiac risk.
  7. Plan delivery at the local center for low-risk lesions, at the reference center for medium-high-risk disease; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.

Treatment

  • Identify dominant lesion; Apply the treatment with the relevant selection in the algorithm according to the severity of that lesion (mitral stenosis, aortic stenosis, mitral or aortic regurgitation) and the pregnancy period.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.

Note

  • Stenosis gradients increase during pregnancy, regurgitation may vary; Interpret ECHO findings accordingly.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs; Captopril, enalapril and benazepril can be used in breastfeeding after birth, ARB is not recommended.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal delivery; some clinicians give it to women with previous infective endocarditis.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Mechanical prosthetic valve
Pregnancy period
Pre-conception (planning)

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Evaluate with the Pregnancy Heart Team before pregnancy; Plan multidisciplinary follow-up with specialists experienced in heart disease during pregnancy; Evaluate valve function, ventricular systolic function, and pulmonary artery pressure by ECHO.
  3. Consider valve type and position, previous valve thrombosis, anticoagulation history, daily VKA (warfarin) dose, feasibility of anti-Xa monitoring if LMWH is used, and the patient's informed choice; Old type valve (ball-cage, single curved disc), mitral position, multiple mechanical valves, and previous arrhythmia or thromboembolism increase the risk of valve thrombosis.
  4. Explain in counseling that valve thrombosis may lead to valve obstruction or systemic thromboembolism.
  5. VKA (warfarin) is the most effective drug against valve thrombosis; However, especially in doses above 5 mg/day in the first trimester, it increases the risk of fetal anomalies and is associated with late fetal loss.
  6. LMWH has a lower fetal risk but a higher risk of maternal mortality and thromboembolism; Insufficient dose increases the risk of valve thrombosis and requires close anti-Xa monitoring.
  7. Write individual anticoagulation plan before pregnancy or as soon as pregnancy is confirmed: continue VKA in the 1st trimester or switch to therapeutic dose LMWH.
  8. Avoid combined hormonal methods containing estrogen for contraception; Select intrauterine device or progestin implant based on anticoagulation status and bleeding profile.

Treatment

  • In women with regular cycles and planning pregnancy, continue warfarin until pregnancy is confirmed.
  • Warfarin: target INR (accepted range) — 2.5 (2.0–3.0) if there are no additional prothrombotic factors with bileaflet or newer-generation tilting-disc aortic valves, and 3.0 (2.5–3.5) if present; 3.0 (2.5–3.5) if there are no additional prothrombotic factors with any mechanical valve in the mitral or tricuspid position or any caged-ball or older-generation disc valve in any position, and 3.5 (3.0–4.0) if present; during pregnancy, check INR weekly, or at least every 2 weeks.
  • During pregnancy, measure INR weekly, at least every 2 weeks.

Note

  • The risk of warfarin embryopathy is low in the first 5 weeks.
  • Do not use DOACs; they are not recommended during pregnancy.
  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
  • Warfarin: Additional prothrombotic factors: hereditary or acquired hypercoagulability, LVEF <35%, AF with significant mitral stenosis, major thrombotic event in the last 12 months (cardioembolic stroke, DVT, PE); The highest target is applied in multiple mechanical valves.
1st trimester
Warfarin dose and valve thrombosis risk factor
Warfarin ≤ 5 mg/day; There is a risk factor for valve thrombosis (mechanical mitral/tricuspid valve, AF/flutter, previous thromboembolism, multiple mechanical valves, old type valve, previous arrhythmia)

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Manage at a specialist center with a Pregnancy Heart Team; Have your cardiology check-up usually every 1-2 months and create your birth plan early.
  3. If pre-pregnancy evaluation was not performed, perform detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation at the first antenatal visit; Determine valve function, ventricular function, and pulmonary pressure.
  4. Perform ECHO every trimester to evaluate valve function and exclude valve thrombosis; A greater than expected increase in gradient, new heart failure, embolic event, syncope, change in valve click, or new murmur requires immediate evaluation.
  5. Finalize the anticoagulation plan with the Pregnancy Heart Team as soon as pregnancy is confirmed; Make the choice based on the balance of valve thrombosis and fetal risk and the patient's informed preference.
  6. VKA (warfarin) is the most effective drug against valve thrombosis; However, especially in doses above 5 mg/day in the first trimester, it increases the risk of fetal anomalies and is associated with late fetal loss.
  7. Explain that early VKA exposure may cause embryopathy and that later exposure may cause fetal bleeding, including intracranial hemorrhage.
  8. LMWH has a lower fetal risk but a higher risk of maternal mortality and thromboembolism; Insufficient dose increases the risk of valve thrombosis and requires close anti-Xa monitoring.
  9. Monitor fetal growth.
  10. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team.

Treatment

  • Continue VKA with INR monitoring (weekly or at least every 2 weeks).
  • Warfarin: target INR (accepted range) — 2.5 (2.0–3.0) if there are no additional prothrombotic factors with bileaflet or newer-generation tilting-disc aortic valves, and 3.0 (2.5–3.5) if present; 3.0 (2.5–3.5) if there are no additional prothrombotic factors with any mechanical valve in the mitral or tricuspid position or any caged-ball or older-generation disc valve in any position, and 3.5 (3.0–4.0) if present; during pregnancy, check INR weekly, or at least every 2 weeks.
  • In women who prioritize fetal risk, dose-adjusted therapeutic LMWH twice daily during the 1st trimester (through week 12) is a reasonable option.
  • VKA is recommended in the 2nd and 3rd trimesters until the 36th week; In women who prioritize fetal risk, therapeutic dose SC LMWH is a reasonable alternative.
  • When switching from LMWH to VKA, continue LMWH with VKA; Discontinue LMWH after INR has been measured within the therapeutic range for two consecutive days.
  • When switching to LMWH, discontinue VKA and start therapeutic dose LMWH on the same day.
  • Enoxaparin: initially 125 anti‑Xa IU/kg (1.25 mg/kg) SC every 12 hours, then 100 anti‑Xa IU/kg (1 mg/kg) SC every 12 hours.
  • Dalteparin: initially 125 IU/kg SC every 12 hours, then 100 IU/kg SC every 12 hours.
  • Tinzaparin: initially 250 IU/kg SC, then 175 IU/kg SC once daily.
  • Measure the peak anti-Xa level for the first time on day 2 or 3, at 4–6 hours after the dose; individualize target by valve type and location (usually 1.0–1.2 IU/mL; accepted range 0.8–1.2 IU/mL); Also measure the pre-dose (trough) level in selected patients at high risk of thrombosis.
  • If anti-Xa is below target, increase dose by 10–20% and remeasure after 48 hours.
  • INR usually falls below 1.5 on days 5–7; From this point on, anticoagulation is entirely dependent on LMWH, adjust the dose every 3–7 days with anti‑Xa measurement.
  • No fixed time is defined for the transition from the initial dose to the next dose; adjust subsequent doses based on measured peak anti-Xa level.
  • Consider adding low-dose aspirin (75–100 mg/day) at very high thrombotic risk.
  • For routine pregnancy anticoagulation, use UFH only if LMWH or anti‑Xa monitoring is not available and the patient refuses VKA; UFH is advantageous when creatinine clearance is < 30 mL/min or rapid reversal is required for delivery, neuraxial intervention, or emergency surgery.
  • If UFH is used — UFH (IV): in the hospital, start with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading; If switching from LMWH, it starts 12 hours after the last LMWH dose; Adjusted according to aPTT every 6 hours; target aPTT at least 2 times (2–2.5 times) control.

Note

  • Prophylactic dose of LMWH is insufficient for mechanical valve.
  • Use enoxaparin or dalteparin schedule when twice daily SC LMWH regimen is selected; Do not mix the once-daily tinzaparin schedule with this two-dose regimen.
  • Do not use LMWH without anti-Xa monitoring.
  • Do not use DOACs; they are not recommended during pregnancy.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs.
  • Warfarin: Additional prothrombotic factors: hereditary or acquired hypercoagulability, LVEF <35%, AF with significant mitral stenosis, major thrombotic event in the last 12 months (cardioembolic stroke, DVT, PE); The highest target is applied in multiple mechanical valves.
  • UFH (IV): Infusion is stopped 4–6 hours before neuraxial intervention and coagulation is evaluated.
Warfarin ≤ 5 mg/day; no risk factors

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Manage at a specialist center with a Pregnancy Heart Team; Have your cardiology check-up usually every 1-2 months and create your birth plan early.
  3. If pre-pregnancy evaluation was not performed, perform detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation at the first antenatal visit; Determine valve function, ventricular function, and pulmonary pressure.
  4. Perform ECHO every trimester to evaluate valve function and exclude valve thrombosis; A greater than expected increase in gradient, new heart failure, embolic event, syncope, change in valve click, or new murmur requires immediate evaluation.
  5. Finalize the anticoagulation plan with the Pregnancy Heart Team as soon as pregnancy is confirmed; Make the choice based on the balance of valve thrombosis and fetal risk and the patient's informed preference.
  6. VKA (warfarin) is the most effective drug against valve thrombosis; However, especially in doses above 5 mg/day in the first trimester, it increases the risk of fetal anomalies and is associated with late fetal loss.
  7. Explain that early VKA exposure may cause embryopathy and that later exposure may cause fetal bleeding, including intracranial hemorrhage.
  8. LMWH has a lower fetal risk but a higher risk of maternal mortality and thromboembolism; Insufficient dose increases the risk of valve thrombosis and requires close anti-Xa monitoring.
  9. Monitor fetal growth.
  10. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team.

Treatment

  • Continue VKA (warfarin) in the first trimester with INR monitoring, or switch to dose-adjusted SC LMWH during weeks 5–12; make the choice together with the patient.
  • Warfarin: target INR (accepted range) — 2.5 (2.0–3.0) if there are no additional prothrombotic factors with bileaflet or newer-generation tilting-disc aortic valves, and 3.0 (2.5–3.5) if present; 3.0 (2.5–3.5) if there are no additional prothrombotic factors with any mechanical valve in the mitral or tricuspid position or any caged-ball or older-generation disc valve in any position, and 3.5 (3.0–4.0) if present; during pregnancy, check INR weekly, or at least every 2 weeks.
  • During pregnancy, measure INR weekly, at least every 2 weeks.
  • VKA is recommended in the 2nd and 3rd trimesters until the 36th week; In women who prioritize fetal risk, therapeutic dose SC LMWH is a reasonable alternative.
  • When switching from LMWH to VKA, continue LMWH with VKA; Discontinue LMWH after INR has been measured within the therapeutic range for two consecutive days.
  • When switching to LMWH, discontinue VKA and start therapeutic dose LMWH on the same day.
  • Enoxaparin: initially 125 anti‑Xa IU/kg (1.25 mg/kg) SC every 12 hours, then 100 anti‑Xa IU/kg (1 mg/kg) SC every 12 hours.
  • Dalteparin: initially 125 IU/kg SC every 12 hours, then 100 IU/kg SC every 12 hours.
  • Tinzaparin: initially 250 IU/kg SC, then 175 IU/kg SC once daily.
  • Measure the peak anti-Xa level for the first time on day 2 or 3, at 4–6 hours after the dose; individualize target by valve type and location (usually 1.0–1.2 IU/mL; accepted range 0.8–1.2 IU/mL); Also measure the pre-dose (trough) level in selected patients at high risk of thrombosis.
  • If anti-Xa is below target, increase dose by 10–20% and remeasure after 48 hours.
  • INR usually falls below 1.5 on days 5–7; From this point on, anticoagulation is entirely dependent on LMWH, adjust the dose every 3–7 days with anti‑Xa measurement.
  • No fixed time is defined for the transition from the initial dose to the next dose; adjust subsequent doses based on measured peak anti-Xa level.
  • Consider adding low-dose aspirin (75–100 mg/day) at very high thrombotic risk.
  • For routine pregnancy anticoagulation, use UFH only if LMWH or anti‑Xa monitoring is not available and the patient refuses VKA; UFH is advantageous when creatinine clearance is < 30 mL/min or rapid reversal is required for delivery, neuraxial intervention, or emergency surgery.
  • If UFH is used — UFH (IV): in the hospital, start with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading; If switching from LMWH, it starts 12 hours after the last LMWH dose; Adjusted according to aPTT every 6 hours; target aPTT at least 2 times (2–2.5 times) control.

Note

  • Prophylactic dose of LMWH is insufficient for mechanical valve.
  • Use enoxaparin or dalteparin schedule when twice daily SC LMWH regimen is selected; Do not mix the once-daily tinzaparin schedule with this two-dose regimen.
  • Do not use LMWH without anti-Xa monitoring.
  • Do not use DOACs; they are not recommended during pregnancy.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs.
  • Warfarin: Additional prothrombotic factors: hereditary or acquired hypercoagulability, LVEF <35%, AF with significant mitral stenosis, major thrombotic event in the last 12 months (cardioembolic stroke, DVT, PE); The highest target is applied in multiple mechanical valves.
  • UFH (IV): Infusion is stopped 4–6 hours before neuraxial intervention and coagulation is evaluated.
Warfarin > 5 mg/day (regardless of risk factor)

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Manage at a specialist center with a Pregnancy Heart Team; Have your cardiology check-up usually every 1-2 months and create your birth plan early.
  3. If pre-pregnancy evaluation was not performed, perform detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation at the first antenatal visit; Determine valve function, ventricular function, and pulmonary pressure.
  4. Perform ECHO every trimester to evaluate valve function and exclude valve thrombosis; A greater than expected increase in gradient, new heart failure, embolic event, syncope, change in valve click, or new murmur requires immediate evaluation.
  5. Finalize the anticoagulation plan with the Pregnancy Heart Team as soon as pregnancy is confirmed; Make the choice based on the balance of valve thrombosis and fetal risk and the patient's informed preference.
  6. VKA (warfarin) is the most effective drug against valve thrombosis; However, especially in doses above 5 mg/day in the first trimester, it increases the risk of fetal anomalies and is associated with late fetal loss.
  7. Explain that early VKA exposure may cause embryopathy and that later exposure may cause fetal bleeding, including intracranial hemorrhage.
  8. LMWH has a lower fetal risk but a higher risk of maternal mortality and thromboembolism; Insufficient dose increases the risk of valve thrombosis and requires close anti-Xa monitoring.
  9. Monitor fetal growth.
  10. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team.

Treatment

  • Switch to dose-adjusted, twice-daily therapeutic LMWH in the 1st trimester (as soon as pregnancy is confirmed, through week 12); After the 13th week, return to VKA or continuation of LMWH is determined by mutual decision.
  • If a risk factor is present (mechanical mitral/tricuspid valve, AF/flutter, prior thromboembolism, multiple mechanical valves, older-generation valve, prior arrhythmia), consider switching back to a VKA in the 2nd–3rd trimesters until week 36 because of high thrombotic risk.
  • If switching to VKA — Warfarin: target INR (accepted range) — 2.5 (2.0–3.0) if there are no additional prothrombotic factors with bileaflet or newer-generation tilting-disc aortic valves, and 3.0 (2.5–3.5) if present; 3.0 (2.5–3.5) if there are no additional prothrombotic factors with any mechanical valve in the mitral or tricuspid position or any caged-ball or older-generation disc valve in any position, and 3.5 (3.0–4.0) if present; during pregnancy, check INR weekly, or at least every 2 weeks.
  • During pregnancy, measure INR weekly, at least every 2 weeks.
  • When switching from LMWH to VKA, continue LMWH with VKA; Discontinue LMWH after INR has been measured within the therapeutic range for two consecutive days.
  • When switching to LMWH, discontinue VKA and start therapeutic dose LMWH on the same day.
  • Enoxaparin: initially 125 anti‑Xa IU/kg (1.25 mg/kg) SC every 12 hours, then 100 anti‑Xa IU/kg (1 mg/kg) SC every 12 hours.
  • Dalteparin: initially 125 IU/kg SC every 12 hours, then 100 IU/kg SC every 12 hours.
  • Tinzaparin: initially 250 IU/kg SC, then 175 IU/kg SC once daily.
  • Measure the peak anti-Xa level for the first time on day 2 or 3, at 4–6 hours after the dose; individualize target by valve type and location (usually 1.0–1.2 IU/mL; accepted range 0.8–1.2 IU/mL); Also measure the pre-dose (trough) level in selected patients at high risk of thrombosis.
  • If anti-Xa is below target, increase dose by 10–20% and remeasure after 48 hours.
  • INR usually falls below 1.5 on days 5–7; From this point on, anticoagulation is entirely dependent on LMWH, adjust the dose every 3–7 days with anti‑Xa measurement.
  • No fixed time is defined for the transition from the initial dose to the next dose; adjust subsequent doses based on measured peak anti-Xa level.
  • Consider adding low-dose aspirin (75–100 mg/day) at very high thrombotic risk.
  • For routine pregnancy anticoagulation, use UFH only if LMWH or anti‑Xa monitoring is not available and the patient refuses VKA; UFH is advantageous when creatinine clearance is < 30 mL/min or rapid reversal is required for delivery, neuraxial intervention, or emergency surgery.
  • If UFH is used — UFH (IV): in the hospital, start with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading; If switching from LMWH, it starts 12 hours after the last LMWH dose; Adjusted according to aPTT every 6 hours; target aPTT at least 2 times (2–2.5 times) control.

Note

  • Prophylactic dose of LMWH is insufficient for mechanical valve.
  • Use enoxaparin or dalteparin schedule when twice daily SC LMWH regimen is selected; Do not mix the once-daily tinzaparin schedule with this two-dose regimen.
  • Do not use LMWH without anti-Xa monitoring.
  • Do not use DOACs; they are not recommended during pregnancy.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs.
  • Warfarin: Additional prothrombotic factors: hereditary or acquired hypercoagulability, LVEF <35%, AF with significant mitral stenosis, major thrombotic event in the last 12 months (cardioembolic stroke, DVT, PE); The highest target is applied in multiple mechanical valves.
  • UFH (IV): Infusion is stopped 4–6 hours before neuraxial intervention and coagulation is evaluated.
2.–3. trimester (until week 36)

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Manage at a specialist center with a Pregnancy Heart Team; Have your cardiology check-up usually every 1-2 months and create your birth plan early.
  3. If pre-pregnancy evaluation was not performed, perform detailed history, previous valve interventions, symptom status, physical examination, ECG, ECHO and functional evaluation at the first antenatal visit; Determine valve function, ventricular function, and pulmonary pressure.
  4. Perform ECHO every trimester to evaluate valve function and exclude valve thrombosis; A greater than expected increase in gradient, new heart failure, embolic event, syncope, change in valve click, or new murmur requires immediate evaluation.
  5. VKA (warfarin) is the most effective drug against valve thrombosis; Explain the risk of fetal bleeding, including late fetal loss and intracranial hemorrhage.
  6. LMWH has a lower fetal risk but a higher risk of maternal mortality and thromboembolism; Insufficient dose increases the risk of valve thrombosis and requires close anti-Xa monitoring.
  7. Monitor fetal growth.
  8. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team.
  9. Prepare and document the peripartum antithrombotic plan in advance with the obstetrician, anesthesiologist, and hematologist.

Treatment

  • Continue VKA (warfarin) with INR monitoring until week 36.
  • Warfarin: target INR (accepted range) — 2.5 (2.0–3.0) if there are no additional prothrombotic factors with bileaflet or newer-generation tilting-disc aortic valves, and 3.0 (2.5–3.5) if present; 3.0 (2.5–3.5) if there are no additional prothrombotic factors with any mechanical valve in the mitral or tricuspid position or any caged-ball or older-generation disc valve in any position, and 3.5 (3.0–4.0) if present; during pregnancy, check INR weekly, or at least every 2 weeks.
  • During pregnancy, measure INR weekly, at least every 2 weeks.
  • When switching from LMWH to VKA, continue LMWH with VKA; Discontinue LMWH after INR has been measured within the therapeutic range for two consecutive days.
  • In women who prioritize fetal risk, therapeutic dose SC LMWH is a reasonable alternative.
  • When switching to LMWH, discontinue VKA and start therapeutic dose LMWH on the same day.
  • Enoxaparin: initially 125 anti‑Xa IU/kg (1.25 mg/kg) SC every 12 hours, then 100 anti‑Xa IU/kg (1 mg/kg) SC every 12 hours.
  • Dalteparin: initially 125 IU/kg SC every 12 hours, then 100 IU/kg SC every 12 hours.
  • Tinzaparin: initially 250 IU/kg SC, then 175 IU/kg SC once daily.
  • Measure the peak anti-Xa level for the first time on day 2 or 3, at 4–6 hours after the dose; individualize target by valve type and location (usually 1.0–1.2 IU/mL; accepted range 0.8–1.2 IU/mL); Also measure the pre-dose (trough) level in selected patients at high risk of thrombosis.
  • If anti-Xa is below target, increase dose by 10–20% and remeasure after 48 hours.
  • INR usually falls below 1.5 on days 5–7; From this point on, anticoagulation is entirely dependent on LMWH, adjust the dose every 3–7 days with anti‑Xa measurement.
  • No fixed time is defined for the transition from the initial dose to the next dose; adjust subsequent doses based on measured peak anti-Xa level.
  • Consider adding low-dose aspirin (75–100 mg/day) at very high thrombotic risk.
  • For routine pregnancy anticoagulation, use UFH only if LMWH or anti‑Xa monitoring is not available and the patient refuses VKA; UFH is advantageous when creatinine clearance is < 30 mL/min or rapid reversal is required for delivery, neuraxial intervention, or emergency surgery.
  • If UFH is used — UFH (IV): in the hospital, start with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading; If switching from LMWH, it starts 12 hours after the last LMWH dose; Adjusted according to aPTT every 6 hours; target aPTT at least 2 times (2–2.5 times) control.

Note

  • Prophylactic dose of LMWH is insufficient for mechanical valve.
  • Use enoxaparin or dalteparin schedule when twice daily SC LMWH regimen is selected; Do not mix the once-daily tinzaparin schedule with this two-dose regimen.
  • Do not use LMWH without anti-Xa monitoring.
  • Do not use DOACs; they are not recommended during pregnancy.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs.
  • Warfarin: Additional prothrombotic factors: hereditary or acquired hypercoagulability, LVEF <35%, AF with significant mitral stenosis, major thrombotic event in the last 12 months (cardioembolic stroke, DVT, PE); The highest target is applied in multiple mechanical valves.
  • UFH (IV): Infusion is stopped 4–6 hours before neuraxial intervention and coagulation is evaluated.
36 weeks to birth (switch from VKA to heparin)

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Continue monitoring at a specialized center with a Pregnancy Heart Team; monitor fetal growth.
  3. Perform ECHO every trimester to evaluate valve function and exclude valve thrombosis; A greater than expected increase in gradient, new heart failure, embolic event, syncope, change in valve click, or new murmur requires immediate evaluation.
  4. Develop the peripartum antithrombotic plan with the obstetrician, anesthesiologist, and hematologist, clearly document it, and explain it in detail to the patient.
  5. Balance the risk of obstetric bleeding with valve thrombosis and the desire for regional anesthesia in the plan; Perform a prenatal obstetric anesthesia evaluation.
  6. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team.
  7. Consider planned delivery at approximately 39 weeks to avoid the risk of spontaneous labor while under anticoagulation in women receiving therapeutic doses of LMWH.

Treatment

  • Discontinue VKA (warfarin) at 36 weeks or at least 2 weeks before planned delivery, whichever is earlier, and initiate therapeutic dose SC LMWH or adjusted dose IV UFH on the same day.
  • Enoxaparin: initially 125 anti‑Xa IU/kg (1.25 mg/kg) SC every 12 hours, then 100 anti‑Xa IU/kg (1 mg/kg) SC every 12 hours.
  • Dalteparin: initially 125 IU/kg SC every 12 hours, then 100 IU/kg SC every 12 hours.
  • Tinzaparin: initially 250 IU/kg SC, then 175 IU/kg SC once daily.
  • Measure the peak anti-Xa level for the first time on day 2 or 3, at 4–6 hours after the dose; individualize target by valve type and location (usually 1.0–1.2 IU/mL; accepted range 0.8–1.2 IU/mL); Also measure the pre-dose (trough) level in selected patients at high risk of thrombosis.
  • If anti-Xa is below target, increase dose by 10–20% and remeasure after 48 hours.
  • INR usually falls below 1.5 on days 5–7; From this point on, anticoagulation is entirely dependent on LMWH, adjust the dose every 3–7 days with anti‑Xa measurement.
  • No fixed time is defined for the transition from the initial dose to the next dose; adjust subsequent doses based on measured peak anti-Xa level.
  • In women previously switched to LMWH, continue LMWH by adjusting the dose every 3–7 days with peak anti‑Xa measurement.
  • Switch to therapeutic IV UFH (aPTT ≥ 2 times control) at least 36 hours before delivery in women using LMWH; Discontinue UFH infusion 4–6 hours before expected delivery or neuraxial intervention; aPTT should be normal before neuraxial anesthesia.
  • When switching to IV UFH — UFH (IV): started in the hospital with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading; If switching from LMWH, it starts 12 hours after the last LMWH dose; Adjusted according to aPTT every 6 hours; target aPTT at least 2 times (2–2.5 times) control.
  • Consider adding low-dose aspirin (75–100 mg/day) at very high thrombotic risk.
  • In women taking aspirin with LMWH, consider discontinuing aspirin 4 days before delivery.
  • For routine pregnancy anticoagulation, use UFH only if LMWH or anti‑Xa monitoring is not available and the patient refuses VKA; UFH is advantageous when creatinine clearance is < 30 mL/min or rapid reversal is required for delivery, neuraxial intervention, or emergency surgery.
  • Actively manage third stage — Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • VKA effects last longer in the fetus than in the mother.
  • Prophylactic dose of LMWH is insufficient for mechanical valve.
  • Use enoxaparin or dalteparin schedule when twice daily SC LMWH regimen is selected; Do not mix the once-daily tinzaparin schedule with this two-dose regimen.
  • Do not use LMWH without anti-Xa monitoring.
  • Review current medications; Discontinue or change teratogenic or fetotoxic cardiac medications during pregnancy, such as ACE inhibitors and ARBs.
  • UFH (IV): Infusion is stopped 4–6 hours before neuraxial intervention and coagulation is evaluated.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Birth

Approach

  1. Uncomplicated and permanently stable mechanical valve mWHO III; In CARPREG II, mechanical valve is 3 points.
  2. Develop the peripartum antithrombotic plan with the obstetrician, anesthesiologist, and hematologist, clearly document it, and explain it in detail to the patient.
  3. Balance the risk of obstetric bleeding with valve thrombosis and the desire for regional anesthesia in the plan; As delivery approaches, tailor antithrombotic therapy to limit maternal and fetal bleeding, including spinal epidural hematoma due to neuraxial techniques.
  4. Plan the birth at a reference center with neonatal intensive care facilities; Prepare the birth plan in advance and distribute it to the entire team.
  5. Perform prenatal obstetric anesthesia evaluation; Apply continuous telemetry during labor.
  6. Consider planned delivery at approximately 39 weeks to avoid the risk of spontaneous labor while under anticoagulation in women receiving therapeutic doses of LMWH.
  7. If labor begins while using VKA or within 2 weeks of discontinuing VKA, perform cesarean section to prevent fetal intracranial hemorrhage.
  8. Plan vaginal delivery with neuraxial analgesia if timed according to the anticoagulation schedule and there is no obstetric indication; Minimize Valsalva.
  9. Involve a hematologist in emergency delivery under VKA.

Treatment

  • Discontinue VKA at least 2 weeks before planned delivery and bridge with therapeutic dose heparin (LMWH or IV UFH).
  • Switch to therapeutic IV UFH (aPTT ≥ 2 times control) at least 36 hours before delivery in women using LMWH; Discontinue UFH infusion 4–6 hours before expected delivery or neuraxial intervention; aPTT should be normal before neuraxial anesthesia.
  • When switching to IV UFH — UFH (IV): started in the hospital with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading; If switching from LMWH, it starts 12 hours after the last LMWH dose; Adjusted according to aPTT every 6 hours; target aPTT at least 2 times (2–2.5 times) control.
  • In women taking aspirin with LMWH, consider discontinuing aspirin 4 days before delivery.
  • Administer four-factor prothrombin complex concentrate IV to rapidly correct INR in emergency labor under VKA; Calculate the dose based on the factor IX unit, according to the product information of the preparation used: 25 IU/kg (maximum 2500 IU) if INR 2–<4, 35 IU/kg (maximum 3500 IU) if INR 4–6, 50 IU/kg (maximum 5000 IU) if INR > 6; For patients weighing over 100 kg, calculate based on 100 kg. Give vitamin K together; Measure the INR again after approximately 30 minutes.
  • Vitamin K: 1–2 mg IV or orally in emergency labor or for INR correction (maternal INR recovers in approximately 6 hours or more); In life-threatening bleeding, 10 mg IV in at least 50 mL of appropriate fluid, with a slow infusion (e.g. over 10–20 minutes) with a rate of up to 1 mg/min, together with four-factor PCC; If INR is still high after 12–24 hours, the dose may be repeated; fresh frozen plasma if four-factor PCC is not available, initial 15–30 mL/kg.
  • If birth occurs within 4–6 hours of UFH (aPTT not normalized) or within 12 hours of therapeutic LMWH — Protamine sulfate: very slow IV, up to 50 mg over 10 minutes; For UFH, 1 mg for every 100 units of heparin calculated to remain in circulation; For enoxaparin, 1 mg/1 mg enoxaparin if < 8 hours have passed since the last dose, 0.5 mg/1 mg if > 8 hours have passed; If aPTT is still prolonged after 2–4 hours, an additional dose of 0.5 mg/1 mg may be given.
  • Actively manage third stage — Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • May be considered at birth at high risk of endocarditis (prosthetic valve, previous endocarditis) — Endocarditis prophylaxis: 30–60 minutes before expected vaginal birth with the regimen of caesarean section prophylaxis (cefazolin 2 g IV; 3 g for 120 kg and over) or ampicillin 2 g IV single dose; In case of cesarean section, routine cesarean prophylaxis is sufficient; In cases of anaphylaxis, angioedema or urticaria to penicillin or ampicillin, vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of cephalosporin, infused over at least 60 minutes and at a maximum rate of 10 mg/min (2 g takes approximately 200 minutes).

Note

  • If IV UFH is used, perform neuraxial intervention after the infusion has stopped (usually 4–6 hours) and aPTT has returned to normal.
  • Do not use SC UFH for a mechanical valve; the risk of valve thrombosis in pregnancy is unacceptably high.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal birth.
  • The safety of repeat dosing with PCC has not been established; In life-threatening bleeding from a more thrombogenic mechanical valve, a lower initial dose (12.5 IU/kg) may be considered.
  • Protamine does not completely counteract the anti-Xa effect of enoxaparin (at most approximately 60%); Rapid administration may lead to severe hypotension and bradycardia.
  • UFH (IV): Infusion is stopped 4–6 hours before neuraxial intervention and coagulation is evaluated.
  • Vitamin K: Vitamin K administered to the mother does not fully correct the fetal INR; the fetus may remain anticoagulated for 8–10 days after VKA discontinuation; The newborn may require fresh frozen plasma and higher doses of vitamin K.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Postpartum period

Approach

  1. Continue Pregnancy Heart Team monitoring during the puerperium.
  2. Determine when to resume anticoagulation by balancing the risk of bleeding from the incision and uterus against the risk of thromboembolic complications, including valve thrombosis.
  3. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  4. Avoid combined hormonal methods containing estrogen for contraception; Select intrauterine device or progestin implant based on anticoagulation status and bleeding profile.

Treatment

  • Unless there is significant bleeding or significant risk of bleeding, restart anticoagulation shortly after birth (usually 4–6 hours after vaginal birth, 6–12 hours after cesarean section); Make the decision to restart with the Pregnancy Heart Team and the patient.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • If starting with LMWH, first dose — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • After 12 hours: enoxaparin 0.75 mg/kg SC every 12 hours (based on postpartum weight).
  • If there is no bleeding, when switching to therapeutic LMWH: enoxaparin 100 anti-Xa IU/kg (1 mg/kg) SC every 12 hours.
  • If starting with IV UFH — UFH (IV): start with an infusion of 1000–1250 IU/hour (approximately 18 IU/kg/hour) without loading, 4–6 hours after birth (6–12 hours after cesarean section), after neuraxial block, and at least 1 hour after epidural catheter removal; By adjusting the aPTT every 6 hours, reach the therapeutic level in 24–48 hours after vaginal birth and in 48–72 hours after cesarean section; target aPTT at least 2 times control.
  • Start VKA after wound healing, 7–14 days or later after birth, to reduce late bleeding; Continue heparin with VKA and discontinue heparin after INR is within the therapeutic range for two consecutive days.
  • Warfarin: target INR (accepted range) — 2.5 (2.0–3.0) if there are no additional prothrombotic factors with bileaflet or newer-generation tilting-disc aortic valves, and 3.0 (2.5–3.5) if present; 3.0 (2.5–3.5) if there are no additional prothrombotic factors with any mechanical valve in the mitral or tricuspid position or any caged-ball or older-generation disc valve in any position, and 3.5 (3.0–4.0) if present; during pregnancy, check INR weekly, or at least every 2 weeks.

Note

  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Do not use DOACs; they are generally contraindicated during breastfeeding.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • Warfarin: Additional prothrombotic factors: hereditary or acquired hypercoagulability, LVEF <35%, AF with significant mitral stenosis, major thrombotic event in the last 12 months (cardioembolic stroke, DVT, PE); The highest target is applied in multiple mechanical valves.
Bioprosthetic valve
Antithrombotic requirement
No additional risk factors (aspirin only)

Approach

  1. Perform pre-pregnancy evaluation; Plan multidisciplinary follow-up with specialists experienced in heart disease in pregnancy and perform cardiology evaluation at least every trimester (more frequently if the clinical situation requires).
  2. Record date, type (surgical or transcatheter) and location of implantation; Further evaluate with the Pregnancy Heart Team the need for anticoagulation during pregnancy within the first 3 months after surgical mitral or tricuspid bioprosthesis implantation.
  3. Evaluate valve type and function, ventricular systolic function, and pulmonary artery pressure by ECHO; Pregnancy outcome varies accordingly.
  4. Do not automatically assign a risk class based solely on the presence of a bioprosthesis; determine mWHO and CARPREG II together by evaluating valve function, ventricular function, pulmonary pressure, and comorbidities.
  5. Monitor fetal growth if moderate or high maternal cardiac risk.
  6. Plan the birth at the reference center; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  7. Determine the frequency of postpartum check-ups according to the lesion and symptoms; It may take up to 6 months for the hemodynamic changes of pregnancy to resolve.
  8. Prefer long-acting reversible methods of contraception (intrauterine device, progestin implant); Individualize selection based on lesion, anticoagulation status, and bleeding profile.

Treatment

  • Administer low-dose aspirin (75–100 mg/day) and continue throughout pregnancy.
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • May be considered at birth at high risk of endocarditis (prosthetic valve, previous endocarditis) — Endocarditis prophylaxis: 30–60 minutes before expected vaginal birth with the regimen of caesarean section prophylaxis (cefazolin 2 g IV; 3 g for 120 kg and over) or ampicillin 2 g IV single dose; In case of cesarean section, routine cesarean prophylaxis is sufficient; In cases of anaphylaxis, angioedema or urticaria to penicillin or ampicillin, vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of cephalosporin, infused over at least 60 minutes and at a maximum rate of 10 mg/min (2 g takes approximately 200 minutes).

Note

  • Long-term anticoagulation is not required if there are no additional risk factors.
  • If no other antithrombotics are planned, do not discontinue aspirin before delivery.
  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal birth.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
Atrial fibrillation or previous bioprosthetic valve thrombosis (anticoagulation required)

Approach

  1. Perform pre-pregnancy evaluation; Plan multidisciplinary follow-up with specialists experienced in heart disease in pregnancy and perform cardiology evaluation at least every trimester (more frequently if the clinical situation requires).
  2. Evaluate valve type and function, ventricular systolic function, and pulmonary artery pressure by ECHO; Pregnancy outcome varies accordingly.
  3. Do not automatically assign a risk class based solely on the presence of a bioprosthesis; determine mWHO and CARPREG II together by evaluating valve function, ventricular function, pulmonary pressure, and comorbidities.
  4. Monitor fetal growth if moderate or high maternal cardiac risk.
  5. Develop the peripartum antithrombotic plan with the obstetrician, anesthesiologist, and hematologist, clearly document it, and explain it in detail to the patient.
  6. Balance the risk of obstetric bleeding with valve thrombosis and the desire for regional anesthesia in the plan; As delivery approaches, tailor antithrombotic therapy to limit maternal and fetal bleeding, including spinal epidural hematoma due to neuraxial techniques.
  7. Plan the birth at the reference center; Plan vaginal delivery with appropriate analgesia, minimizing Valsalva.
  8. Determine frequency of postpartum check-ups based on lesion and symptoms (hemodynamic changes of pregnancy may take up to 6 months to resolve); Choose long-acting reversible methods of contraception (intrauterine device, progestin implant) and individualize the choice according to the lesion, anticoagulation status and bleeding profile.

Treatment

  • Individualize anticoagulation according to maternal thromboembolic risk; LMWH is generally preferred during pregnancy (therapeutic dose: enoxaparin according to weight, usually 1 mg/kg SC every 12 hours).
  • Consider the recommendation of low-dose aspirin (75–100 mg/day) for a bioprosthetic valve within the antithrombotic plan; Let the expert team determine its use with anticoagulation, considering the risk of bleeding.
  • For women using low-dose aspirin with anticoagulants, consider stopping aspirin 4 days before the planned birth with the anesthesia and Pregnancy Heart Team.
  • Discontinue heparin before planned delivery: wait at least 12 hours after last prophylactic dose of LMWH, wait at least 24 hours after intermediate or therapeutic dose.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulation 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the intervention, and in any case at least 4 hours after the epidural catheter is removed.
  • Actively manage third stage — Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • If cesarean delivery is to be performed — Cefazolin: 2 g IV as a single dose for weight under 120 kg, or 3 g IV for 120 kg and above, within 60 minutes before skin incision; repeat the dose if the procedure is still ongoing 4 hours after the first dose or if blood loss exceeds 1500 mL; for cesarean delivery performed during labor or after membrane rupture, add azithromycin 500 mg as a single IV infusion in 250 mL of compatible fluid over 1 hour (do not administer as an IV bolus or IM); in severe beta-lactam allergy, administer clindamycin 900 mg IV + gentamicin 5 mg/kg IV single dose instead of cefazolin (if actual weight is more than 20% above ideal weight, dosing weight = ideal weight + 0.4 × (actual weight − ideal weight)); if in labor or membranes are ruptured, add azithromycin in the same manner.
  • May be considered at birth at high risk of endocarditis (prosthetic valve, previous endocarditis) — Endocarditis prophylaxis: 30–60 minutes before expected vaginal birth with the regimen of caesarean section prophylaxis (cefazolin 2 g IV; 3 g for 120 kg and over) or ampicillin 2 g IV single dose; In case of cesarean section, routine cesarean prophylaxis is sufficient; In cases of anaphylaxis, angioedema or urticaria to penicillin or ampicillin, vancomycin 15–20 mg/kg IV as a single dose (maximum 2 g per dose) instead of cephalosporin, infused over at least 60 minutes and at a maximum rate of 10 mg/min (2 g takes approximately 200 minutes).

Note

  • If UFH is used, perform neuraxial intervention after aPTT returns to normal; Normalization usually takes approximately 6 hours after therapeutic IV infusion and up to 24 hours after SC administration.
  • Do not use DOACs; they are not recommended during pregnancy and are generally contraindicated during breastfeeding.
  • Review current medications; Discontinue or change teratogenic or fetotoxic heart medications such as ACE inhibitors and ARBs.
  • Caesarean section prophylaxis also protects against endocarditis; Routine antibiotic prophylaxis is not required for vaginal birth.
  • Review the breastfeeding safety of each medication in the breastfeeding woman prior to discharge.
  • Oxytocin: Slow infusion is used instead of rapid bolus.

Cardiomyopathies and heart failure

Peripartum cardiomyopathy (new diagnosis)
Period
Pregnancy continues (antepartum)
Clinical situation
Cardiogenic shock

Approach

  1. Confirm the diagnosis: heart failure due to left ventricular systolic dysfunction with LVEF < 45%, not explained by another cause, developing toward the end of pregnancy or in the months following delivery (or miscarriage/pregnancy termination). Do not use the time window between the last month of pregnancy and the 5th month postpartum as a mandatory exclusion criterion.
  2. Manage with the Pregnancy Heart Team in the intensive care unit, consisting of cardiology, perinatology, anesthesia, intensive care, cardiac surgery and neonatal specialists.
  3. Plan early referral to a center that can provide mechanical circulatory support (VA-ECMO or Impella).
  4. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  5. If the fetus has reached the limit of viability in cardiogenic shock, recommend combined spinal/epidural analgesia or emergency cesarean section under general anesthesia, taking into account the gestational age, accompanying diseases and the facilities of the center; Don't expect refractoriness. Prioritize maternal stabilization before the limit of viability and make the birth decision with the Pregnancy Heart Team.
  6. Caesarean section can be performed under VA-ECMO or Impella support.
  7. Monitor fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  8. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  9. Closely monitor the first 24–72 hours after birth as the most critical period.
  10. Plan discontinuation of heparin before planned delivery based on dose and risk of thrombosis; for neuraxial intervention, at least 12 hours must elapse after the last prophylactic dose of LMWH and at least 24 hours after the intermediate or therapeutic dose; plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).

Treatment

  • Dobutamine: Start with 2–5 µg/kg/min IV infusion, titrating to 2–20 µg/kg/min according to response.
  • Milrinone: 0.375–0.75 µg/kg/min IV infusion; If creatinine clearance is 10–50 mL/min, start with 0.0625–0.125 µg/kg/min and titrate carefully and do not exceed 0.375 µg/kg/min in significant renal impairment; If creatinine clearance is <10 mL/min, another agent is chosen; Hypotension may be prolonged in renal impairment.
  • Levosimendan: 0.1 µg/kg/min IV infusion without loading dose; 0.05–0.2 µg/kg/min depending on response; The recommended infusion time is 24 hours.
  • If hypotension persists despite inotrope — Noradrenaline: Start with 0.05 µg/kg/min IV infusion, titrate according to mean arterial pressure and organ perfusion; usual range 0.05–0.4 µg/kg/min, up to 1.0 µg/kg/min if necessary.
  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • In congestion — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Venoarterial ECMO (VA-ECMO) or percutaneous left ventricular assist device (Impella): apply as a bridge to transplant or myocardial recovery in inotrope-resistant shock.
  • If LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • ACE inhibitor, ARB and ARNI are contraindicated during pregnancy.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Milrinone: Since it crosses the placenta during pregnancy, it is used only when the benefit outweighs the risk.
  • Levosimendan: Not used in creatinine clearance <30 mL/min, severe liver failure, significant mechanical obstruction affecting ventricular filling or outflow, history of torsade de pointes, severe hypotension, and tachycardia; In hypotension, if necessary, it is given together with noradrenaline; The hemodynamic effect may last up to 9 days after the 24-hour infusion ends.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
No shock (hemodynamically stable)

Approach

  1. Confirm the diagnosis: heart failure due to left ventricular systolic dysfunction with LVEF < 45%, not explained by another cause, developing toward the end of pregnancy or in the months following delivery (or miscarriage/pregnancy termination). Do not use the time window between the last month of pregnancy and the 5th month postpartum as a mandatory exclusion criterion.
  2. Manage with the Pregnancy Heart Team; Prepare the birth plan in writing with the team.
  3. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  4. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  5. Initiate epidural analgesia early in labor and maintain it rigorously; It reduces systemic vascular resistance and provides an advantage in left ventricular systolic dysfunction.
  6. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  7. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  8. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  9. Closely monitor the first 24–72 hours after birth as the most critical period.
  10. Plan discontinuation of heparin before planned delivery based on dose and risk of thrombosis; for neuraxial intervention, at least 12 hours must elapse after the last prophylactic dose of LMWH and at least 24 hours after the intermediate or therapeutic dose; plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).

Treatment

  • After resolution of acute pulmonary edema, in stable and euvolemic patient — Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • After resolution of acute pulmonary edema, in stable and euvolemic patient — Bisoprolol: 1.25 mg once daily is started; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • If IV nitrate is required — Nitroglycerin: Start with 5 µg/min IV infusion, increasing every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In mild, stable congestion in the absence of acute pulmonary edema — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • If LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Immediately after birth — Enalapril: Start at 2.5 mg twice daily; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.

Note

  • Use only cardioselective beta blockers (metoprolol, bisoprolol) during pregnancy.
  • ACE inhibitor, ARB and ARNI are contraindicated during pregnancy.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • Cardiac MRI is safe; do not use gadolinium during pregnancy.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
Postpartum
Clinical status and LVEF
Cardiogenic shock

Approach

  1. Confirm the diagnosis: heart failure due to left ventricular systolic dysfunction with LVEF < 45%, not explained by another cause, developing toward the end of pregnancy or in the months following delivery (or miscarriage/pregnancy termination). Do not use the time window between the last month of pregnancy and the 5th month postpartum as a mandatory exclusion criterion.
  2. Manage with the Pregnancy Heart Team in the intensive care unit, consisting of cardiology, perinatology, anesthesia, intensive care, cardiac surgery and neonatal specialists.
  3. Plan early referral to a center that can provide mechanical circulatory support (VA-ECMO or Impella).
  4. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  5. Closely monitor the first 24–72 hours after birth as the most critical period.
  6. In subsequent pregnancy counseling, indicate that more than mild residual left ventricular dysfunction is mWHO IV (risk of recurrence may reach 50%, maternal mortality 15–20%) and recommend avoiding pregnancy.
  7. Maximum risk of mild residual dysfunction is mWHO III; Individualize recurrence risk and pregnancy recommendation with the Pregnancy Heart Team.
  8. State that even with full recovery of LVEF above 50%, the risk of relapse in a new pregnancy is approximately 20%; Detailed informed consent and multidisciplinary management are required.
  9. If anticoagulation remains indicated and there is no significant bleeding or significant risk of bleeding, plan for postpartum resumption; The general obstetric range is 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  10. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Dobutamine: Start with 2–5 µg/kg/min IV infusion, titrating to 2–20 µg/kg/min according to response.
  • Milrinone: 0.375–0.75 µg/kg/min IV infusion; If creatinine clearance is 10–50 mL/min, start with 0.0625–0.125 µg/kg/min and titrate carefully and do not exceed 0.375 µg/kg/min in significant renal impairment; If creatinine clearance is <10 mL/min, another agent is chosen; Hypotension may be prolonged in renal impairment.
  • Levosimendan: 0.1 µg/kg/min IV infusion without loading dose; 0.05–0.2 µg/kg/min depending on response; The recommended infusion time is 24 hours.
  • If hypotension persists despite inotrope — Noradrenaline: Start with 0.05 µg/kg/min IV infusion, titrate according to mean arterial pressure and organ perfusion; usual range 0.05–0.4 µg/kg/min, up to 1.0 µg/kg/min if necessary.
  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • In congestion — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Venoarterial ECMO (VA-ECMO) or percutaneous left ventricular assist device (Impella): apply as a bridge to transplant or myocardial recovery in inotrope-resistant shock.
  • Bromocriptine may be considered in moderate–severe PPCM: 2.5 mg twice daily for 2 weeks, followed by 2.5 mg once daily for 6 weeks; Add at least prophylactic dose of LMWH.
  • If LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient, bromocriptine use) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • Breastfeeding is controversial; bromocriptine suppresses milk production and breastfeeding cannot be continued during treatment; Discuss the baby's feeding plan before treatment.
  • Bromocriptine is not used in uncontrolled hypertension; If you have preeclampsia/eclampsia or postpartum hypertension, make use decisions with the Pregnancy Heart Team. Monitor blood pressure, especially in the first days and during dose increases; In case of new hypertension, severe or persistent headache, visual impairment, or chest pain, discontinue the medication and seek immediate evaluation.
  • If bromocriptine is considered if LVEF ≥ 30% with severe shock, individualize the dosing schedule with the specialist team.
  • Excessive diuresis reduces intravascular volume; Titrate furosemide dose.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Levosimendan: Not used in creatinine clearance <30 mL/min, severe liver failure, significant mechanical obstruction affecting ventricular filling or outflow, history of torsade de pointes, severe hypotension, and tachycardia; In hypotension, if necessary, it is given together with noradrenaline; The hemodynamic effect may last up to 9 days after the 24-hour infusion ends.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
No shock, LVEF < 25%

Approach

  1. Confirm the diagnosis: heart failure due to left ventricular systolic dysfunction with LVEF < 45%, not explained by another cause, developing toward the end of pregnancy or in the months following delivery (or miscarriage/pregnancy termination). Do not use the time window between the last month of pregnancy and the 5th month postpartum as a mandatory exclusion criterion.
  2. Watch with the Pregnancy Heart Team.
  3. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  4. Closely monitor the first 24–72 hours after birth as the most critical period.
  5. In subsequent pregnancy counseling, indicate that more than mild residual left ventricular dysfunction is mWHO IV (risk of recurrence may reach 50%, maternal mortality 15–20%) and recommend avoiding pregnancy.
  6. Maximum risk of mild residual dysfunction is mWHO III; Individualize recurrence risk and pregnancy recommendation with the Pregnancy Heart Team.
  7. State that even with full recovery of LVEF above 50%, the risk of relapse in a new pregnancy is approximately 20%; Detailed informed consent and multidisciplinary management are required.
  8. If anticoagulation remains indicated and there is no significant bleeding or significant risk of bleeding, plan for postpartum resumption; The general obstetric range is 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  9. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Immediately after birth — Enalapril: Start at 2.5 mg twice daily; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • After resolution of acute pulmonary edema, in stable and euvolemic patient — Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • After resolution of acute pulmonary edema, in stable and euvolemic patient — Bisoprolol: 1.25 mg once daily is started; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In mild, stable congestion in the absence of acute pulmonary edema — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • Bromocriptine may be considered in moderate–severe PPCM: 2.5 mg twice daily for 2 weeks, followed by 2.5 mg once daily for 6 weeks; Add at least prophylactic dose of LMWH.
  • If LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient, bromocriptine use) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • Breastfeeding is controversial; bromocriptine suppresses milk production and breastfeeding cannot be continued during treatment; Discuss the baby's feeding plan before treatment.
  • Bromocriptine is not used in uncontrolled hypertension; If you have preeclampsia/eclampsia or postpartum hypertension, make use decisions with the Pregnancy Heart Team. Monitor blood pressure, especially in the first days and during dose increases; In case of new hypertension, severe or persistent headache, visual impairment, or chest pain, discontinue the medication and seek immediate evaluation.
  • Excessive diuresis reduces intravascular volume; Titrate furosemide dose.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
No shock, LVEF ≥ 25%

Approach

  1. Confirm the diagnosis: heart failure due to left ventricular systolic dysfunction with LVEF < 45%, not explained by another cause, developing toward the end of pregnancy or in the months following delivery (or miscarriage/pregnancy termination). Do not use the time window between the last month of pregnancy and the 5th month postpartum as a mandatory exclusion criterion.
  2. Watch with the Pregnancy Heart Team.
  3. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  4. Closely monitor the first 24–72 hours after birth as the most critical period.
  5. In subsequent pregnancy counseling, indicate that more than mild residual left ventricular dysfunction is mWHO IV (risk of recurrence may reach 50%, maternal mortality 15–20%) and recommend avoiding pregnancy.
  6. Maximum risk of mild residual dysfunction is mWHO III; Individualize recurrence risk and pregnancy recommendation with the Pregnancy Heart Team.
  7. State that even with full recovery of LVEF above 50%, the risk of relapse in a new pregnancy is approximately 20%; Detailed informed consent and multidisciplinary management are required.
  8. If anticoagulation remains indicated and there is no significant bleeding or significant risk of bleeding, plan for postpartum resumption; The general obstetric range is 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  9. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Immediately after birth — Enalapril: Start at 2.5 mg twice daily; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • After resolution of acute pulmonary edema, in stable and euvolemic patient — Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • After resolution of acute pulmonary edema, in stable and euvolemic patient — Bisoprolol: 1.25 mg once daily is started; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • In pulmonary edema — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In mild, stable congestion in the absence of acute pulmonary edema — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • If bromocriptine is considered: 2.5 mg twice daily for 2 weeks, then 2.5 mg once daily for 6 weeks if LVEF ≥ 25% and < 30%; If LVEF > 30%, 2.5 mg once daily for 1 week; In all cases, use at least the prophylactic dose of LMWH.
  • If LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient, bromocriptine use) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • Breastfeeding is controversial; bromocriptine suppresses milk production and breastfeeding cannot be continued during treatment; Discuss the baby's feeding plan before treatment.
  • Bromocriptine is not used in uncontrolled hypertension; If you have preeclampsia/eclampsia or postpartum hypertension, make use decisions with the Pregnancy Heart Team. Monitor blood pressure, especially in the first days and during dose increases; In case of new hypertension, severe or persistent headache, visual impairment, or chest pain, discontinue the medication and seek immediate evaluation.
  • In mild disease with LVEF > 30%, breastfeeding may be allowed with close monitoring if function improves rapidly.
  • If LVEF is exactly 30%, individualize the dosing schedule with the expert team.
  • Excessive diuresis reduces intravascular volume; Titrate furosemide dose.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Previous peripartum cardiomyopathy (subsequent pregnancy)
Left ventricular function
Complete recovery (LVEF > 50%)
Period
Pre-pregnancy counseling

Approach

  1. Risk class: mWHO III (high risk); Complete recovery does not eliminate the risk of recurrence.
  2. Explain that the risk of recurrence in a new pregnancy is approximately 20%; Obtain detailed informed consent.
  3. Manage with the multidisciplinary Pregnancy Heart Team from pre-pregnancy.
  4. Explain that if pregnancy occurs, close ECHO monitoring will be required at the reference center; individualize viewing.
  5. Document NYHA class, ECG, ECHO and baseline BNP/NT‑proBNP before pregnancy.
  6. If pregnancy planning is continued, complete medication changes before pregnancy and verify cardiac stability on the new regimen.

Treatment

  • Beta blocker required — Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Beta blocker required — Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Effective contraception: use a progestin implant or levonorgestrel-releasing intrauterine device until pregnancy is planned.

Note

  • If pregnancy is planned, discontinue ACE inhibitors, ARBs and ARNIs before conception.
  • Avoid spironolactone.
  • Avoid atenolol if beta blocker is required.
  • Combined hormonal contraceptive is contraindicated in the first 6 months after PPCM or NYHA III–IV dysfunction; If function is normal or slightly impaired and at least 6 months have passed, it is generally not recommended; Prefer progestin methods.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy has occurred

Approach

  1. Risk class: mWHO III; Make close ECHO monitoring at the reference center.
  2. The risk of recurrence is approximately 20%; Manage with the multidisciplinary Pregnancy Heart Team.
  3. Consider paroxysmal nocturnal dyspnea, persistent cough, chest pain, and significant tachycardia as red flags; look for jugular venous distension, basal crackles and an S3 gallop.
  4. Measure BNP/NT‑proBNP when relapse is suspected; normal value makes PPCM unlikely, significant elevation favors heart failure.
  5. Monitor fetal growth with serial USG.
  6. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  7. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  8. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  9. Initiate epidural analgesia early in labor and maintain it meticulously.
  10. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  11. Closely monitor the first 24–72 hours after birth as the most critical period.

Treatment

  • Beta blocker required — Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Beta blocker required — Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • ACE inhibitor, ARB and ARNI are contraindicated during pregnancy.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • If beta blockers are used, monitor the newborn for bradycardia and hypoglycemia.
  • Combined oral contraceptive is strictly contraindicated.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
At most mild residual dysfunction
Period
Pre-pregnancy counseling

Approach

  1. Risk class: mWHO III.
  2. Individualize risk of relapse and mortality and pregnancy recommendation with the Pregnancy Heart Team; Obtain detailed informed consent.
  3. If LVEF is exactly 50%, evaluate pregnancy risk and recommendation for subsequent pregnancy individually with the Pregnancy Heart Team.
  4. State in counseling that complications are common and intensive antenatal, intrapartum and postpartum monitoring is required.
  5. Manage with the multidisciplinary Pregnancy Heart Team at the specialist center from pre-pregnancy.
  6. Document NYHA class, ECG, ECHO and baseline BNP/NT‑proBNP before pregnancy.
  7. If pregnancy planning is continued, complete medication changes before pregnancy and verify cardiac stability on the new regimen.

Treatment

  • If pregnancy is planned and afterload reduction is to be maintained, instead of ACE inhibitor, ARB, or ARNI — Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Beta blocker required — Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Beta blocker required — Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Effective contraception: use a progestin implant or levonorgestrel-releasing intrauterine device until pregnancy is planned.

Note

  • If pregnancy is planned, discontinue ACE inhibitors, ARBs and ARNIs before conception.
  • Avoid spironolactone.
  • Avoid atenolol if beta blocker is required.
  • Combined hormonal contraceptive is contraindicated in the first 6 months after PPCM or NYHA III–IV dysfunction; If function is normal or slightly impaired and at least 6 months have passed, it is generally not recommended; Prefer progestin methods.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy has occurred

Approach

  1. Risk class: mWHO III; Manage with the Pregnancy Heart Team at the specialist centre.
  2. Perform cardiology follow-up usually every 1–2 months; Monitor ventricular function with ECHO, individualizing the frequency of checks according to symptoms, functional status and ventricular function.
  3. If LVEF is exactly 50%, evaluate pregnancy risk individually with the Pregnancy Heart Team.
  4. Monitor for red flags (paroxysmal nocturnal dyspnea, persistent cough, chest pain, marked tachycardia, jugular venous distention, basal rales, S3 gallop); When in doubt, measure BNP/NT‑proBNP.
  5. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  6. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  7. Initiate epidural analgesia early in labor and maintain it rigorously; It reduces systemic vascular resistance and provides an advantage in left ventricular systolic dysfunction.
  8. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  9. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  10. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  11. Closely monitor the first 24–72 hours after birth as the most critical period.
  12. If acute pulmonary edema or hypoperfusion develops, switch to an acute decompensated heart failure approach.
  13. If anticoagulation is administered, plan discontinuation of heparin before planned delivery according to dose and risk of thrombosis (for neuroaxial intervention, at least 12 hours should elapse from the last prophylactic LMWH dose and at least 24 hours from the intermediate or therapeutic dose); plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).
  14. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • If IV nitrate is required — Nitroglycerin: Start with 5 µg/min IV infusion, increasing every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • If congestion develops — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • After birth, in hemodynamically stable patient — Enalapril: 2.5 mg twice daily is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • If documented intracardiac thrombus or LVEF < 35%, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • In other cases, if thromboprophylaxis is to be applied by individual decision — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH during pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • If creatinine clearance is < 30 mL/min, instead of postpartum prophylactic LMWH — UFH (SC, prophylactic): postpartum 5000 IU SC every 8–12 hours.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • ACE inhibitor, ARB and ARNI are contraindicated in pregnancy; If using it, stop it.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • Use only cardioselective beta blockers (metoprolol, bisoprolol) during pregnancy.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
More than mild residual dysfunction (e.g. LVEF < 50% and significant)
Period
Pre-pregnancy counseling

Approach

  1. Risk class: mWHO IV; Recommend avoiding pregnancy.
  2. Explain that the risk of recurrence in a new pregnancy can reach 50% and maternal mortality can reach 15–20%.
  3. Address fetal effects, uncertainty of evidence, reproductive alternatives, and effective contraception in counseling with the expert multidisciplinary team.
  4. Clearly state in the record that pregnancy is contraindicated, the rationale, and the patient's understanding and preferences.
  5. Recommend tubal ligation; Share the decision without forcing it and evaluate the procedure and anesthesia risk in advance.
  6. If pregnancy planning is continued, complete medication changes before pregnancy and verify cardiac stability on the new regimen.

Treatment

  • If the woman still plans pregnancy, instead of ACE inhibitor, ARB and ARNI — Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Contraception: use a progestin implant or levonorgestrel-releasing intrauterine device.

Note

  • If pregnancy is planned, discontinue ACE inhibitors, ARBs and ARNIs before conception.
  • Avoid spironolactone.
  • Combined oral contraceptive is strictly contraindicated.
  • Maintain beta blocker therapy; Avoid atenolol.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy has occurred

Approach

  1. Risk class: mWHO IV; The risk of relapse can reach 50% and maternal mortality can reach 15–20%.
  2. Discuss the option of terminating the pregnancy if it is legally and ethically appropriate; Make the decision jointly with the woman.
  3. If pregnancy persists, continue care with the Pregnancy Heart Team at a tertiary or quaternary center with advanced cardiac, obstetrics, anesthesia, intensive care and neonatal expertise.
  4. Monitor ventricular function with echocardiography, individualizing follow-up frequency according to symptoms, functional status, and ventricular function; watch for red flags (paroxysmal nocturnal dyspnea, persistent cough, chest pain, marked tachycardia, jugular venous distention, basal rales, S3 gallop), and measure BNP/NT-proBNP when in doubt.
  5. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  6. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  7. Initiate epidural analgesia early in labor and maintain it rigorously; It reduces systemic vascular resistance and provides an advantage in left ventricular systolic dysfunction.
  8. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  9. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  10. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  11. Closely monitor the first 24–72 hours after birth as the most critical period.
  12. If acute pulmonary edema or hypoperfusion develops, switch to an acute decompensated heart failure approach.
  13. Recommend tubal ligation before discharge.
  14. If anticoagulation is administered, plan discontinuation of heparin before planned delivery according to dose and risk of thrombosis (for neuroaxial intervention, at least 12 hours should elapse from the last prophylactic LMWH dose and at least 24 hours from the intermediate or therapeutic dose); plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).
  15. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • If IV nitrate is required — Nitroglycerin: Start with 5 µg/min IV infusion, increasing every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • If congestion develops — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • After birth, in hemodynamically stable patient — Enalapril: 2.5 mg twice daily is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • If documented intracardiac thrombus or LVEF < 35%, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • In other cases, if thromboprophylaxis is to be applied by individual decision — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH during pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • If creatinine clearance is < 30 mL/min, instead of postpartum prophylactic LMWH — UFH (SC, prophylactic): postpartum 5000 IU SC every 8–12 hours.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • ACE inhibitor, ARB and ARNI are contraindicated in pregnancy; If using it, stop it.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • Use only cardioselective beta blockers (metoprolol, bisoprolol) during pregnancy.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Dilated cardiomyopathy / left ventricular systolic dysfunction before pregnancy
LVEF and NYHA class
LVEF < 30% or NYHA III–IV
Period
Pre-conception (planning)

Approach

  1. Risk class: mWHO IV; Pregnancy is contraindicated, recommend avoiding pregnancy.
  2. Explain in counseling that maternal mortality is 15–50%.
  3. Address fetal effects, uncertainty of evidence, reproductive alternatives, and effective contraception in counseling with the expert multidisciplinary team.
  4. Conduct reproductive planning with the Pregnancy Heart Team, which includes a perinatologist, cardiologist and geneticist.
  5. Clearly state in the record that pregnancy is contraindicated, the rationale, and the patient's understanding and preferences.
  6. Recommend tubal ligation; Share the decision without forcing it and evaluate the procedure and anesthesia risk in advance.

Treatment

  • If the woman still plans pregnancy, instead of ACE inhibitor, ARB and ARNI — Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Contraception: use a progestin implant or levonorgestrel-releasing intrauterine device.

Note

  • ACE inhibitor, ARB and ARNI are teratogenic; If the woman still plans the pregnancy, stop it.
  • Optimize beta blocker therapy; Avoid atenolol.
  • Avoid spironolactone if pregnancy is planned.
  • Combined oral contraceptive is strictly contraindicated; It worsens heart failure through sodium and water retention and increases the risk of VTE.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy, birth and puerperium

Approach

  1. Risk class: mWHO IV; maternal mortality is 15–50%.
  2. Recommend termination of pregnancy in the first trimester; Make the decision jointly with the woman within the legal and ethical framework.
  3. If pregnancy persists, continue care with the Pregnancy Heart Team at a tertiary or quaternary center with advanced cardiac, obstetrics, anesthesia, intensive care and neonatal expertise.
  4. Monitor ventricular function with ECHO, individualizing the frequency of checks according to symptoms, functional status and ventricular function; Monitor closely in early pregnancy as rapid decompensation may occur after discontinuation of an ACE inhibitor, ARB, or ARNI.
  5. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  6. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  7. Initiate epidural analgesia early in labor and maintain it rigorously; It reduces systemic vascular resistance and provides an advantage in left ventricular systolic dysfunction.
  8. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  9. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  10. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  11. Closely monitor the first 24–72 hours after birth as the most critical period; Intensify postpartum monitoring.
  12. If acute pulmonary edema or hypoperfusion develops, switch to an acute decompensated heart failure approach.
  13. Recommend tubal ligation before discharge.
  14. If anticoagulation is administered, plan discontinuation of heparin before planned delivery according to dose and risk of thrombosis (for neuroaxial intervention, at least 12 hours should elapse from the last prophylactic LMWH dose and at least 24 hours from the intermediate or therapeutic dose); plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).
  15. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • If congestion develops — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • After birth, in hemodynamically stable patient — Enalapril: 2.5 mg twice daily is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • If documented intracardiac thrombus or LVEF < 35%, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • In other cases, if thromboprophylaxis is to be applied by individual decision — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH during pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • If creatinine clearance is < 30 mL/min, instead of postpartum prophylactic LMWH — UFH (SC, prophylactic): postpartum 5000 IU SC every 8–12 hours.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • ACE inhibitor, ARB and ARNI are contraindicated in pregnancy; If using it, stop it.
  • Optimize beta blocker therapy; Avoid atenolol.
  • Avoid spironolactone.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
LVEF ≥ 30% and NYHA I–II
Period
Pre-conception (planning)

Approach

  1. Risk class: mWHO III if LVEF 30–45%, mWHO II–III if LVEF > 45%.
  2. If LVEF is 30–45%, close ECHO monitoring is required at the reference center during pregnancy.
  3. Explain that a stable woman with LVEF > 40% and NYHA I–II usually tolerates pregnancy.
  4. Explain that even in moderate dysfunction, clinically significant heart failure, arrhythmia, or medication increase may be required.
  5. Conduct pregnancy planning with the Pregnancy Heart Team, which includes a perinatologist, cardiologist and geneticist.
  6. Document NYHA class, ECG, ECHO and baseline BNP/NT‑proBNP before pregnancy; Perform a stress test on the selected patient.
  7. Plan pregnancy while clinically stable, at optimal functional capacity, and on a pregnancy-compatible medication regimen; Make medication changes before pregnancy and verify stability on the new regimen.
  8. If assisted reproductive technology is planned, make the decision with the reproductive medicine specialist and the Pregnancy Heart Team before treatment; If possible, recommend single embryo transfer.

Treatment

  • Instead of ACE inhibitor, ARB, or ARNI — Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Effective contraception: use a progestin implant or levonorgestrel-releasing intrauterine device until pregnancy is planned.

Note

  • Discontinue ACE inhibitor, ARB, and ARNI when pregnancy is planned due to teratogenicity.
  • Optimize beta blocker therapy; Avoid atenolol.
  • Avoid spironolactone.
  • Combined oral contraceptive is contraindicated.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy, birth and puerperium

Approach

  1. Risk class: mWHO III if LVEF 30–45%, mWHO II–III if LVEF > 45%.
  2. Watch with the Pregnancy Heart Team; If LVEF is 30–45%, perform close ECHO monitoring at the reference center.
  3. Stable women with LVEF > 40% and NYHA I–II usually tolerate pregnancy.
  4. Monitor closely in early pregnancy as rapid decompensation may occur after discontinuation of an ACE inhibitor, ARB, or ARNI.
  5. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  6. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  7. Initiate epidural analgesia early in labor and maintain it rigorously; It reduces systemic vascular resistance and provides an advantage in left ventricular systolic dysfunction.
  8. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  9. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  10. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  11. Closely monitor the first 24–72 hours after birth as the most critical period; Intensify postpartum monitoring.
  12. If acute pulmonary edema or hypoperfusion develops, switch to an acute decompensated heart failure approach.
  13. If anticoagulation is administered, plan discontinuation of heparin before planned delivery according to dose and risk of thrombosis (for neuroaxial intervention, at least 12 hours should elapse from the last prophylactic LMWH dose and at least 24 hours from the intermediate or therapeutic dose); plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).
  14. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • If congestion develops — Furosemide (oral): 20–40 mg once daily is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • After birth, in hemodynamically stable patient — Enalapril: 2.5 mg twice daily is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • Metoprolol succinate (long-acting, oral): Start at 25 mg once daily in NYHA II, 12.5 mg once daily in more severe heart failure; If tolerated, double the dose every 2 weeks to a maximum of 200 mg once a day.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • If documented intracardiac thrombus or LVEF < 35%, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • In other cases, if thromboprophylaxis is to be applied by individual decision — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH during pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • If creatinine clearance is < 30 mL/min, instead of postpartum prophylactic LMWH — UFH (SC, prophylactic): postpartum 5000 IU SC every 8–12 hours.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • ACE inhibitor, ARB and ARNI are contraindicated in pregnancy; If using it, stop it.
  • Optimize beta blocker therapy; Avoid atenolol.
  • Avoid spironolactone.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Metoprolol succinate (long-acting, oral): Started only in stable and euvolemic patients; It is not started new in acute decompensated heart failure; In symptomatic bradycardia, the dose is reduced.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Hypertrophic cardiomyopathy
Obstruction and symptom
Severe symptomatic LVOT obstruction (≥ 50 mmHg), severe symptomatic left ventricular dysfunction (LVEF < 50%), LVEF < 30% or NYHA III–IV
Period
Pre-conception (planning)

Approach

  1. Risk class: mWHO IV (symptomatic severe outflow tract obstruction ≥ 50 mmHg or severe symptomatic left ventricular dysfunction, LVEF < 50%); pregnancy is not recommended, recommend avoiding pregnancy.
  2. Discuss maternal risk, fetal effects, uncertainty of evidence, reproductive alternatives, and effective contraception in counseling with an expert multidisciplinary team.
  3. Assess symptoms, family history of sudden cardiac death, history of arrhythmia, ventricular function, left ventricular outflow tract (LVOT) gradient, and medications used.
  4. Conduct reproductive planning with the Pregnancy Heart Team, which includes a perinatologist, cardiologist and geneticist; The disease is inherited in an autosomal dominant manner.
  5. Clearly state in the record that pregnancy is contraindicated, the rationale, and the patient's understanding and preferences.
  6. Recommend tubal ligation; Share the decision without forcing it and evaluate the procedure and anesthesia risk in advance.

Treatment

  • Metoprolol (oral): immediate-release tartrate starts at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Contraception: use a progestin implant or levonorgestrel-releasing intrauterine device.

Note

  • Maintain beta blocker therapy as baseline therapy.
  • Avoid positive inotropes such as digoxin, dobutamine, and milrinone if left ventricular outflow tract obstruction is present (at rest or with provocation); They increase the outflow tract gradient.
  • Avoid excessive diuresis and vasodilation leading to hypovolemia.
  • Combined oral contraceptive is strictly contraindicated.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy, birth and puerperium

Approach

  1. Risk class: mWHO IV (symptomatic severe outflow tract obstruction ≥ 50 mmHg or severe symptomatic left ventricular dysfunction, LVEF < 50%); Discuss the risk to the mother in early counseling, continuation in a specialist center and the option of termination if legally and ethically appropriate.
  2. If pregnancy persists, continue care with the Pregnancy Heart Team at a tertiary or quaternary center with advanced cardiac, obstetrics, anesthesia, intensive care and neonatal expertise.
  3. Protect against hypovolemia (bleeding, excessive diuresis), vasodilation and tachycardia; These can suddenly worsen the obstruction and lead to syncope or fatal cardiogenic shock.
  4. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  5. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  6. Avoid excessive vasodilation in epidural analgesia.
  7. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  8. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  9. Closely monitor the first 24–72 hours after birth as the most critical period.
  10. Recommend tubal ligation before discharge.

Treatment

  • Metoprolol (oral): immediate-release tartrate starts at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • Maintain beta-blocker therapy as baseline therapy; Avoid atenolol.
  • Avoid positive inotropes such as digoxin, dobutamine, and milrinone if left ventricular outflow tract obstruction is present (at rest or with provocation); They increase the outflow tract gradient.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • Combined oral contraceptive is strictly contraindicated.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
None of these high-risk features
Period
Pre-conception (planning)

Approach

  1. Risk class: mWHO II–III in uncomplicated HCM; mWHO III if arrhythmic and/or moderate hemodynamic complications are present. Pregnancy is usually well tolerated when symptoms are mild and systolic function is preserved.
  2. Indicate in counseling that significant left ventricular outflow tract (LVOT) obstruction, previous syncope, arrhythmia, heart failure symptoms, or impaired systolic function increases risk.
  3. Assess symptoms, family history of sudden cardiac death, history of arrhythmia, ventricular function, outflow tract gradient, and medications used.
  4. Conduct pregnancy planning with the Pregnancy Heart Team, which includes a perinatologist, cardiologist and geneticist; The disease is inherited in an autosomal dominant manner.
  5. Document NYHA class, ECG, ECHO and baseline BNP/NT‑proBNP before pregnancy; Perform a stress test on the selected patient.
  6. Explain that increased preload during pregnancy often relieves obstruction, but hypovolemia, vasodilation, and tachycardia can lead to sudden worsening.

Treatment

  • Metoprolol (oral): immediate-release tartrate starts at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Effective contraception: use a progestin implant or levonorgestrel-releasing intrauterine device until pregnancy is planned.

Note

  • Maintain beta-blocker therapy as baseline therapy; Avoid atenolol.
  • Avoid positive inotropes such as digoxin, dobutamine, and milrinone if left ventricular outflow tract obstruction is present (at rest or with provocation); They increase the outflow tract gradient.
  • Avoid excessive diuresis and vasodilation leading to hypovolemia.
  • Combined oral contraceptive is contraindicated.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
Pregnancy, birth and puerperium

Approach

  1. Risk class: mWHO II–III in uncomplicated HCM, mWHO III with arrhythmic and/or moderate hemodynamic complications; follow with the Pregnancy Heart Team and perform close echocardiographic surveillance at a referral center.
  2. Protect against hypovolemia (bleeding, excessive diuresis), vasodilation and tachycardia; These can suddenly worsen the obstruction and lead to syncope or fatal cardiogenic shock.
  3. Prepare a written birth plan with the Pregnancy Heart Team by approximately week 32.
  4. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  5. Initiate epidural analgesia early in labor and maintain it rigorously; Avoid excessive vasodilation.
  6. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  7. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  8. Closely monitor the first 24–72 hours after birth as the most critical period.

Treatment

  • Metoprolol (oral): immediate-release tartrate starts at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • Maintain beta-blocker therapy as baseline therapy; Avoid atenolol.
  • Avoid positive inotropes such as digoxin, dobutamine, and milrinone if left ventricular outflow tract obstruction is present (at rest or with provocation); They increase the outflow tract gradient.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • Combined oral contraceptive is strictly contraindicated.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Arrhythmogenic right ventricular cardiomyopathy
Clinical situation
Stable (no active ventricular arrhythmia)
Period
Pre-conception (planning)

Approach

  1. Assess ventricular function and NYHA class even in the absence of active arrhythmia; If LVEF < 30% or NYHA III–IV, consider mWHO IV and recommend avoiding pregnancy; If there are no mWHO IV criteria, low-risk ARVC with genotype positive and no or mild phenotype is considered mWHO II–III, and moderate or severe ARVC is considered mWHO III.
  2. The main risk is fatal ventricular arrhythmia; Explain in counseling that the volume load of pregnancy may trigger right heart failure and arrhythmic storm.
  3. Conduct pregnancy planning with the Pregnancy Heart Team, which includes a perinatologist, cardiologist and geneticist.
  4. Assess ventricular function, ischemic disease, hereditary channelopathies, and family history of sudden cardiac death; Request electrophysiology consultation if necessary.
  5. Perform baseline ECG and ECHO; If ECHO is inadequate, use non-contrast cardiac MRI for right ventricular morphology.
  6. Provide arrhythmia control before pregnancy if it can be safely improved.
  7. ICD indications do not change with pregnancy; Plan for appropriate reprogramming of the ICD during pregnancy.

Treatment

  • Metoprolol (oral): immediate-release tartrate starts at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • If sotalol has been used before or if antiarrhythmic treatment is required, with the decision of the expert team, instead of metoprolol/bisoprolol — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Effective contraception: use a progestin implant or levonorgestrel-releasing intrauterine device until pregnancy is planned.

Note

  • Maintain beta blocker therapy, including sotalol.
  • Avoid amiodarone; Use only in life-threatening arrhythmias.
  • Combined oral contraceptive is contraindicated.
  • Sotalol is used with caution in low ejection fraction; Fetal growth is monitored due to the beta blocker effect; It is not given with another beta blocker.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Pregnancy, birth and puerperium

Approach

  1. Assess ventricular function and NYHA class even in the absence of active arrhythmia; If LVEF < 30% or NYHA III–IV, accept mWHO IV and seek early risk and pregnancy options counseling with specialist Pregnancy Heart Team; If there are no mWHO IV criteria, low-risk ARVC with genotype positive and no or mild phenotype is considered mWHO II–III, and moderate or severe ARVC is considered mWHO III.
  2. Follow closely with the Pregnancy Heart Team, which is run jointly by cardiology and obstetrics at the Tertiary center; Perform regular ECG/Holter, monitor for signs of right heart failure and arrhythmic storm.
  3. Prepare a written antenatal emergency plan; Determine the birth plan, intrapartum emergency algorithm and team roles in writing by approximately the 32nd week.
  4. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  5. Initiate epidural analgesia early in labor and maintain it meticulously.
  6. Use assisted vaginal birth in conjunction with cardiology according to individual risk assessment to shorten the second stage.
  7. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  8. Intensify monitoring during puerperium: plan early check-up before discharge, teach warning symptoms, and review compatibility of antiarrhythmic therapy with breast-feeding.

Treatment

  • Metoprolol (oral): immediate-release tartrate starts at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • If sotalol has been used before or if antiarrhythmic treatment is required, with the decision of the expert team, instead of metoprolol/bisoprolol — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Reprogram ICD appropriately.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • Do not discontinue beta blocker treatment, including sotalol, throughout pregnancy and the postpartum period.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor the newborn of a mother using beta blockers for bradycardia and hypoglycemia.
  • Combined oral contraceptive is strictly contraindicated.
  • Sotalol is used with caution in low ejection fraction; Fetal growth is monitored due to the beta blocker effect; It is not given with another beta blocker.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Attack of ventricular arrhythmia (during pregnancy or puerperium)
Hemodynamics
Hemodynamic instability (hypotension, hypoperfusion, chest pain, acute dyspnea or altered consciousness)

Approach

  1. Start treatment and ensure maternal stabilization without waiting for detailed rhythm classification.
  2. Quickly restore maternal circulation; this is the strongest modifiable predictor of fetal outcome.
  3. After stabilization, evaluate electrolytes, thyroid function, complete blood count, and ECHO.
  4. Refer to tertiary center and multidisciplinary committee.
  5. Conduct maternal rhythm therapy in conjunction with fetal monitoring.
  6. Prepare the birth and postpartum follow-up plan in writing.
  7. Schedule early follow-up prior to discharge, teach warning symptoms of arrhythmia recurrence, and review compatibility of antiarrhythmic therapy with breast-feeding.

Treatment

  • In VT with a pulse — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT or VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • In life-threatening refractory arrhythmia — Amiodarone: 150 mg IV over 10 minutes (may be repeated if arrhythmia recurs); followed by 1 mg/min IV infusion for 6 hours, then 0.5 mg/min for 18 hours; 300 mg IV/IO bolus in cardiac arrest, second dose 150 mg if necessary.
  • Reprogram ICD appropriately.
  • Catheter ablation: perform in refractory arrhythmia in an experienced center with individual risk-benefit evaluation.

Note

  • Pregnancy week is not a reason to postpone cardioversion or defibrillation.
  • Maintain beta blocker therapy; If sotalol is used, carry out under specialist supervision and with close ECG monitoring; Reconsider sotalol if cardiogenic shock or decompensated heart failure develops.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Amiodarone: Avoided during pregnancy due to risk of fetal adverse effects; It is used only in life-threatening arrhythmia that cannot be controlled with other treatments; If administered, the fetus is monitored for bradycardia, growth restriction, and thyroid function.
Hemodynamically stable

Approach

  1. Obtain a 12-lead ECG, perform rhythm monitoring if necessary; Request electrolytes, thyroid function, complete blood count and ECHO.
  2. Assess for triggers and concomitant structural heart disease.
  3. Refer to tertiary center and multidisciplinary committee.
  4. Conduct maternal rhythm therapy in conjunction with fetal monitoring.
  5. Prepare the birth and postpartum follow-up plan in writing.
  6. Schedule early follow-up prior to discharge, teach warning symptoms of arrhythmia recurrence, and review compatibility of antiarrhythmic therapy with breast-feeding.

Treatment

  • Regardless of hemodynamic status in sustained VT (without waiting if drug conversion has been attempted and the drug is ineffective or contraindicated) — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • Lidocaine: 1–1.5 mg/kg IV bolus at 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • Procainamide: 15 mg/kg IV loading over at least 30 minutes and the infusion rate not to exceed 50 mg/min (total loading not more than 17 mg/kg); followed by 1–4 mg/min IV infusion.
  • In life-threatening refractory arrhythmia — Amiodarone: 150 mg IV over 10 minutes (may be repeated if arrhythmia recurs); followed by 1 mg/min IV infusion for 6 hours, then 0.5 mg/min for 18 hours; 300 mg IV/IO bolus in cardiac arrest, second dose 150 mg if necessary.
  • If pulseless VT or VF develops — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), if unknown, highest energy of the device; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • Reprogram ICD appropriately.
  • Catheter ablation: perform in refractory arrhythmia in an experienced center with individual risk-benefit evaluation.

Note

  • Maintain beta blocker therapy; If sotalol is used, carry out under specialist supervision and with close ECG monitoring; Reconsider sotalol if cardiogenic shock or decompensated heart failure develops.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Procainamide: Stop loading if arrhythmia is suppressed, hypotension develops, or QRS duration is prolonged by more than 50%; It is avoided in prolonged QT and heart failure.
  • Procainamide: Uterine irritability and preterm labor have been reported with IV use during pregnancy.
  • Amiodarone: Avoided during pregnancy due to risk of fetal adverse effects; It is used only in life-threatening arrhythmia that cannot be controlled with other treatments; If administered, the fetus is monitored for bradycardia, growth restriction, and thyroid function.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
Acute decompensated heart failure / cardiogenic shock
Clinical picture
Congestion/acute pulmonary edema (no hypoperfusion)
Period
Pregnancy continues

Approach

  1. In case of signs of heart failure (increased jugular venous pressure, lung rales, dyspnea at rest, orthopnea, marked edema, hepatomegaly, oxygen desaturation), perform urgent cardiovascular evaluation and refer to the Pregnancy Heart Team.
  2. Evaluate ventricular function and heart failure by ECHO; LVEF < 45% supports the diagnosis of peripartum cardiomyopathy.
  3. If the suspicion of pulmonary embolism persists, do not terminate the evaluation with the ECHO result; Pursue appropriate V/Q scintigraphy or CT pulmonary angiography based on chest radiography and clinical evaluation.
  4. Measure BNP/NT‑proBNP and troponin; BNP/NT‑proBNP is usually within the normal range in normal pregnancy and significant elevation supports heart failure, whereas elevated troponin is associated with poor prognosis.
  5. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  6. Monitor fetal growth with serial USG and fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  7. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  8. Closely monitor the first 24–72 hours after birth as the most critical period.
  9. Individually evaluate the need for anticoagulation if there is significant low LVEF, atrial arrhythmia, or ventricular thrombus in other cardiomyopathies.
  10. Plan discontinuation of heparin before planned delivery based on dose and risk of thrombosis; for neuraxial intervention, at least 12 hours must elapse after the last prophylactic dose of LMWH and at least 24 hours after the intermediate or therapeutic dose; plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).

Treatment

  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In congestion and adequate blood pressure — Nitroglycerin: Start with 5 µg/min IV infusion, increasing every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • Hydralazine + isosorbide dinitrate (oral): start with hydralazine 25–37.5 mg and isosorbide dinitrate 20 mg 3 times daily; Target hydralazine 75 mg and isosorbide dinitrate 40 mg 3 times a day, increasing every 1–2 weeks.
  • In PPCM with LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • ACE inhibitor, ARB and ARNI are contraindicated during pregnancy.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • Avoid vasodilation and excessive diuresis leading to hypovolemia in hypertrophic cardiomyopathy; these can suddenly worsen outflow tract obstruction.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Postpartum

Approach

  1. In case of signs of heart failure (increased jugular venous pressure, lung rales, dyspnea at rest, orthopnea, marked edema, hepatomegaly, oxygen desaturation), perform urgent cardiovascular evaluation and refer to the Pregnancy Heart Team.
  2. Evaluate ventricular function and heart failure by ECHO; LVEF < 45% supports the diagnosis of peripartum cardiomyopathy.
  3. If the suspicion of pulmonary embolism persists, do not terminate the evaluation with the ECHO result; Pursue appropriate V/Q scintigraphy or CT pulmonary angiography based on chest radiography and clinical evaluation.
  4. Measure BNP/NT‑proBNP and troponin; Significant elevation of BNP/NT‑proBNP supports heart failure, elevation of troponin is associated with poor prognosis.
  5. Closely monitor the first 24–72 hours after birth as the most critical period.
  6. Individually evaluate the need for anticoagulation if there is significant low LVEF, atrial arrhythmia, or ventricular thrombus in other cardiomyopathies.
  7. If anticoagulation remains indicated and there is no significant bleeding or significant risk of bleeding, plan for postpartum resumption; The general obstetric range is 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  8. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In congestion and adequate blood pressure — Nitroglycerin: Start with 5 µg/min IV infusion, increasing every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • If there is left ventricular systolic dysfunction (low LVEF), regardless of the cause, after birth in the hemodynamically stable patient — Enalapril: 2.5 mg twice daily is started; The target is 10–20 mg twice a day, doubling the dose every 1–2 weeks.
  • Bromocriptine may be considered if PPCM is the cause: if LVEF < 30%, 2.5 mg twice daily for 2 weeks, then 2.5 mg once daily for 6 weeks; If LVEF > 30%, 2.5 mg once daily for 1 week; Use at least prophylactic dose of LMWH together.
  • In PPCM with LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours; If bromocriptine is used, add at least a prophylactic dose of LMWH.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient, bromocriptine use) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • If bromocriptine is started in peripartum cardiomyopathy, breastfeeding cannot be continued during treatment; Discuss the baby's feeding plan before treatment.
  • Bromocriptine is not used in uncontrolled hypertension; If you have preeclampsia/eclampsia or postpartum hypertension, make use decisions with the Pregnancy Heart Team. Monitor blood pressure, especially in the first days and during dose increases; In case of new hypertension, severe or persistent headache, visual impairment, or chest pain, discontinue the medication and seek immediate evaluation.
  • If LVEF is exactly 30%, individualize the dosing schedule with the expert team.
  • Excessive diuresis reduces intravascular volume; Titrate furosemide dose.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • Avoid vasodilation and excessive diuresis leading to hypovolemia in hypertrophic cardiomyopathy; these can suddenly worsen outflow tract obstruction.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • Combined oral contraceptive is strictly contraindicated.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Do not give an ACE inhibitor in HCM with left ventricular outflow tract obstruction.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
  • Enalapril: Contraindicated in pregnancy; Can be used during breastfeeding. If serum sodium is <130 mEq/L or serum creatinine is >1.6 mg/dL, start with 2.5 mg once daily under close supervision; The dose is increased every 4 days at the earliest unless there is severe hypotension or significant deterioration of renal function.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Hypoperfusion / cardiogenic shock — other than inotrope-resistant shock
Period
Pregnancy continues

Approach

  1. Manage with the Pregnancy Heart Team in the intensive care unit, consisting of cardiology, perinatology, anesthesia, intensive care, cardiac surgery and neonatal specialists.
  2. Plan early referral to a center that can provide mechanical circulatory support (VA-ECMO or Impella).
  3. Evaluate ventricular function and heart failure by ECHO; LVEF < 45% supports the diagnosis of peripartum cardiomyopathy.
  4. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  5. If the fetus has reached the limit of viability in cardiogenic shock, recommend combined spinal/epidural analgesia or emergency cesarean section under general anesthesia, taking into account the gestational age, accompanying diseases and the facilities of the center; Don't expect refractoriness. Prioritize maternal stabilization before the limit of viability and make the birth decision with the Pregnancy Heart Team.
  6. Monitor fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  7. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  8. Closely monitor the first 24–72 hours after birth as the most critical period.
  9. Individually evaluate the need for anticoagulation if there is significant low LVEF, atrial arrhythmia, or ventricular thrombus in other cardiomyopathies.
  10. Plan discontinuation of heparin before planned delivery based on dose and risk of thrombosis; for neuraxial intervention, at least 12 hours must elapse after the last prophylactic dose of LMWH and at least 24 hours after the intermediate or therapeutic dose; plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).

Treatment

  • Dobutamine: Start with 2–5 µg/kg/min IV infusion, titrating to 2–20 µg/kg/min according to response.
  • Milrinone: 0.375–0.75 µg/kg/min IV infusion; If creatinine clearance is 10–50 mL/min, start with 0.0625–0.125 µg/kg/min and titrate carefully and do not exceed 0.375 µg/kg/min in significant renal impairment; If creatinine clearance is <10 mL/min, another agent is chosen; Hypotension may be prolonged in renal impairment.
  • Levosimendan: 0.1 µg/kg/min IV infusion without loading dose; 0.05–0.2 µg/kg/min depending on response; The recommended infusion time is 24 hours.
  • If hypotension persists despite inotrope — Noradrenaline: Start with 0.05 µg/kg/min IV infusion, titrate according to mean arterial pressure and organ perfusion; usual range 0.05–0.4 µg/kg/min, up to 1.0 µg/kg/min if necessary.
  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If accompanied by congestion — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Venoarterial ECMO (VA-ECMO) or percutaneous left ventricular assist device (Impella): apply as a bridge to transplant or myocardial recovery in inotrope-resistant shock.
  • In PPCM with LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • Positive inotropes such as digoxin, dobutamine and milrinone are contraindicated if there is left ventricular outflow tract obstruction in hypertrophic cardiomyopathy; Choose a vasoconstrictor that does not have an inotropic effect in hypotension with an expert team; Avoid vasodilation and excessive diuresis leading to hypovolemia.
  • Individualize shock treatment in HCM without obstruction with impaired systolic function by an expert team according to ECHO and hemodynamics.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • ACE inhibitor, ARB and ARNI are contraindicated during pregnancy.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Milrinone: Since it crosses the placenta during pregnancy, it is used only when the benefit outweighs the risk.
  • Levosimendan: Not used in creatinine clearance <30 mL/min, severe liver failure, significant mechanical obstruction affecting ventricular filling or outflow, history of torsade de pointes, severe hypotension, and tachycardia; In hypotension, if necessary, it is given together with noradrenaline; The hemodynamic effect may last up to 9 days after the 24-hour infusion ends.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Postpartum

Approach

  1. Manage with the Pregnancy Heart Team in the intensive care unit, consisting of cardiology, perinatology, anesthesia, intensive care, cardiac surgery and neonatal specialists.
  2. Plan early referral to a center that can provide mechanical circulatory support (VA-ECMO or Impella).
  3. Evaluate ventricular function and heart failure by ECHO; LVEF < 45% supports the diagnosis of peripartum cardiomyopathy.
  4. Measure BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  5. Closely monitor the first 24–72 hours after birth as the most critical period.
  6. Individually evaluate the need for anticoagulation if there is significant low LVEF, atrial arrhythmia, or ventricular thrombus in other cardiomyopathies.
  7. If anticoagulation remains indicated and there is no significant bleeding or significant risk of bleeding, plan for postpartum resumption; The general obstetric range is 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  8. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Dobutamine: Start with 2–5 µg/kg/min IV infusion, titrating to 2–20 µg/kg/min according to response.
  • Milrinone: 0.375–0.75 µg/kg/min IV infusion; If creatinine clearance is 10–50 mL/min, start with 0.0625–0.125 µg/kg/min and titrate carefully and do not exceed 0.375 µg/kg/min in significant renal impairment; If creatinine clearance is <10 mL/min, another agent is chosen; Hypotension may be prolonged in renal impairment.
  • Levosimendan: 0.1 µg/kg/min IV infusion without loading dose; 0.05–0.2 µg/kg/min depending on response; The recommended infusion time is 24 hours.
  • If hypotension persists despite inotrope — Noradrenaline: Start with 0.05 µg/kg/min IV infusion, titrate according to mean arterial pressure and organ perfusion; usual range 0.05–0.4 µg/kg/min, up to 1.0 µg/kg/min if necessary.
  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If accompanied by congestion — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Venoarterial ECMO (VA-ECMO) or percutaneous left ventricular assist device (Impella): apply as a bridge to transplant or myocardial recovery in inotrope-resistant shock.
  • Bromocriptine may be considered in moderate–severe PPCM: 2.5 mg twice daily for 2 weeks, followed by 2.5 mg once daily for 6 weeks; Add at least prophylactic dose of LMWH.
  • In PPCM with LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient, bromocriptine use) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • If bromocriptine is started in peripartum cardiomyopathy, breastfeeding cannot be continued during treatment; Discuss the baby's feeding plan before treatment.
  • Bromocriptine is not used in uncontrolled hypertension; If you have preeclampsia/eclampsia or postpartum hypertension, make use decisions with the Pregnancy Heart Team. Monitor blood pressure, especially in the first days and during dose increases; In case of new hypertension, severe or persistent headache, visual impairment, or chest pain, discontinue the medication and seek immediate evaluation.
  • If bromocriptine is considered if LVEF ≥ 30% with severe shock, individualize the dosing schedule with the specialist team.
  • Positive inotropes such as digoxin, dobutamine and milrinone are contraindicated if there is left ventricular outflow tract obstruction in hypertrophic cardiomyopathy; Choose a vasoconstrictor that does not have an inotropic effect in hypotension with an expert team; Avoid vasodilation and excessive diuresis leading to hypovolemia.
  • Individualize shock treatment in HCM without obstruction with impaired systolic function by an expert team according to ECHO and hemodynamics.
  • Excessive diuresis reduces intravascular volume; Titrate furosemide dose.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Combined oral contraceptive is strictly contraindicated.
  • Levosimendan: Not used in creatinine clearance <30 mL/min, severe liver failure, significant mechanical obstruction affecting ventricular filling or outflow, history of torsade de pointes, severe hypotension, and tachycardia; In hypotension, if necessary, it is given together with noradrenaline; The hemodynamic effect may last up to 9 days after the 24-hour infusion ends.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Cardiogenic shock resistant to inotropes
Period
Pregnancy continues

Approach

  1. Manage with the Pregnancy Heart Team in the intensive care unit, consisting of cardiology, perinatology, anesthesia, intensive care, cardiac surgery and neonatal specialists.
  2. If the fetus has reached the limit of viability in cardiogenic shock, recommend combined spinal/epidural analgesia or emergency cesarean section under general anesthesia, taking into account the gestational age, accompanying diseases and the facilities of the center; Don't expect refractoriness. Prioritize maternal stabilization before the limit of viability and make the birth decision with the Pregnancy Heart Team.
  3. Caesarean section can be performed under VA-ECMO or Impella support.
  4. Monitor with ECHO, BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  5. Monitor fetal well-being with umbilical artery and middle cerebral artery (MCA) Doppler.
  6. If the fetus has reached viability, perform intermittent or continuous fetal heart rate monitoring during hospitalization for maternal decompensation according to gestational age and clinical status; monitor continuously during anesthesia administration, labor, and delivery. Do not delay maternal stabilization for fetal assessment.
  7. Closely monitor the first 24–72 hours after birth as the most critical period.
  8. Individually evaluate the need for anticoagulation if there is significant low LVEF, atrial arrhythmia, or ventricular thrombus in other cardiomyopathies.
  9. Plan discontinuation of heparin before planned delivery based on dose and risk of thrombosis; for neuraxial intervention, at least 12 hours must elapse after the last prophylactic dose of LMWH and at least 24 hours after the intermediate or therapeutic dose; plan postpartum resumption time if indication persists and there is no significant bleeding or significant risk of bleeding (general obstetric range 4–6 hours after vaginal birth, 6–12 hours after cesarean section).

Treatment

  • Bridge to transplant or myocardial recovery with venoarterial ECMO (VA-ECMO) or percutaneous left ventricular assist device (Impella).
  • Dobutamine: Start with 2–5 µg/kg/min IV infusion, titrating to 2–20 µg/kg/min according to response.
  • Milrinone: 0.375–0.75 µg/kg/min IV infusion; If creatinine clearance is 10–50 mL/min, start with 0.0625–0.125 µg/kg/min and titrate carefully and do not exceed 0.375 µg/kg/min in significant renal impairment; If creatinine clearance is <10 mL/min, another agent is chosen; Hypotension may be prolonged in renal impairment.
  • Levosimendan: 0.1 µg/kg/min IV infusion without loading dose; 0.05–0.2 µg/kg/min depending on response; The recommended infusion time is 24 hours.
  • If hypotension persists despite inotrope — Noradrenaline: Start with 0.05 µg/kg/min IV infusion, titrate according to mean arterial pressure and organ perfusion; usual range 0.05–0.4 µg/kg/min, up to 1.0 µg/kg/min if necessary.
  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If accompanied by congestion — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • In PPCM with LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is < 30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • Positive inotropes such as digoxin, dobutamine and milrinone are contraindicated if there is left ventricular outflow tract obstruction in hypertrophic cardiomyopathy; Choose a vasoconstrictor that does not have an inotropic effect in hypotension with an expert team; Avoid vasodilation and excessive diuresis leading to hypovolemia.
  • Individualize shock treatment in HCM without obstruction with impaired systolic function by an expert team according to ECHO and hemodynamics.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • ACE inhibitor, ARB and ARNI are contraindicated during pregnancy.
  • Avoid spironolactone during pregnancy; Review current therapy due to possible antiandrogenic effects and limited pregnancy safety data.
  • If UFH is used, before neuraxial intervention: stop IV infusion at least 4–6 hours before; Wait 4–6 hours after SC 5000 IU (2–3 times a day), wait 12 hours after SC 7500–10 000 IU every 12 hours, wait 24 hours after single dose > 10 000 IU or total daily > 20 000 IU; In all cases, verify that coagulation (aPTT) is normal before intervention. Check platelet count if UFH has been used for more than 4 days.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Milrinone: Since it crosses the placenta during pregnancy, it is used only when the benefit outweighs the risk.
  • Levosimendan: Not used in creatinine clearance <30 mL/min, severe liver failure, significant mechanical obstruction affecting ventricular filling or outflow, history of torsade de pointes, severe hypotension, and tachycardia; In hypotension, if necessary, it is given together with noradrenaline; The hemodynamic effect may last up to 9 days after the 24-hour infusion ends.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Postpartum

Approach

  1. Manage with the Pregnancy Heart Team in the intensive care unit, consisting of cardiology, perinatology, anesthesia, intensive care, cardiac surgery and neonatal specialists.
  2. Monitor with ECHO, BNP/NT‑proBNP and troponin; Elevated troponin is associated with poor prognosis.
  3. Closely monitor the first 24–72 hours after birth as the most critical period.
  4. Individually evaluate the need for anticoagulation if there is significant low LVEF, atrial arrhythmia, or ventricular thrombus in other cardiomyopathies.
  5. If anticoagulation remains indicated and there is no significant bleeding or significant risk of bleeding, plan for postpartum resumption; The general obstetric range is 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  6. Write the indication for anticoagulation and the end/reassessment date in the discharge plan: If anticoagulation is administered due to LVEF < 35%, continue for at least 6 weeks postpartum and reassess based on LVEF; If bromocriptine is used, maintain at least a prophylactic dose of LMWH throughout treatment; In case of intracardiac thrombus, embolism or permanent indication, the expert team determines the duration.

Treatment

  • Bridge to transplant or myocardial recovery with venoarterial ECMO (VA-ECMO) or percutaneous left ventricular assist device (Impella).
  • Dobutamine: Start with 2–5 µg/kg/min IV infusion, titrating to 2–20 µg/kg/min according to response.
  • Milrinone: 0.375–0.75 µg/kg/min IV infusion; If creatinine clearance is 10–50 mL/min, start with 0.0625–0.125 µg/kg/min and titrate carefully and do not exceed 0.375 µg/kg/min in significant renal impairment; If creatinine clearance is <10 mL/min, another agent is chosen; Hypotension may be prolonged in renal impairment.
  • Levosimendan: 0.1 µg/kg/min IV infusion without loading dose; 0.05–0.2 µg/kg/min depending on response; The recommended infusion time is 24 hours.
  • If hypotension persists despite inotrope — Noradrenaline: Start with 0.05 µg/kg/min IV infusion, titrate according to mean arterial pressure and organ perfusion; usual range 0.05–0.4 µg/kg/min, up to 1.0 µg/kg/min if necessary.
  • In Hypoxemia — Oxygen: Oxygen is administered if SpO2 is below 90% or PaO2 is below 60 mmHg; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If accompanied by congestion — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in those who have not used diuretics before; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Bromocriptine may be considered in moderate–severe PPCM: 2.5 mg twice daily for 2 weeks, followed by 2.5 mg once daily for 6 weeks; Add at least prophylactic dose of LMWH.
  • In PPCM with LVEF < 35% or intracardiac thrombus, administer therapeutic dose of LMWH: enoxaparin 1 mg/kg SC every 12 hours.
  • If therapeutic anticoagulation is not applied and thromboprophylaxis is indicated (inpatient, bromocriptine use) — Enoxaparin (prophylactic): 40 mg SC once a day for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance < 30 mL/min, instead of therapeutic LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If creatinine clearance is < 30 mL/min, instead of prophylactic LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • In active HIT, stop all heparin and start a non-heparin anticoagulant at therapeutic intensity even if thrombosis has not been demonstrated; by decision of the specialist team — Fondaparinux: for prophylaxis, 2.5 mg SC once daily; for active HIT or thrombosis requiring treatment, 5 mg SC once daily for body weight <50 kg, 7.5 mg SC once daily for 50–100 kg, and 10 mg SC once daily for >100 kg.
  • In the past history of HIT, instead of LMWH and UFH, at the intensity required by the current indication — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge.

Note

  • If bromocriptine is started in peripartum cardiomyopathy, breastfeeding cannot be continued during treatment; Discuss the baby's feeding plan before treatment.
  • Bromocriptine is not used in uncontrolled hypertension; If you have preeclampsia/eclampsia or postpartum hypertension, make use decisions with the Pregnancy Heart Team. Monitor blood pressure, especially in the first days and during dose increases; In case of new hypertension, severe or persistent headache, visual impairment, or chest pain, discontinue the medication and seek immediate evaluation.
  • If bromocriptine is considered if LVEF ≥ 30% with severe shock, individualize the dosing schedule with the specialist team.
  • Positive inotropes such as digoxin, dobutamine and milrinone are contraindicated if there is left ventricular outflow tract obstruction in hypertrophic cardiomyopathy; Choose a vasoconstrictor that does not have an inotropic effect in hypotension with an expert team; Avoid vasodilation and excessive diuresis leading to hypovolemia.
  • Individualize shock treatment in HCM without obstruction with impaired systolic function by an expert team according to ECHO and hemodynamics.
  • Excessive diuresis reduces intravascular volume; Titrate furosemide dose.
  • Do not administer vasodilators in hypotension or cardiogenic shock.
  • If neuraxial block was performed, initiate prophylactic LMWH at least 12 hours after the procedure, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In case of shock, high bleeding risk or imminent need for emergency intervention, choose a short-acting IV non-heparin agent with an expert team.
  • Combined oral contraceptive is strictly contraindicated.
  • Levosimendan: Not used in creatinine clearance <30 mL/min, severe liver failure, significant mechanical obstruction affecting ventricular filling or outflow, history of torsade de pointes, severe hypotension, and tachycardia; In hypotension, if necessary, it is given together with noradrenaline; The hemodynamic effect may last up to 9 days after the 24-hour infusion ends.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Woman with left ventricular assist device (LVAD)
Period
Pre-pregnancy counseling

Approach

  1. Risk class: mWHO IV; Pregnancy is absolutely contraindicated.
  2. Explain the rationale: the high doses of VKA (warfarin) required to prevent device thrombosis are teratogenic and it is unclear whether the device can provide the pulsatile flow required for fetal circulation.
  3. Address fetal effects, uncertainty of evidence, reproductive alternatives, and effective contraception in counseling with the expert multidisciplinary team.
  4. Clearly state in the record that pregnancy is contraindicated, the rationale, and the patient's understanding and preferences.
  5. Recommend tubal ligation; Share the decision without forcing it and evaluate the procedure and anesthesia risk in advance.

Treatment

  • Contraception: use a progestin implant or levonorgestrel-releasing intrauterine device.

Note

  • Combined oral contraceptive is strictly contraindicated.
  • Consider anticoagulation status and bleeding profile when choosing a contraception method.
Pregnancy has occurred

Approach

  1. Risk class: mWHO IV; Pregnancy is absolutely contraindicated.
  2. Discuss maternal risk in early counseling, continuation in specialist centre, and the option of termination if legally and ethically appropriate.
  3. If pregnancy persists, continue care with the Pregnancy Heart Team at a tertiary or quaternary center with advanced cardiac, obstetrics, anesthesia, intensive care and neonatal expertise.
  4. VKA (warfarin) crosses the placenta and anticoagulates the fetus; Explain that early exposure may cause embryopathy and late exposure may cause fetal bleeding, including intracranial hemorrhage.
  5. Make decisions jointly with the woman and record them.

Treatment

  • Device anticoagulation: continue to prevent device thrombosis; Determine the choice of agent and dose during pregnancy with the expert team.
  • Contraception: insertion of a progestin implant or levonorgestrel-releasing intrauterine device before discharge after termination of pregnancy or birth.

Note

  • Combined oral contraceptive is strictly contraindicated.
  • Consider anticoagulation status and bleeding profile when choosing a contraception method.
Pregnancy after heart transplantation
Period
Pre-conception (planning)
Post-transplant time and graft status
The waiting period of at least 1–2 years determined by the expert team has not been completed, the risk of acute rejection has not been reduced sufficiently, or the graft function is not stable

Approach

  1. Delay pregnancy until at least 1–2 years after transplantation, until the risk of acute rejection has decreased and graft function has stabilized.
  2. Plan your pregnancy with the Pregnancy Heart Team.

Treatment

  • Effective contraception: use a long-acting reversible method (intrauterine device, progestin implant) in most women during the deferral period.
  • Azathioprine: start instead of mycophenolate mofetil when pregnancy is planned; Individualize the dose with the expert team.
  • Maintain tacrolimus or cyclosporine with close monitoring of blood levels.

Note

  • Mycophenolate mofetil is significantly teratogenic; When pregnancy is planned, stop at least 6-8 weeks before conception.
The specified waiting period has been completed, the risk of acute rejection has decreased and the graft function is stable.

Approach

  1. Pregnancy can be planned; risk class mWHO III.
  2. Manage with the multidisciplinary Pregnancy Heart Team at the specialist center from pre-pregnancy.
  3. State in counseling that complications are common and intensive antenatal, intrapartum and postpartum monitoring is required.
  4. Explain that the risks of preeclampsia, preterm birth, and low birth weight remain high.
  5. Document NYHA class, ECG, ECHO and baseline BNP/NT‑proBNP before pregnancy.

Treatment

  • Azathioprine: start instead of mycophenolate mofetil when pregnancy is planned; Individualize the dose with the expert team.
  • Maintain tacrolimus or cyclosporine with close monitoring of blood levels.

Note

  • Mycophenolate mofetil is significantly teratogenic; Stop at least 6-8 weeks before getting pregnant.
Pregnancy continues

Approach

  1. Pregnancy at least 1–2 years after transplantation and with stable graft function is mWHO III; Manage with the Pregnancy Heart Team at the specialist centre.
  2. Perform cardiology follow-up usually every 1–2 months.
  3. Create your birth plan early.
  4. Perform intensive antenatal, intrapartum and postpartum monitoring.
  5. Monitor closely for preeclampsia, preterm birth, and low birth weight; monitor fetal growth.
  6. Plan your birth in a center with neonatal intensive care facilities.
  7. Closely monitor tacrolimus/cyclosporine blood levels and graft function after birth with the transplant team; Readjust doses changed during pregnancy based on postpartum measurements.

Treatment

  • Maintain tacrolimus or cyclosporine with close monitoring of blood levels.
  • Azathioprine: use instead of mycophenolate mofetil; Individualize the dose with the expert team.
  • Aspirin if there are no contraindications: 75–150 mg/day, at bedtime; give from week 12 until week 36/37.

Note

  • Mycophenolate mofetil is markedly teratogenic; do not use it during pregnancy.

Arrhythmias

Presentation with palpitations, syncope, or arrhythmia—initial evaluation
Hemodynamic status
Hemodynamic instability (hypotension, hypoperfusion, persistent chest pain, acute dyspnea or altered consciousness)

Approach

  1. First evaluate pulse and monitor rhythm: if there is no pulse, proceed to cardiac arrest management, in unstable bradycardia, proceed to bradycardia management; Perform synchronized cardioversion in pulsed tachyarrhythmia causing instability (usually heart rate ≥ 150/min), treat the underlying cause in sinus tachycardia; Provide maternal stabilization without waiting for detailed rhythm classification.
  2. After stabilization, evaluate triggers and structural heart disease with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO.
  3. After stabilization, select treatment according to arrhythmia type (SVT, AF/atrial flutter, VT).
  4. If there is syncope/presyncope, previous sustained VT/VF, ventricular dysfunction/cardiomyopathy, complex congenital heart disease, new arrhythmia in hypertensive disease of pregnancy, or family history of sudden cardiac death at an early age, refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee.
  5. Conduct maternal rhythm management in conjunction with fetal monitoring.
  6. Put the birth and puerperium follow-up plan in writing.
  7. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  8. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Pulsating, unstable tachyarrhythmia — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • In emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In the absence of prolonged QT and torsade, emergency control of ventricular arrhythmia (in advanced center) — Procainamide: 15 mg/kg IV loading over at least 30 minutes and with an infusion rate not exceeding 50 mg/min (total loading not more than 17 mg/kg); followed by 1–4 mg/min IV infusion.
  • In life-threatening refractory VT/VF in the absence of prolonged QT and torsades — Amiodarone: 150 mg IV over 10 minutes (may be repeated if arrhythmia recurs); followed by 1 mg/min IV infusion for 6 hours, then 0.5 mg/min for 18 hours; 300 mg IV/IO bolus in cardiac arrest, second dose 150 mg if necessary.

Note

  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Procainamide: Stop loading if arrhythmia is suppressed, hypotension develops, or QRS duration is prolonged by more than 50%; It is avoided in prolonged QT and heart failure.
  • Procainamide: Uterine irritability and preterm labor have been reported with IV use during pregnancy.
  • Amiodarone: Avoided during pregnancy due to risk of fetal adverse effects; It is used only in life-threatening arrhythmia that cannot be controlled with other treatments; If administered, the fetus is monitored for bradycardia, growth restriction, and thyroid function.
Hemodynamically stable
Risk phenotype
No high-risk features

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; evaluate electrolytes, thyroid function, complete blood count, and ECHO; Look for triggers and structural heart disease.
  2. Select treatment according to arrhythmia type (SVT, AF/atrial flutter, VT).
  3. Control rhythm load early; Conduct maternal rhythm management together with fetal monitoring.
  4. Put the birth and puerperium follow-up plan in writing.
  5. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  6. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • First step in SVT with narrow QRS—Vagal maneuver: modified Valsalva—15 seconds of straining (approximately 40 mmHg) against the closed glottis in a semi-sitting position, followed immediately by lying supine and lifting the legs 45° and holding for 15 seconds; Carotid sinus massage—first auscultating the carotid murmur, pressing the unilateral carotid sinus for 5–10 seconds.
  • For acute SVT termination (first drug) — Adenosine: 6 mg rapid IV bolus (in 1–2 seconds), immediately followed by flushing with 20 mL saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • In the absence of structural heart disease, in rhythm control — Flecainide: start at 50 mg orally every 12 hours (in ventricular arrhythmia, 50–100 mg every 12 hours); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • Rhythm control — Sotalol: start at 80 mg by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Structural or congenital heart disease (non-cardiomyopathy)

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. Classify the documented rhythm as SVT, AF/flutter, or VT and select treatment based on arrhythmia type.
  3. Consider other concomitant risk phenotypes together; If accompanied by hypertensive disease, keep the observation threshold low in the hospital; Consider advanced rhythm examination and genetic counseling in cases of suspected hereditary arrhythmia.
  4. Risk stratify with mWHO, CARPREG II (previous arrhythmia 3 points) and ZAHARA in congenital heart disease.
  5. Plan close follow-up at a tertiary center and evaluate by a multidisciplinary committee; Perform regular ECG/Holter monitoring.
  6. Prepare a written antenatal emergency plan; establish the intrapartum emergency algorithm and team role assignments in writing.
  7. Monitor fetal growth for risk of preterm birth and fetal growth restriction; If the mother or father has congenital heart disease, discuss the risk of recurrence in the fetus and recommend fetal echocardiography at 18–22 weeks
  8. Apply pharmacological treatment aggressively in congenital heart disease as arrhythmia may trigger decompensation.
  9. Determine the mode of delivery according to obstetric indications; do not routinely perform cesarean delivery.
  10. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • First step in SVT with narrow QRS—Vagal maneuver: modified Valsalva—15 seconds of straining (approximately 40 mmHg) against the closed glottis in a semi-sitting position, followed immediately by lying supine and lifting the legs 45° and holding for 15 seconds; Carotid sinus massage—first auscultating the carotid murmur, pressing the unilateral carotid sinus for 5–10 seconds.
  • For acute SVT termination (first drug) — Adenosine: 6 mg rapid IV bolus (in 1–2 seconds), immediately followed by flushing with 20 mL saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Rhythm control in a selected patient with preserved LVEF and no heart failure — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Cardiomyopathy or low ejection fraction

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. Classify the documented rhythm as SVT, AF/flutter, or VT and select treatment based on arrhythmia type.
  3. Consider other concomitant risk phenotypes together; If accompanied by hypertensive disease, keep the observation threshold low in the hospital; Consider advanced rhythm examination and genetic counseling in cases of suspected hereditary arrhythmia.
  4. Provide cardiology–obstetrics co-management; evaluate by a multidisciplinary committee at a tertiary center; Closely monitor for decreased effort capacity, recurrent tachyarrhythmia, and tendency to decompensation.
  5. Save LVEF and NYHA functional class. If LVEF < 30% or NYHA III–IV heart failure, consider mWHO IV; Discuss the maternal risk, continuation in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy in the early period.
  6. LVEF < 35% or if thrombus is present, therapeutic anticoagulation required; In other conditions, such as atrial arrhythmia, individualize anticoagulation according to thromboembolic risk.
  7. Monitor fetal growth with serial ultrasonography and fetal well-being with umbilical artery and middle cerebral artery Doppler.
  8. Prepare a written birth plan at approximately week 32; Determine birth order emergency algorithm and team tasks.
  9. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  10. Administer epidural analgesia, initiated early in labor and continued without interruption; Avoid excessive vasodilation in HCM; Use assisted vaginal birth in conjunction with cardiology based on individual risk assessment to shorten the second stage.
  11. Intensify postpartum monitoring; plan proactive monitoring for recurrence in the first weeks and early check-up before discharge; teach warning signs and review antiarrhythmic therapy for breastfeeding compatibility.
  12. Closely monitor patients with reduced myocardial reserve for pulmonary edema, hypoxia and cardiogenic shock, especially in the first 24–72 hours after birth.

Treatment

  • First step in SVT with narrow QRS—Vagal maneuver: modified Valsalva—15 seconds of straining (approximately 40 mmHg) against the closed glottis in a semi-sitting position, followed immediately by lying supine and lifting the legs 45° and holding for 15 seconds; Carotid sinus massage—first auscultating the carotid murmur, pressing the unilateral carotid sinus for 5–10 seconds.
  • For acute SVT termination (first drug) — Adenosine: 6 mg rapid IV bolus (in 1–2 seconds), immediately followed by flushing with 20 mL saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • In low LVEF/heart failure only in stable and euvolemic patients; In this case, the long-acting form of succinate or bisoprolol is preferred; if using non-selective beta blocker, switch to beta-1 selective — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • In ARVC, if there is no left ventricular systolic dysfunction, first-line antiarrhythmic in addition to beta-blocker — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • In ARVC or selected cardiomyopathy with preserved LVEF, rhythm control by expert judgment — Sotalol: start at 80 mg by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • If there is documented intracardiac thrombus or LVEF < 35% in cardiomyopathy (including PPCM), therapeutic dose LMWH (enoxaparin): 1 mg/kg SC based on early pregnancy weight every 12 hours (if renal function is appropriate).
  • If bromocriptine is used in PPCM (minimum prophylactic dose) — Enoxaparin (prophylactic): 40 mg SC once daily under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • When using bromocriptine in PPCM, if LVEF < 35%, intracardiac thrombus or AF with high thromboembolic risk, therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours according to early pregnancy weight.
  • At birth, by slow infusion rather than rapid IV bolus — Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • Avoid positive inotropes (digoxin, dobutamine) and hypovolemia, excessive diuresis, vasodilation and tachycardia in HCM.
  • In ARVC, do not discontinue beta-blocker therapy, including sotalol, during pregnancy or the postpartum period; reprogram the ICD appropriately.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
New arrhythmia on the basis of hypertensive disease of pregnancy

Approach

  1. Document the rhythm early with a 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. Classify the documented rhythm as SVT, AF/flutter, or VT and select treatment based on arrhythmia type.
  3. Consider other concomitant risk phenotypes together; If accompanied by hypertensive disease, keep the observation threshold low in the hospital; Consider advanced rhythm examination and genetic counseling in cases of suspected hereditary arrhythmia.
  4. Perform joint maternal–fetal assessment; Consider cardiac findings as maternal risk indicators that can directly determine the timing of birth.
  5. Refer to a tertiary center and evaluate by a multidisciplinary committee.
  6. Determine the birth order emergency algorithm and team task distribution in writing.
  7. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  8. Proactively monitor for arrhythmia recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review medications for breastfeeding compatibility.

Treatment

  • First step in SVT with narrow QRS—Vagal maneuver: modified Valsalva—15 seconds of straining (approximately 40 mmHg) against the closed glottis in a semi-sitting position, followed immediately by lying supine and lifting the legs 45° and holding for 15 seconds; Carotid sinus massage—first auscultating the carotid murmur, pressing the unilateral carotid sinus for 5–10 seconds.
  • For acute SVT termination (first drug) — Adenosine: 6 mg rapid IV bolus (in 1–2 seconds), immediately followed by flushing with 20 mL saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Treat acute severe hypertension without delay; Select the agent and dose from the 'Acute severe hypertension' algorithm.
  • In the absence of structural heart disease, in rhythm control — Flecainide: start at 50 mg orally every 12 hours (in ventricular arrhythmia, 50–100 mg every 12 hours); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • Rhythm control — Sotalol: start at 80 mg by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Do not use labetalol in bradycardia, heart block, decompensated heart function, or asthma.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Suspected hereditary arrhythmia: family history of sudden cardiac death at an early age (may be accompanied by syncope or VT)

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. Classify the documented rhythm as SVT, AF/flutter, or VT and select treatment based on arrhythmia type.
  3. Consider other concomitant risk phenotypes together; If accompanied by hypertensive disease, keep the observation threshold low in the hospital; Consider advanced rhythm examination and genetic counseling in cases of suspected hereditary arrhythmia.
  4. Refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee; Assess the need for electrophysiology consultation.
  5. Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death.
  6. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  7. Prepare an individualized birth-puerperium plan; Determine the intrapartum emergency algorithm and team tasks in writing.
  8. Conduct maternal rhythm management in conjunction with fetal monitoring.
  9. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  10. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • Except for Brugada syndrome, in emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • If beta blocker is required — Propranolol (oral): 60–320 mg daily in divided doses every 6–12 hours.
  • As a beta blocker if diagnosed with long QT or CPVT — Nadolol: 40–80 mg once daily; up to 240 mg if necessary.
  • If syncope, VT or cardiac arrest develops despite beta blocker in CPVT or is stable on beta blocker–flecainide combination before pregnancy, in addition to beta blocker (continue both drugs throughout pregnancy and puerperium) — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.

Note

  • Avoid drugs that prolong QT (including class IA, such as procainamide) in long QT syndrome.
  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Avoid sodium channel blockers (procainamide, flecainide) in Brugada syndrome; Anesthetics/antiarrhythmics that block the sodium channel, such as propofol and lidocaine, can trigger arrhythmia, select anesthetic drugs according to the list of drugs to avoid in Brugada; Treat fever immediately. Quinidine is a treatment used with expert judgment in Brugada and short QT syndrome; is not covered by this avoidance warning.
  • Do not administer adrenaline for ventricular arrhythmia or cardiac arrest due to CPVT; after defibrillation, manage with an IV beta blocker, flecainide, and anesthesia/sedation; do not delay defibrillation.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Propranolol (oral): After delivery, increased dosage in pregnancy is reconsidered.
  • Propranolol (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Nadolol: After delivery, increased dosage in pregnancy is reconsidered.
  • Nadolol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
Other high-risk feature: previous sustained VT/VF or recurrent/unexplained syncope–presyncope

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. Classify the documented rhythm as SVT, AF/flutter, or VT and select treatment based on arrhythmia type.
  3. Consider other concomitant risk phenotypes together; If accompanied by hypertensive disease, keep the observation threshold low in the hospital; Consider advanced rhythm examination and genetic counseling in cases of suspected hereditary arrhythmia.
  4. Refer to a tertiary center with a low threshold and perform multidisciplinary evaluation without assuming a diagnosis of hereditary arrhythmia; Assess the need for electrophysiology consultation.
  5. Perform advanced rhythm review; Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death.
  6. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  7. Determine in writing the birth order emergency algorithm and team task distribution; Write down your birth and puerperium follow-up plan.
  8. Conduct maternal rhythm management in conjunction with fetal monitoring.
  9. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  10. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • In emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In the absence of prolonged QT and torsade, emergency control of ventricular arrhythmia (in advanced center) — Procainamide: 15 mg/kg IV loading over at least 30 minutes and with an infusion rate not exceeding 50 mg/min (total loading not more than 17 mg/kg); followed by 1–4 mg/min IV infusion.
  • Beta blocker required — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Procainamide: Stop loading if arrhythmia is suppressed, hypotension develops, or QRS duration is prolonged by more than 50%; It is avoided in prolonged QT and heart failure.
  • Procainamide: Uterine irritability and preterm labor have been reported with IV use during pregnancy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Premature beats (extrasystole)
Floor
Isolated premature beats (no structural heart disease and no high-risk features)

Approach

  1. Classify as mWHO I (very low risk).
  2. Document rhythm with 12-lead ECG; Evaluate electrolytes, thyroid function, complete blood count, and ECHO for structural heart disease.
  3. Perform routine obstetric care; Specialized intervention is rarely required.
  4. Perform cardiology evaluation 1–2 times during pregnancy according to symptoms and local practice.
  5. Provide counseling stating that pregnancy is generally safe; Advise the patient to avoid triggers such as caffeine, alcohol, stimulants, and beta-agonist drugs.
  6. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.

Treatment

  • No medication is required for asymptomatic or well-tolerated premature beats.
  • For intolerable symptom, start with lowest effective dose — Metoprolol (oral): immediate-release tartrate 25 mg twice daily, gradually increased as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Associated with structural heart disease, syncope, or other high-risk feature

Approach

  1. Request specialist cardiology evaluation; determine whether premature beats are isolated or reflect underlying structural heart disease.
  2. Evaluate with 12-lead ECG, Holter/rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO.
  3. Determine the risk class according to the underlying disease with mWHO and CARPREG II.
  4. If there is syncope/presyncope, previous sustained VT/VF, ventricular dysfunction/cardiomyopathy, complex congenital heart disease, new arrhythmia in hypertensive disease of pregnancy, or family history of sudden cardiac death at an early age, refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee.
  5. Prepare a written antenatal emergency plan and a birth and postpartum follow-up plan.
  6. Choose antiarrhythmic medication according to arrhythmia type, structural heart disease, gestational age, and maternal–fetal risk.
  7. Conduct maternal rhythm management in conjunction with fetal monitoring.
  8. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  9. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Beta blocker required — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile. Avoid inappropriate prophylactic medication strategies.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Supraventricular tachycardia (SVT)
Clinical situation
Pre-pregnancy counseling (history of SVT)

Approach

  1. In cases of persistent SVT, obtain a pre-pregnancy specialist cardiology evaluation; Determine whether the arrhythmia is isolated or due to structural heart disease with ECG and ECHO.
  2. Evaluate exercise-induced arrhythmia and functional capacity with exercise testing in the selected patient.
  3. Determine mWHO class, CARPREG II score (prior arrhythmia 3 points), and medication schedule based on findings.
  4. Classify uncomplicated SVT as mWHO II; Schedule a cardiology check-up at least once per trimester of pregnancy.
  5. Consider the need for electrophysiology consultation.
  6. Control arrhythmia before pregnancy if it can be treated safely; make medication changes before pregnancy; Allow time to verify maternal stability in the new regimen.
  7. Plan pregnancy while clinically stable, at optimal functional capacity, and on a medication regimen compatible with pregnancy.
  8. Provide contraception counseling.

Treatment

  • If beta blockers are required during pregnancy — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Depending on arrhythmia type and maternal status — Digoxin: oral maintenance 0.125–0.5 mg daily in 2 divided doses; The dose is adjusted according to the serum digoxin level.
  • If LVEF is normal and there is no heart failure, depending on arrhythmia type and maternal status — Verapamil (oral): immediate-release form 40 mg 3–4 times a day is started, increasing according to heart rate control; maintenance 120–480 mg per day, maximum 480 mg per day.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use positive inotropes such as digoxin and dobutamine if HCM is present.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Avoid amiodarone; Reserve only for life-threatening arrhythmia.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Verapamil (oral): Not administered in the presence of pre-excitation (WPW) or in patients with reduced LVEF. Postpartum, any dose increased during pregnancy should be re-evaluated.
  • Verapamil (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
Acute attack during pregnancy
Hemodynamic status
Hemodynamic instability (hypotension, hypoperfusion, persistent chest pain, acute dyspnea or altered consciousness)

Approach

  1. Start treatment without waiting for detailed rhythm classification; Provide maternal stabilization first.
  2. After stabilization, evaluate triggers and structural heart disease with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO.
  3. If there is syncope/presyncope, previous sustained VT/VF, ventricular dysfunction/cardiomyopathy, complex congenital heart disease, new arrhythmia in hypertensive disease of pregnancy, or family history of sudden cardiac death at an early age, refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee.
  4. Conduct maternal rhythm management in conjunction with fetal monitoring.
  5. Put the birth and puerperium follow-up plan in writing.
  6. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  7. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.

Note

  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
Hemodynamically stable

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; evaluate electrolytes, thyroid function, complete blood count, and ECHO; Look for triggers and structural heart disease.
  2. If there is syncope/presyncope, previous sustained VT/VF, ventricular dysfunction/cardiomyopathy, complex congenital heart disease, new arrhythmia in hypertensive disease of pregnancy, or family history of sudden cardiac death at an early age, refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee.
  3. Conduct maternal rhythm management in conjunction with fetal monitoring.
  4. Put the birth and puerperium follow-up plan in writing.
  5. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  6. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • First step — Vagal maneuver: modified Valsalva—15 seconds of straining (approximately 40 mmHg) against the closed glottis in a semi-sitting position, followed immediately by lying supine and lifting the legs 45° and holding for 15 seconds; Carotid sinus massage—first auscultating the carotid murmur, pressing the unilateral carotid sinus for 5–10 seconds.
  • If vagal maneuver unresponsive (first drug) — Adenosine: 6 mg rapid IV bolus (over 1–2 seconds), immediately followed by flushing with 20 mL saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • Alternative in SVT termination or rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • In symptomatic SVT that is drug-resistant or drug-inappropriate — Synchronized electrical cardioversion: initial energy on a biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use positive inotropes such as digoxin and dobutamine if HCM is present.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
Non-attack monitoring and prophylaxis during pregnancy
Floor
Uncomplicated SVT (no structural heart disease and no high-risk features)

Approach

  1. Classify as mWHO II; Provide individualized counseling.
  2. Perform cardiology check at least once per trimester.
  3. Delivery may take place at the local hospital if specialist consultation and a pathway for transfer to a higher-level center are available.
  4. Control rhythm load early; Conduct maternal rhythm management together with fetal monitoring.
  5. Put the birth and puerperium follow-up plan in writing.
  6. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  7. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • First step in an SVT episode — Vagal maneuvers: modified Valsalva—straining against a closed glottis for 15 seconds (approximately 40 mmHg) in a semi-recumbent position, immediately followed by laying the patient supine and elevating the legs to 45° for 15 seconds; carotid sinus massage—first auscultate for a carotid bruit, then apply unilateral carotid sinus pressure for 5–10 seconds.
  • For acute termination of an SVT attack (first drug) — Adenosine: 6 mg rapid IV bolus (in 1–2 seconds), immediately followed by flushing with 20 mL physiological saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • For prophylaxis, rate control and symptom suppression — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • If LVEF is normal and there is no heart failure, alternative prophylaxis — Verapamil (oral): immediate-release form is started at 40 mg 3–4 times a day, increased according to heart rate control; maintenance 120–480 mg per day, maximum 480 mg per day.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.
  • As first choice if there is pre-excitation (WPW) on the resting ECG, in SVT resistant to beta-blockers or verapamil if there is no pre-excitation — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • Rhythm control — Sotalol: start at 80 mg by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile. Avoid inappropriate prophylactic medication strategies.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil (oral): Not administered in the presence of pre-excitation (WPW) or in patients with reduced LVEF. Postpartum, any dose increased during pregnancy should be re-evaluated.
  • Verapamil (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Structural/congenital heart disease, cardiomyopathy, syncope or other high-risk feature

Approach

  1. Plan close follow-up at a tertiary center and evaluate by a multidisciplinary committee; Perform regular ECG/Holter monitoring.
  2. Risk stratify with mWHO, CARPREG II (previous arrhythmia 3 points) and ZAHARA in congenital heart disease.
  3. Prepare a written antenatal emergency plan; establish the intrapartum emergency algorithm and team role assignments in writing.
  4. Plan cardiology-gynecology joint management and intensified puerperal monitoring in cardiomyopathy.
  5. Monitor fetal growth for risk of preterm birth and fetal growth restriction; If the mother or father has congenital heart disease, discuss the risk of recurrence in the fetus and recommend fetal echocardiography at 18–22 weeks
  6. Apply pharmacological treatment aggressively in congenital heart disease as arrhythmia may trigger decompensation.
  7. Determine the mode of delivery according to obstetric indications; do not routinely perform cesarean delivery.
  8. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • First step in an SVT episode — Vagal maneuvers: modified Valsalva—straining against a closed glottis for 15 seconds (approximately 40 mmHg) in a semi-recumbent position, immediately followed by laying the patient supine and elevating the legs to 45° for 15 seconds; carotid sinus massage—first auscultate for a carotid bruit, then apply unilateral carotid sinus pressure for 5–10 seconds.
  • For acute termination of an SVT attack (first drug) — Adenosine: 6 mg rapid IV bolus (in 1–2 seconds), immediately followed by flushing with 20 mL physiological saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • Prophylaxis, rate control and symptom suppression (in low LVEF/heart failure only in stable and euvolemic patients; in this case, long-acting succinate form or bisoprolol is preferred; if non-selective beta blockers are used, switch to beta-1 selective) — Metoprolol (oral): immediate-release tartrate is started at 25 mg twice a day, gradually increasing as tolerated to a maximum of 200 mg per day, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Cardioselective alternative in cardiomyopathy — Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • If LVEF is normal and there is no heart failure, alternative prophylaxis — Verapamil (oral): immediate-release form is started at 40 mg 3–4 times a day, increased according to heart rate control; maintenance 120–480 mg per day, maximum 480 mg per day.
  • Other than HCM, for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily in 2 divided doses; The dose is adjusted according to the serum digoxin level.
  • Rhythm control in a selected patient with preserved LVEF and no heart failure — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Avoid positive inotropes (digoxin, dobutamine) and hypovolemia, excessive diuresis, vasodilation and tachycardia in HCM.
  • In ARVC, do not discontinue beta-blocker therapy, including sotalol, during pregnancy or the postpartum period; reprogram the ICD appropriately.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Vagal maneuver: No pressure is applied to the eyeball.
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Verapamil (oral): Not administered in the presence of pre-excitation (WPW) or in patients with reduced LVEF. Postpartum, any dose increased during pregnancy should be re-evaluated.
  • Verapamil (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Atrial fibrillation / atrial flutter
Clinical situation
Pre-pregnancy counseling (AF/flutter history)

Approach

  1. Obtain specialist cardiology evaluation before pregnancy.
  2. Search for valve disease, cardiomyopathy, congenital heart disease, thyroid disease and other systemic causes of AF in young women.
  3. Evaluate with ECG and ECHO; Perform a stress test on the selected patient.
  4. Determine mWHO class, CARPREG II score (prior arrhythmia 3 points), and medication schedule based on findings.
  5. Consider the need for electrophysiology consultation.
  6. Due to thromboembolic risk, make an individual anticoagulation plan before pregnancy; If using DOAC, plan the switch to an anticoagulant compatible with pregnancy before pregnancy; Clearly discuss maternal protection, fetal safety and monitoring needs.
  7. Control arrhythmia before pregnancy if it can be treated safely; make medication changes before pregnancy; Allow time to verify maternal stability in the new regimen.
  8. Avoid estrogen-containing combined hormonal contraception; Choose long-acting methods such as intrauterine device or progestin implant.

Treatment

  • If beta blockers are required during pregnancy — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Depending on arrhythmia type and maternal status — Digoxin: oral maintenance 0.125–0.5 mg daily in 2 divided doses; The dose is adjusted according to the serum digoxin level.
  • If LVEF is normal and there is no heart failure, depending on arrhythmia type and maternal status — Verapamil (oral): immediate-release form 40 mg 3–4 times a day is started, increasing according to heart rate control; maintenance 120–480 mg per day, maximum 480 mg per day.
  • If anticoagulation is required during pregnancy (except for mechanical valve and moderate to severe mitral stenosis): therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours).

Note

  • DOAC is not recommended during pregnancy. VKA (warfarin) carries the risk of embryopathy and pregnancy loss; Generally avoid VKA during pregnancy except for mechanical valve and moderate to severe mitral stenosis. LMWH and UFH do not cross the placenta; They are safe anticoagulant options for the fetus.
  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use positive inotropes such as digoxin and dobutamine if HCM is present.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Avoid amiodarone; Reserve only for life-threatening arrhythmia.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Verapamil (oral): Not administered in the presence of pre-excitation (WPW) or in patients with reduced LVEF. Postpartum, any dose increased during pregnancy should be re-evaluated.
  • Verapamil (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
Hemodynamic instability during pregnancy (hypotension, hypoperfusion, persistent chest pain, acute dyspnea or altered consciousness)

Approach

  1. Start treatment without waiting for detailed rhythm classification; Provide maternal stabilization first.
  2. After stabilization, evaluate with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO; Look for valve disease, cardiomyopathy, congenital heart disease, thyroid disease, and other systemic causes.
  3. If there is syncope/presyncope, previous sustained VT/VF, ventricular dysfunction/cardiomyopathy, complex congenital heart disease, new arrhythmia in hypertensive disease of pregnancy, or family history of sudden cardiac death at an early age, refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee.
  4. Conduct maternal rhythm management in conjunction with fetal monitoring.
  5. Evaluate the need for anticoagulation with LMWH or transesophageal ECHO before cardioversion as in a non-pregnant woman; After stabilization, individualize anticoagulation according to maternal thromboembolic risk.
  6. AF/flutter is a risk factor for valve thrombosis in mechanical valve prosthesis; finalize the anticoagulation plan with the Pregnancy Heart Team as soon as pregnancy is confirmed; Make the choice based on the balance of valve thrombosis and fetal risk and the patient's informed preference.
  7. Put the birth and puerperium follow-up plan in writing; determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  8. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • Anticoagulation in AF other than mechanical valve and moderate to severe mitral stenosis: generally prefer therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours).
  • Maintain anticoagulation for at least 4 weeks after cardioversion.
  • In acute rate control after stabilization — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative to rate control after stabilization — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control after stabilization — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.

Note

  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use positive inotropes such as digoxin and dobutamine if HCM is present.
  • VKA (warfarin) is the most effective drug against valve thrombosis in mechanical valve prosthesis, but it increases the risk of fetal anomalies, especially at doses above 5 mg/day in the 1st trimester; LMWH has a lower fetal risk but a higher risk of maternal thromboembolism and requires close anti-Xa monitoring; Record the choice as the patient's informed choice.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
Hemodynamically stable during pregnancy
Underlying condition
No structural heart disease

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; evaluate electrolytes, thyroid function, complete blood count, and ECHO; Look for valve disease, cardiomyopathy, congenital heart disease, thyroid disease, and other systemic causes.
  2. If there is more than one concomitant disease, apply the anticoagulation plan for valve disease or prosthesis, ventricular dysfunction and drug restrictions for cardiomyopathy together in the same management plan.
  3. Control rhythm load early; Conduct maternal rhythm management together with fetal monitoring.
  4. Individualize anticoagulation requirement according to maternal thromboembolic risk; Previous thromboembolism increases the risk.
  5. If AF/flutter exceeds 48 hours or duration is unknown, provide at least 3 weeks of therapeutic anticoagulation before pharmacological or electrical cardioversion or initiate therapeutic anticoagulation and exclude left atrial thrombus by transesophageal ECHO; Maintain anticoagulation for at least 4 weeks after cardioversion. Do not delay shock in an unstable patient for this reason.
  6. Put the birth and puerperium follow-up plan in writing.
  7. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  8. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.
  9. Avoid estrogen-containing combined hormonal contraception; Choose long-acting methods such as intrauterine device or progestin implant.

Treatment

  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.
  • If symptoms or rapid ventricular response persist despite rate control or if hemodynamics is impaired — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pharmacological cardioversion — Flecainide: 2 mg/kg IV over at least 10 minutes (with a history of heart failure, the first dose is within 30 minutes); single bolus maximum 150 mg; It is given under continuous ECG monitoring, the injection is stopped when arrhythmia is checked; In severe renal impairment (creatinine clearance below 35 mL/min/1.73 m²), the dose is halved.
  • In pharmacological cardioversion — Ibutilide: 0.01 mg/kg under 60 kg, 1 mg IV over 10 minutes for those weighing 60 kg or more; If the arrhythmia continues 10 minutes after the end of the first infusion, the same dose is given again in 10 minutes (maximum 2 doses in total); The infusion is stopped when the arrhythmia ends, sustained or non-sustained VT develops, or QT/QTc is significantly prolonged.
  • In the absence of structural heart disease, in addition to beta-blockers for rhythm control — Flecainide: start at 50 mg orally every 12 hours (in ventricular arrhythmia, 50–100 mg every 12 hours); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • Rhythm control — Sotalol: start at 80 mg by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • If anticoagulation is required: generally choose therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours).
  • Stop heparin before planned delivery: wait at least 12 hours after prophylactic LMWH, wait at least 24 hours after an intermediate or therapeutic dose; If there is no significant bleeding or significant risk of bleeding, restart 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • Only a prophylactic dose of LMWH may be administered with the neuraxial catheter in place; remove the catheter at least 12 hours after the last prophylactic dose and at least 24 hours after the last therapeutic dose; Start prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In drug-resistant and poorly tolerated cavotricuspid isthmus-dependent (typical) atrial flutter, consider catheter ablation in an experienced center with a mapping system without fluoroscopy and preferably after the first trimester.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Do not use DOACs during pregnancy or breastfeeding; VKA (warfarin) is generally avoided during pregnancy except for mechanical valve and moderate to severe mitral stenosis.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Do not routinely plan ablation for AF during pregnancy; consider it exceptionally through a specialist multidisciplinary team for recurrent hemodynamically unstable AF that cannot be controlled with medication.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Ibutilide: Hypokalemia and hypomagnesemia are first corrected; Other QT prolonging drugs are discontinued before and after; ECG is monitored continuously for at least 4 hours after the infusion and until QTc returns to the initial value. If arrhythmia occurs, it is monitored longer.
  • Ibutilide: Experience in pregnancy is limited; It is avoided in the first trimester.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Structural or congenital heart disease (other than valve prosthesis, mitral stenosis and cardiomyopathy)

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. If there is more than one concomitant disease, apply the anticoagulation plan for valve disease or prosthesis, ventricular dysfunction and drug restrictions for cardiomyopathy together in the same management plan.
  3. Risk stratify with mWHO, CARPREG II (previous arrhythmia 3 points) and ZAHARA in congenital heart disease.
  4. Plan close follow-up at a tertiary center and evaluate by a multidisciplinary committee; Perform regular ECG/Holter monitoring.
  5. Prepare a written antenatal emergency plan; establish the intrapartum emergency algorithm and team role assignments in writing.
  6. Monitor fetal growth for risk of preterm birth and fetal growth restriction; If the mother or father has congenital heart disease, discuss the risk of recurrence in the fetus and recommend fetal echocardiography at 18–22 weeks
  7. Apply pharmacological treatment aggressively in congenital heart disease as arrhythmia may trigger decompensation.
  8. AF in structural heart disease increases the risk of systemic embolism; individualize anticoagulation according to thromboembolic risk; In congenital heart disease, determine the strategy according to the lesion, history of thrombosis, oxygen saturation and ventricular function.
  9. If AF/flutter exceeds 48 hours or duration is unknown, provide at least 3 weeks of therapeutic anticoagulation before pharmacological or electrical cardioversion or initiate therapeutic anticoagulation and exclude left atrial thrombus by transesophageal ECHO; Maintain anticoagulation for at least 4 weeks after cardioversion. Do not delay shock in an unstable patient for this reason.
  10. Determine the mode of delivery according to obstetric indications; do not routinely perform cesarean delivery.
  11. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.
  12. Avoid estrogen-containing combined hormonal contraception; Choose long-acting methods such as intrauterine device or progestin implant.

Treatment

  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.
  • If symptoms or rapid ventricular response persist despite rate control or if hemodynamics is impaired — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • Rhythm control in a selected patient with preserved LVEF and no heart failure — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • If anticoagulation is required: generally choose therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours).
  • Stop heparin before planned delivery: wait at least 12 hours after prophylactic LMWH, wait at least 24 hours after an intermediate or therapeutic dose; If there is no significant bleeding or significant risk of bleeding, restart 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • Only a prophylactic dose of LMWH may be administered with the neuraxial catheter in place; remove the catheter at least 12 hours after the last prophylactic dose and at least 24 hours after the last therapeutic dose; Start prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In drug-resistant and poorly tolerated cavotricuspid isthmus-dependent (typical) atrial flutter, consider catheter ablation in an experienced center with a mapping system without fluoroscopy and preferably after the first trimester.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Do not use DOACs during pregnancy or breastfeeding; VKA (warfarin) is generally avoided during pregnancy except for mechanical valve and moderate to severe mitral stenosis.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Do not routinely plan ablation for AF during pregnancy; consider it exceptionally through a specialist multidisciplinary team for recurrent hemodynamically unstable AF that cannot be controlled with medication.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Cardiomyopathy or low ejection fraction

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. If there is more than one concomitant disease, apply the anticoagulation plan for valve disease or prosthesis, ventricular dysfunction and drug restrictions for cardiomyopathy together in the same management plan.
  3. Provide cardiology–obstetrics co-management; evaluate by a multidisciplinary committee at a tertiary center; Closely monitor for decreased effort capacity, recurrent tachyarrhythmia, and tendency to decompensation.
  4. Save LVEF and NYHA functional class. If LVEF < 30% or NYHA III–IV heart failure, consider mWHO IV; Discuss the maternal risk, continuation in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy in the early period.
  5. LVEF < 35% or if thrombus is present, therapeutic anticoagulation required; In other conditions, such as atrial arrhythmia, individualize anticoagulation according to thromboembolic risk.
  6. If AF/flutter exceeds 48 hours or duration is unknown, provide at least 3 weeks of therapeutic anticoagulation before pharmacological or electrical cardioversion or initiate therapeutic anticoagulation and exclude left atrial thrombus by transesophageal ECHO; Maintain anticoagulation for at least 4 weeks after cardioversion. Do not delay shock in an unstable patient for this reason.
  7. Monitor fetal growth with serial ultrasonography and fetal well-being with umbilical artery and middle cerebral artery Doppler.
  8. Prepare a written birth plan at approximately week 32; Determine birth order emergency algorithm and team tasks.
  9. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  10. Administer epidural analgesia, initiated early in labor and continued without interruption; Avoid excessive vasodilation in HCM; Use assisted vaginal birth in conjunction with cardiology based on individual risk assessment to shorten the second stage.
  11. Intensify postpartum monitoring; plan proactive monitoring for recurrence in the first weeks and early check-up before discharge; teach warning signs and review antiarrhythmic therapy for breastfeeding compatibility.
  12. Closely monitor patients with reduced myocardial reserve for pulmonary edema, hypoxia and cardiogenic shock, especially in the first 24–72 hours after birth.
  13. Prioritize cardioversion for rhythm control in AF in HCM.

Treatment

  • In acute rate control (unless decompensated heart failure) — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • Alternative for acute rate control other than HCM — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • In low LVEF/heart failure only in stable and euvolemic patients; In this case, the long-acting form of succinate or bisoprolol is preferred; if using non-selective beta blocker, switch to beta-1 selective — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • Other than HCM, for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily in 2 divided doses; The dose is adjusted according to the serum digoxin level.
  • If LVEF is normal and there is no heart failure, alternative to rate control — Verapamil (oral): immediate-release form is started at 40 mg 3–4 times daily, increasing according to heart rate control; maintenance 120–480 mg per day, maximum 480 mg per day.
  • If symptoms or rapid ventricular response persist despite rate control or if hemodynamics is impaired — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • If anticoagulation is required: generally choose therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours).
  • If there is documented intracardiac thrombus or LVEF < 35% in cardiomyopathy (including PPCM), therapeutic dose LMWH (enoxaparin): 1 mg/kg SC based on early pregnancy weight every 12 hours (if renal function is appropriate).
  • If bromocriptine is used in PPCM (minimum prophylactic dose) — Enoxaparin (prophylactic): 40 mg SC once daily under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • When using bromocriptine in PPCM, if LVEF < 35%, intracardiac thrombus or AF with high thromboembolic risk, therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours according to early pregnancy weight.
  • Stop heparin before planned delivery: wait at least 12 hours after prophylactic LMWH, wait at least 24 hours after an intermediate or therapeutic dose; If there is no significant bleeding or significant risk of bleeding, restart 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • Only a prophylactic dose of LMWH may be administered with the neuraxial catheter in place; remove the catheter at least 12 hours after the last prophylactic dose and at least 24 hours after the last therapeutic dose; Start prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • At birth, by slow infusion rather than rapid IV bolus — Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • Avoid positive inotropes (digoxin, dobutamine) and hypovolemia, excessive diuresis, vasodilation and tachycardia in HCM.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • In ARVC, do not discontinue beta-blocker therapy, including sotalol, during pregnancy or the postpartum period; reprogram the ICD appropriately.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Do not use DOACs during pregnancy or breastfeeding; VKA (warfarin) is generally avoided during pregnancy except for mechanical valve and moderate to severe mitral stenosis.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • If HCM is accompanied by clinical AF/flutter, apply therapeutic anticoagulation regardless of thromboembolic risk score (therapeutic dose LMWH during pregnancy).
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Verapamil (oral): Not administered in the presence of pre-excitation (WPW) or in patients with reduced LVEF. Postpartum, any dose increased during pregnancy should be re-evaluated.
  • Verapamil (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
Mitral stenosis

Approach

  1. Classify moderate mitral stenosis as mWHO III and severe mitral stenosis as mWHO IV; In case of severe mitral stenosis, discuss the risk of the mother in the early period, the option of continuing in a specialist center and terminating the pregnancy if it is legally and ethically appropriate.
  2. Monitor at the specialist center with the Pregnancy Heart Team; In mWHO III, do cardiology check-ups generally every 1–2 months and create a birth plan early.
  3. Evaluate with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO; Take into account that stenosis gradients increase during pregnancy.
  4. If there is more than one concomitant disease, apply the anticoagulation plan for valve disease or prosthesis, ventricular dysfunction and drug restrictions for cardiomyopathy together in the same management plan.
  5. Conduct maternal rhythm management in conjunction with fetal monitoring.
  6. If AF/flutter exceeds 48 hours or duration is unknown, provide at least 3 weeks of therapeutic anticoagulation before pharmacological or electrical cardioversion or initiate therapeutic anticoagulation and exclude left atrial thrombus by transesophageal ECHO; Maintain anticoagulation for at least 4 weeks after cardioversion. Do not delay shock in an unstable patient for this reason.
  7. Plan the birth at the reference centre; Prepare the birth plan in advance and share it with the whole team, perform a prenatal obstetric anesthesia evaluation and apply continuous telemetry during birth.
  8. Plan vaginal delivery with appropriate analgesia and minimal Valsalva; Cesarean section is preferred in severe mitral stenosis and resistant heart failure.
  9. Determine the frequency of postpartum check-ups according to symptoms; It may take up to 6 months for hemodynamic changes to improve.
  10. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.
  11. Avoid estrogen-containing combined hormonal contraception; Choose long-acting methods such as intrauterine device or progestin implant.

Treatment

  • Basic treatment, acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • Basic treatment, maintenance — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.
  • If symptoms or rapid ventricular response persist despite rate control or if hemodynamics is impaired — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • If AF in mitral stenosis, left atrial thrombus, or previous embolism, administer full therapeutic dose anticoagulation: therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours) or VKA; Make the selection with the Pregnancy Heart Team based on gestational age, warfarin dose and the patient's informed preference.
  • In heart failure that persists despite rate control — Furosemide (oral): 20–40 mg once a day is started; If the response is insufficient, the single dose is adjusted by doubling instead of giving the same dose more frequently; The new dose is given no earlier than 6–8 hours after the previous dose; usual dose 40–240 mg per day; Maximum total daily dose of 600 mg (up to 1000 mg in severe renal impairment).
  • Valvuloplasty or valve replacement: only apply during pregnancy for symptoms resistant to medical treatment.
  • Stop heparin before planned delivery: wait at least 12 hours after prophylactic LMWH, wait at least 24 hours after an intermediate or therapeutic dose; If there is no significant bleeding or significant risk of bleeding, restart 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • Only a prophylactic dose of LMWH may be administered with the neuraxial catheter in place; remove the catheter at least 12 hours after the last prophylactic dose and at least 24 hours after the last therapeutic dose; Start prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.

Note

  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Do not use DOACs during pregnancy or breastfeeding; VKA (warfarin) is generally avoided during pregnancy except for mechanical valve and moderate to severe mitral stenosis.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Furosemide (oral): No breastfeeding difficulties have been reported with the low dose (20 mg/day) in breastfeeding.
  • Furosemide (oral): Use with caution during pregnancy as excessive diuresis may reduce uterine blood flow and placental perfusion.
Bioprosthetic valve

Approach

  1. Perform cardiology evaluation at least once per trimester, more frequently if the clinical situation warrants; Plan multidisciplinary follow-up with experts experienced in heart disease and pregnancy.
  2. Evaluate with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO.
  3. If there is more than one concomitant disease, apply the anticoagulation plan for valve disease or prosthesis, ventricular dysfunction and drug restrictions for cardiomyopathy together in the same management plan.
  4. AF in bioprosthetic valve is an additional risk factor; Individualize anticoagulant selection based on maternal thromboembolic risk.
  5. If AF/flutter exceeds 48 hours or duration is unknown, provide at least 3 weeks of therapeutic anticoagulation before pharmacological or electrical cardioversion or initiate therapeutic anticoagulation and exclude left atrial thrombus by transesophageal ECHO; Maintain anticoagulation for at least 4 weeks after cardioversion. Do not delay shock in an unstable patient for this reason.
  6. Conduct maternal rhythm management in conjunction with fetal monitoring.
  7. Plan the birth at the reference center; The peripartum antithrombotic plan should be determined by gynecology, anesthesia and hematology together, documented in writing and explained to the patient; Be aware of the risk of spinal epidural hematoma with neuraxial technique.
  8. Plan vaginal delivery with appropriate analgesia and minimal Valsalva; Isolated arrhythmia is not an indication for cesarean section.
  9. Determine the frequency of check-ups during puerperium according to symptoms; plan proactive monitoring and early check-up for recurrence; Teach warning signs at discharge and review antiarrhythmic therapy for breastfeeding compatibility.
  10. Avoid estrogen-containing combined hormonal contraception; Choose long-acting methods such as intrauterine device or progestin implant.

Treatment

  • Apply long-term anticoagulation: usually prefer therapeutic dose LMWH (enoxaparin based on weight, usually 1 mg/kg SC every 12 hours).
  • Low-dose aspirin (75–100 mg/day): consider in antithrombotic plan for bioprosthetic valve; Let the expert team determine its use with anticoagulation, considering the risk of bleeding.
  • Stop heparin before planned delivery: wait at least 12 hours after prophylactic LMWH, wait at least 24 hours after an intermediate or therapeutic dose; If there is no significant bleeding or significant risk of bleeding, restart 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • Only a prophylactic dose of LMWH may be administered with the neuraxial catheter in place; remove the catheter at least 12 hours after the last prophylactic dose and at least 24 hours after the last therapeutic dose; Start prophylactic LMWH at least 12 hours after the intervention, therapeutic LMWH at least 24 hours after the procedure, and in any case at least 4 hours after catheter withdrawal.
  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.
  • If symptoms or rapid ventricular response persist despite rate control or if hemodynamics is impaired — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • Rhythm control in a selected patient with preserved LVEF and no heart failure — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.

Note

  • Do not use DOACs during pregnancy and breastfeeding.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Mechanical valve prosthesis

Approach

  1. Manage at a specialist center with a Pregnancy Heart Team; Have your cardiology check-up usually every 1-2 months and create your birth plan early.
  2. Evaluate with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO.
  3. If there is more than one concomitant disease, apply the anticoagulation plan for valve disease or prosthesis, ventricular dysfunction and drug restrictions for cardiomyopathy together in the same management plan.
  4. Conduct maternal rhythm management in conjunction with fetal monitoring.
  5. If AF/flutter exceeds 48 hours or duration is unknown, provide at least 3 weeks of therapeutic anticoagulation before pharmacological or electrical cardioversion or initiate therapeutic anticoagulation and exclude left atrial thrombus by transesophageal ECHO; Maintain anticoagulation for at least 4 weeks after cardioversion. Do not delay shock in an unstable patient for this reason.
  6. Plan the birth at a reference center with neonatal intensive care facilities; prepare the birth plan in advance and distribute it to the entire team; perform a prenatal obstetric anesthesia evaluation; Apply continuous telemetry during labor.
  7. Develop the peripartum antithrombotic plan with the obstetrician, anesthesiologist, and hematologist, clearly document it, and explain it in detail to the patient.
  8. Plan vaginal delivery with neuraxial analgesia if timed according to the anticoagulation schedule and there is no obstetric indication; Minimize Valsalva.
  9. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • AF/flutter is a risk factor for valve thrombosis with a mechanical valve. Apply anticoagulation according to the pregnancy stage by selecting 'Mechanical prosthetic valve' in the algorithm. In that selection, use the 1st-trimester option 'Warfarin ≤ 5 mg/day; risk factor for valve thrombosis present' or 'Warfarin > 5 mg/day'.
  • Maintain therapeutic anticoagulation without interruption.
  • If symptoms or rapid ventricular response persist despite rate control or if hemodynamics is impaired — Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In acute rate control — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • For rate control and maintenance of symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative for rate control — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • Alternative for rate control — Digoxin: 0.5 mg IV by slow injection over at least 5 minutes; additional dose of 0.25 mg every 6 hours if necessary; Total 8–12 µg/kg (calculated by lean body weight) and not exceeding 0.75–1.5 mg in 24 hours; If creatinine clearance is 15 mL/min or less, the dose is halved.
  • Alternative for rate control maintenance — Digoxin: oral maintenance 0.125–0.5 mg daily divided into 2 doses; The dose is adjusted according to the serum digoxin level.
  • In drug-resistant and poorly tolerated cavotricuspid isthmus-dependent (typical) atrial flutter, consider catheter ablation in an experienced center with a mapping system without fluoroscopy and preferably after the first trimester.

Note

  • Prophylactic dose of LMWH is insufficient for mechanical valve.
  • Do not use verapamil in LVEF < 40% or heart failure. Do not use diltiazem during pregnancy and breastfeeding.
  • Do not use AV nodal blockers (beta blockers, verapamil, digoxin or adenosine) in pre-excited AF (WPW); if the patient is hemodynamically unstable, perform electrical cardioversion immediately.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Do not routinely plan ablation for AF during pregnancy; consider it exceptionally through a specialist multidisciplinary team for recurrent hemodynamically unstable AF that cannot be controlled with medication.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Digoxin: Not given in the presence of pre-excitation (WPW); Dosage adjustment may be required after birth.
  • Digoxin: Maternal serum digoxin level is closely monitored during pregnancy; In later stages of pregnancy, higher doses may be required for therapeutic levels.
Ventricular tachycardia / ventricular arrhythmias
Clinical situation
Pre-pregnancy counseling (history of VT/VF or ventricular arrhythmia)

Approach

  1. Obtain specialist cardiology evaluation before pregnancy; A history of ICD therapy also requires expert evaluation; ICD indications do not change with pregnancy.
  2. Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death; Evaluate with ECG and ECHO; If ECHO is inadequate, add non-contrast cardiac MRI.
  3. Evaluate exercise-induced arrhythmia and functional capacity with exercise testing in the selected patient.
  4. Determine mWHO class, CARPREG II score (prior arrhythmia 3 points), and medication schedule based on findings.
  5. Consider the need for electrophysiology consultation.
  6. Control arrhythmia before pregnancy if it can be treated safely; make medication changes before pregnancy; Allow time to verify maternal stability in the new regimen.
  7. Discuss and record risks, uncertainty, and options through shared decision making; Provide contraception counseling.

Treatment

  • If beta blockers are required during pregnancy — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Avoid amiodarone; Reserve only for life-threatening arrhythmia.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
Acute attack during pregnancy
Hemodynamic status
Hemodynamic instability (hypotension, hypoperfusion, persistent chest pain, acute dyspnea or altered consciousness)

Approach

  1. Start treatment without waiting for detailed rhythm classification; Provide maternal stabilization first.
  2. After stabilization, evaluate triggers and structural heart disease with 12-lead ECG, rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO; Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death.
  3. Rule out underlying peripartum cardiomyopathy in new-onset VT in the last 6 weeks of pregnancy or the first month postpartum.
  4. Ventricular arrhythmia carries a high acute risk; Refer to a tertiary center with a low threshold and evaluate in a multidisciplinary committee.
  5. Conduct maternal rhythm management in conjunction with fetal monitoring.
  6. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  7. Determine in writing the birth order emergency algorithm and team task distribution; Maintain obstetric flexibility.
  8. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  9. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Synchronized electrical cardioversion: initial energy on the biphasic device is 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • In emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In the absence of prolonged QT and torsade, emergency control of ventricular arrhythmia (in advanced center) — Procainamide: 15 mg/kg IV loading over at least 30 minutes and with an infusion rate not exceeding 50 mg/min (total loading not more than 17 mg/kg); followed by 1–4 mg/min IV infusion.
  • In life-threatening refractory VT/VF in the absence of prolonged QT and torsades — Amiodarone: 150 mg IV over 10 minutes (may be repeated if arrhythmia recurs); followed by 1 mg/min IV infusion for 6 hours, then 0.5 mg/min for 18 hours; 300 mg IV/IO bolus in cardiac arrest, second dose 150 mg if necessary.

Note

  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Procainamide: Stop loading if arrhythmia is suppressed, hypotension develops, or QRS duration is prolonged by more than 50%; It is avoided in prolonged QT and heart failure.
  • Procainamide: Uterine irritability and preterm labor have been reported with IV use during pregnancy.
  • Amiodarone: Avoided during pregnancy due to risk of fetal adverse effects; It is used only in life-threatening arrhythmia that cannot be controlled with other treatments; If administered, the fetus is monitored for bradycardia, growth restriction, and thyroid function.
Hemodynamically stable

Approach

  1. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  2. Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death.
  3. Rule out underlying peripartum cardiomyopathy in new-onset VT in the last 6 weeks of pregnancy or the first month postpartum.
  4. Ventricular arrhythmia carries a high acute risk; Refer to a tertiary center with a low threshold and evaluate in a multidisciplinary committee.
  5. Conduct maternal rhythm management in conjunction with fetal monitoring.
  6. Determine in writing the birth order emergency algorithm and team task distribution; Maintain obstetric flexibility.
  7. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  8. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Also immediately in stable sustained VT — Synchronized electrical cardioversion: initial energy in the biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after a shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • If cardioversion cannot be performed immediately (drug of choice; in advanced center; in the absence of prolonged QT and torsade) — Procainamide: 15 mg/kg IV loading over at least 30 minutes and the infusion rate not to exceed 50 mg/min (total loading not more than 17 mg/kg); followed by 1–4 mg/min IV infusion.
  • If cardioversion cannot be performed immediately (in advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In idiopathic outflow tract VT — Metoprolol: 2.5–5 mg IV over 2 minutes; If necessary, repeat every 5 minutes, maximum 15 mg in total.
  • Alternative in idiopathic outflow tract VT — Adenosine: 6 mg rapid IV bolus (over 1–2 seconds), immediately followed by flushing with 20 mL saline; If tachycardia does not stop within 1–2 minutes, 12 mg rapid IV bolus; 12 mg once more if necessary.
  • In expertly diagnosed idiopathic fascicular VT (if LVEF is preserved) — Verapamil: 2.5–10 mg IV, slow bolus over 5 minutes; It can be repeated after 30 minutes if necessary.
  • For symptom suppression — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Rhythm control in a selected patient with preserved LVEF and no heart failure — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • In the absence of structural heart disease, in rhythm control — Flecainide: start at 50 mg orally every 12 hours (in ventricular arrhythmia, 50–100 mg every 12 hours); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.

Note

  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Procainamide: Stop loading if arrhythmia is suppressed, hypotension develops, or QRS duration is prolonged by more than 50%; It is avoided in prolonged QT and heart failure.
  • Procainamide: Uterine irritability and preterm labor have been reported with IV use during pregnancy.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Metoprolol: Not given IV in decompensated heart failure (electrical cardioversion is preferred).
  • Adenosine: Given under continuous ECG recording; The initial dose is reduced to 3 mg in those receiving dipyridamole or carbamazepine, in those with heart transplantation and when given through the central vein; It is not given in bronchospasm or asthma, second-third degree AV block and sick sinus syndrome or symptomatic bradycardia (if there is no functional pacemaker); If advanced block develops with one dose, no additional dose is given.
  • Verapamil: Used only in patients with preserved LVEF; may cause maternal hypotension; If possible, adenosine or beta blocker is preferred in SVT, and IV beta-1 selective blocker is priority in AF/flutter rate control; It is not given concurrently with IV beta blockers; It is not given in pre-excitation (WPW) AF/flutter, ventricular tachycardia and wide QRS tachycardia of unknown supraventricular origin.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
Non-attack monitoring during pregnancy
Floor
Structural or congenital heart disease (non-cardiomyopathy)

Approach

  1. Plan close follow-up at a tertiary center and evaluate by a multidisciplinary committee; Perform regular ECG/Holter monitoring.
  2. Risk stratify with mWHO, CARPREG II (previous arrhythmia 3 points) and ZAHARA in congenital heart disease.
  3. Prepare a written antenatal emergency plan; establish the intrapartum emergency algorithm and team role assignments in writing.
  4. Consider the need for electrophysiology consultation.
  5. Monitor fetal growth for risk of preterm birth and fetal growth restriction; If the mother or father has congenital heart disease, discuss the risk of recurrence in the fetus and recommend fetal echocardiography at 18–22 weeks
  6. Apply pharmacological treatment aggressively in congenital heart disease as arrhythmia may trigger decompensation.
  7. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  8. Determine the mode of delivery according to obstetric indications; do not routinely perform cesarean delivery.
  9. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • Beta blocker required — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Rhythm control in a selected patient with preserved LVEF and no heart failure — Sotalol: 80 mg is started by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.
  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
Cardiomyopathy or low ejection fraction

Approach

  1. Provide cardiology–obstetrics co-management; evaluate by a multidisciplinary committee at a tertiary center; closely monitor for decreased effort capacity, recurrent tachyarrhythmia, and tendency to decompensation; Perform regular ECG/Holter monitoring.
  2. Save LVEF and NYHA functional class. If LVEF < 30% or NYHA III–IV heart failure, consider mWHO IV; Discuss the maternal risk, continuation in a specialist center and, if legally and ethically appropriate, the option of terminating the pregnancy in the early period.
  3. LVEF < 35% or if thrombus is present, therapeutic anticoagulation required; In other conditions, such as atrial arrhythmia, individualize anticoagulation according to thromboembolic risk.
  4. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  5. Monitor fetal growth with serial ultrasonography and fetal well-being with umbilical artery and middle cerebral artery Doppler.
  6. Prepare a written birth plan at approximately week 32; Determine birth order emergency algorithm and team tasks.
  7. Vaginal delivery is recommended for most cardiomyopathies; Caesarean section is preferred if there is obstetric indication, severe heart failure (LVEF < 30% and/or NYHA III–IV), uncontrolled arrhythmia, outflow tract obstruction ≥ 50 mmHg in HCM, or labor under VKA (warfarin).
  8. Administer epidural analgesia, initiated early in labor and continued without interruption; Avoid excessive vasodilation in HCM; Use assisted vaginal birth in conjunction with cardiology based on individual risk assessment to shorten the second stage.
  9. Intensify postpartum monitoring; plan proactive monitoring for recurrence in the first weeks and early check-up before discharge; teach warning signs and review antiarrhythmic therapy for breastfeeding compatibility.
  10. Closely monitor patients with reduced myocardial reserve for pulmonary edema, hypoxia and cardiogenic shock, especially in the first 24–72 hours after birth.

Treatment

  • In low LVEF/heart failure only in stable and euvolemic patients; In this case, the long-acting form of succinate or bisoprolol is preferred; if using non-selective beta blocker, switch to beta-1 selective — Metoprolol (oral): start with immediate-release tartrate 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Bisoprolol: Start at 1.25 mg once daily; The target is 10 mg once a day, doubling the dose every 1–2 weeks.
  • In ARVC, if there is no left ventricular systolic dysfunction, first-line antiarrhythmic in addition to beta-blocker — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • If there is documented intracardiac thrombus or LVEF < 35% in cardiomyopathy (including PPCM), therapeutic dose LMWH (enoxaparin): 1 mg/kg SC based on early pregnancy weight every 12 hours (if renal function is appropriate).
  • If bromocriptine is used in PPCM (minimum prophylactic dose) — Enoxaparin (prophylactic): 40 mg SC once daily under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • When using bromocriptine in PPCM, if LVEF < 35%, intracardiac thrombus or AF with high thromboembolic risk, therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours according to early pregnancy weight.
  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • At birth, by slow infusion rather than rapid IV bolus — Oxytocin: 2 IU immediately after birth, slow IV infusion over 10 minutes; followed by 12 mU/min for 4 hours (10 IU oxytocin in 500 mL physiological saline, 36 mL/hour).

Note

  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • In ARVC, do not discontinue beta-blocker therapy, including sotalol, during pregnancy or the postpartum period; reprogram the ICD appropriately.
  • Avoid positive inotropes (digoxin, dobutamine) and hypovolemia, excessive diuresis, vasodilation and tachycardia in HCM.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Assess renal function before enoxaparin; if creatinine clearance is <30 mL/min, do not apply the standard regimens unchanged; reduce the dose according to renal function or consider UFH with the specialist team.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Bisoprolol: Started only in stable and euvolemic patients.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Oxytocin: Slow infusion is used instead of rapid bolus.
No structural heart disease (unexplained VT)

Approach

  1. Perform further rhythm evaluation and consider genetic counseling.
  2. Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death.
  3. Refer to a tertiary center with a low threshold and evaluate by a multidisciplinary committee; Assess the need for electrophysiology consultation.
  4. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  5. Prepare an individualized birth-puerperium plan; Determine the intrapartum emergency algorithm and team tasks in writing.
  6. Conduct maternal rhythm management in conjunction with fetal monitoring.
  7. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  8. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • In emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In the absence of prolonged QT and torsade, emergency control of ventricular arrhythmia (in advanced center) — Procainamide: 15 mg/kg IV loading over at least 30 minutes and with an infusion rate not exceeding 50 mg/min (total loading not more than 17 mg/kg); followed by 1–4 mg/min IV infusion.
  • Beta blocker required — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Alternative to beta-blocker in the prevention of idiopathic outflow tract VT (if LVEF is preserved) — Verapamil (oral): immediate-release form started at 40 mg 3–4 times daily, increased according to heart rate control; maintenance 120–480 mg per day, maximum 480 mg per day.
  • If beta-blocker or verapamil are ineffective in idiopathic outflow tract VT — Flecainide: start at 50 mg orally every 12 hours (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.
  • If beta blocker or verapamil are ineffective in idiopathic outflow tract VT, if initial QTc ≤ 450 ms — Sotalol: start at 80 mg by calculating creatinine clearance; Dosing range depends on creatinine clearance: ≥60 mL/min every 12 hours, 30–59 mL/min every 24 hours, 10–29 mL/min every 36–48 hours, not used at <10 mL/min; If QTc remains below 500 ms, increase every 3 days at the earliest to 120–160 mg per dose; maintenance 160–320 mg per day; If QTc increases to 500 ms or above, the dose is reduced, the dose interval is extended, or the drug is discontinued.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.

Note

  • Use flecainide and sotalol only under the supervision of a specialist team, in selected patients and with close ECG monitoring. Do not use flecainide in structural heart disease, previous myocardial infarction or LVEF ≤ 40% (addition to beta-blocker, based on expert judgment in ARVC). Sotalol may be used with expert judgment in selected structural heart disease and ARVC; It is not a routine option in severe left ventricular dysfunction (LVEF < 40%) and is not used in decompensated heart failure.
  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Procainamide: Stop loading if arrhythmia is suppressed, hypotension develops, or QRS duration is prolonged by more than 50%; It is avoided in prolonged QT and heart failure.
  • Procainamide: Uterine irritability and preterm labor have been reported with IV use during pregnancy.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Verapamil (oral): Not administered in the presence of pre-excitation (WPW) or in patients with reduced LVEF. Postpartum, any dose increased during pregnancy should be re-evaluated.
  • Verapamil (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Sotalol: Do not start if baseline QTc is greater than 450 ms; starting, re-starting and increasing the dose are done in a center with continuous ECG monitoring and resuscitation facilities, while starting, the patient is monitored in the hospital for at least 3 days; Proarrhythmia may develop with each dose increase; potassium and magnesium are kept within normal range before starting and throughout treatment (do not start if serum potassium <4 mEq/L); It is not used in asthma or bronchospastic disease, sinus bradycardia <50/min while awake, second-third degree AV block (if there is no functional pacemaker), sick sinus syndrome, congenital or acquired long QT syndrome, cardiogenic shock and decompensated heart failure; Concomitant use with amiodarone and other QT prolonging antiarrhythmics is avoided. After delivery, the increased dose during pregnancy is re-evaluated.
  • Sotalol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels. Fetal growth is monitored due to the beta blocker effect.
Hereditary arrhythmia syndromes
Period
Pre-pregnancy counseling

Approach

  1. Obtain specialist cardiology evaluation before pregnancy; ICD indications do not change with pregnancy.
  2. Assess for hereditary channelopathy, ventricular function, ischemic disease, cardiomyopathy, and family history of sudden cardiac death; Evaluate with ECG and ECHO.
  3. Evaluate exercise-induced arrhythmia and functional capacity with exercise testing in the selected patient.
  4. Determine mWHO class, CARPREG II score (prior arrhythmia 3 points), and medication schedule based on findings.
  5. Consider the need for electrophysiology consultation.
  6. Control arrhythmia before pregnancy if it can be treated safely; make medication changes before pregnancy; Allow time to verify maternal stability in the new regimen.
  7. Consider genetic counseling; Address and record fetal and genetic risks in pre-pregnancy counseling.
  8. Discuss and record risks, uncertainty, and options through shared decision making; Provide contraception counseling.

Treatment

  • In long QT syndrome or CPVT — Nadolol: 40–80 mg once daily; up to 240 mg if necessary.
  • In long QT syndrome or CPVT — Propranolol (oral): 60–320 mg daily in divided doses every 6–12 hours.
  • If syncope, VT or cardiac arrest develops despite beta blocker in CPVT or is stable on beta blocker–flecainide combination before pregnancy, in addition to beta blocker (continue both drugs throughout pregnancy and puerperium) — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.

Note

  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Avoid amiodarone; Reserve only for life-threatening arrhythmia.
  • Flecainide is not used in Brugada syndrome.
  • Nadolol: After delivery, increased dosage in pregnancy is reconsidered.
  • Nadolol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Propranolol (oral): After delivery, increased dosage in pregnancy is reconsidered.
  • Propranolol (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
Pregnancy and birth

Approach

  1. Consider family history of sudden cardiac death at an early age a high-risk phenotype; Refer to a tertiary center with a low threshold and evaluate in a multidisciplinary committee.
  2. Document rhythm with 12-lead ECG; perform rhythm monitoring if necessary; Assess electrolytes, thyroid function, complete blood count, and ECHO.
  3. Perform further rhythm evaluation and consider genetic counseling; Assess the need for electrophysiology consultation.
  4. If hyperemesis is present, monitor for hypokalemia and hypomagnesemia and correct immediately.
  5. ICD indications do not change during pregnancy; If there is an indication, perform the procedure with a plan adapted to mother-fetus safety.
  6. Prepare an individualized birth-puerperium plan; Determine the intrapartum emergency algorithm and team tasks in writing.
  7. Plan labor with rhythm monitoring and electrolyte control; Perform continuous telemetry during labor, use neuraxial analgesia to reduce adrenergic discharge, and continue ECG monitoring until the effects of anesthetic drugs wear off; In CPVT, keep the heart rate below the early ventricular beat threshold (usually 100–110/min) during labor.
  8. Conduct maternal rhythm management in conjunction with fetal monitoring.
  9. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  10. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • Except for Brugada syndrome, in emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In long QT syndrome and CPVT, with pre-pregnancy dosage throughout pregnancy, birth and puerperium (preferred) — Nadolol: 40–80 mg once daily; up to 240 mg if necessary.
  • In long QT syndrome and CPVT, with preconception dosing throughout pregnancy, labor and puerperium (preference) — Propranolol (oral): 60–320 mg daily in divided doses every 6–12 hours.
  • If syncope, VT or cardiac arrest develops despite beta blocker in CPVT or is stable on beta blocker–flecainide combination before pregnancy, in addition to beta blocker (continue both drugs throughout pregnancy and puerperium) — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.

Note

  • Do not reduce the dose in long QT syndrome type 2, especially during the puerperium.
  • Note that the excretion of nadolol into milk is higher in breastfeeding women.
  • In long QT syndrome, avoid drugs that prolong QT (including class IA, such as procainamide) and monitor ECG; All antiemetics can prolong QT; if necessary, give them under ECG monitoring.
  • Do not use atenolol routinely; Choose a beta blocker with a safer profile.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Avoid sodium channel blockers (procainamide, flecainide) in Brugada syndrome; Anesthetics/antiarrhythmics that block the sodium channel, such as propofol and lidocaine, can trigger arrhythmia, select anesthetic drugs according to the list of drugs to avoid in Brugada; Treat fever immediately. Quinidine is a treatment used with expert judgment in Brugada and short QT syndrome; is not covered by this avoidance warning.
  • Do not administer adrenaline for ventricular arrhythmia or cardiac arrest due to CPVT; after defibrillation, manage with an IV beta blocker, flecainide, and anesthesia/sedation; do not delay defibrillation.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Nadolol: After delivery, increased dosage in pregnancy is reconsidered.
  • Nadolol: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Propranolol (oral): After delivery, increased dosage in pregnancy is reconsidered.
  • Propranolol (oral): Lowest effective dose used in pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
  • Flecainide: The lowest effective dose is used during pregnancy; In later stages of pregnancy, higher doses may be required to achieve therapeutic serum levels.
Postpartum period

Approach

  1. In long QT, especially LQT2, consider the first 9–12 months postpartum as a period of increased arrhythmic risk; Maintain beta blocker at pre-pregnancy dose, avoid dose reductions and drug changes; Perform proactive monitoring during the first postpartum weeks, plan early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for compatibility with breastfeeding.
  2. In long QT syndrome, the newborn should have a 12-lead ECG after birth and at the 2nd week; schedule genetic screening for the familial variant as soon as possible; If the newborn is affected, beta blockers are started immediately.
  3. Implement an individualized postpartum plan; Create a structured transition plan.
  4. Provide contraception counseling.
  5. Evaluate ICD indications with criteria other than pregnancy.

Treatment

  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • In polymorphic VT due to prolonged QT / torsades de pointes with a pulse (discontinue instigating drugs, restore potassium) — Magnesium sulfate: 1–2 g IV over 15 minutes; If there is no response or torsades de pointes recurs, repeat up to a total of 4 g in 1 hour; followed by 0.5–1 g/h IV infusion if necessary.
  • In pulseless torsades de pointes, with defibrillation: magnesium sulfate 2 g IV/IO over at least 1–2 minutes; if ineffective, 2 g bolus up to 2 more times (maximum 6 g total).
  • Except for Brugada syndrome, in emergency control of ventricular arrhythmia (advanced center) — Lidocaine: 1–1.5 mg/kg IV bolus, at a rate of 25–50 mg/min; additional bolus of 0.5–0.75 mg/kg every 5–10 minutes if necessary; Cumulative dose in one hour is up to 3 mg/kg and 300 mg; maintenance 1–4 mg/min IV infusion.
  • In long QT syndrome and CPVT, with pre-pregnancy dosage throughout pregnancy, birth and puerperium (preferred) — Nadolol: 40–80 mg once daily; up to 240 mg if necessary.
  • In long QT syndrome and CPVT, with preconception dosing throughout pregnancy, labor and puerperium (preference) — Propranolol (oral): 60–320 mg daily in divided doses every 6–12 hours.
  • If syncope, VT or cardiac arrest develops despite beta blocker in CPVT or is stable on beta blocker–flecainide combination before pregnancy, in addition to beta blocker (continue both drugs throughout pregnancy and puerperium) — Flecainide: oral 50 mg every 12 hours is started (50–100 mg every 12 hours in ventricular arrhythmia); If necessary, increase each dose by 50 mg every 4 days at the earliest; Maximum daily dose is 300 mg in AF/flutter and SVT, 400 mg in ventricular arrhythmia; Every 12 hours, in divided doses every 8 hours if necessary.

Note

  • Do not reduce the dose in long QT syndrome type 2, especially during the puerperium.
  • Note that the excretion of nadolol into milk is higher in breastfeeding women.
  • In long QT syndrome, avoid drugs that prolong QT (including class IA such as procainamide) and monitor ECG.
  • If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Avoid sodium channel blockers (procainamide, flecainide) in Brugada syndrome; Anesthetics/antiarrhythmics that block the sodium channel, such as propofol and lidocaine, can trigger arrhythmia, select anesthetic drugs according to the list of drugs to avoid in Brugada; Treat fever immediately. Quinidine is a treatment used with expert judgment in Brugada and short QT syndrome; is not covered by this avoidance warning.
  • Do not administer adrenaline for ventricular arrhythmia or cardiac arrest due to CPVT; after defibrillation, manage with an IV beta blocker, flecainide, and anesthesia/sedation; do not delay defibrillation.
  • Magnesium sulfate: If the patellar reflex disappears or the serum level exceeds 6 mEq/L, the infusion is discontinued.
  • Lidocaine: All lidocaine doses, including lidocaine given for regional anesthesia, are included in the total dose.
  • Nadolol: After delivery, increased dosage in pregnancy is reconsidered.
  • Propranolol (oral): After delivery, increased dosage in pregnancy is reconsidered.
  • Flecainide: Not given in structural or ischemic heart disease. If there is no pre-excitation (WPW), it is used together with an AV node blocker such as a beta blocker against the risk of 1:1 AV conduction in atrial flutter; In WPW, no AV node blocker is added. After delivery, the increased dose during pregnancy is re-evaluated.
Bradyarrhythmia, conduction disturbance and permanent device (pacemaker, ICD)
Device / indication
Bradyarrhythmia or conduction disturbance — pacemaker indication or carriage

Approach

  1. Identify conduction disturbances with 12-lead ECG; Evaluate with rhythm monitoring, electrolytes, thyroid functions, complete blood count and ECHO.
  2. In case of hemodynamic instability findings, start treatment and ensure maternal stabilization without waiting for detailed rhythm classification.
  3. Pacemaker indications do not change during pregnancy.
  4. Let cardiology, obstetrics, anesthesia and neonatology plan the timing of device operation, radiation reduction measures and the emergency plan together.
  5. Conduct maternal rhythm management in conjunction with fetal monitoring.
  6. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  7. Put the birth and puerperium follow-up plan in writing.
  8. Plan proactive monitoring during the first weeks of postpartum and early check-up before discharge; Teach the warning signs.

Treatment

  • Atropine for unstable bradycardia with a pulse: 1 mg IV bolus, repeatable every 3–5 minutes, maximum 3 mg total; Skip atropine in complete block with wide QRS or Mobitz type II block.
  • If no or missed response to atropine: temporary transvenous pacing (transcutaneous pacing if not immediately available) and/or chronotropic infusion—dopamine 5–20 micrograms/kg/min IV or adrenaline 2–10 micrograms/min IV (either), titrated to response; Isoproterenol may be used if the benefit outweighs the risk.
  • Pacemaker implantation: if indicated, implement with a plan adapted to maternal–fetal safety.

Note

  • Prophylactic temporary pacemaker is not recommended at birth with asymptomatic congenital complete AV block, normal cardiac structure and function, narrow QRS, and acceptable ventricular rate (≥ 50/min).
  • If there is no structural heart disease, no intervention is required in asymptomatic sinus bradycardia and Mobitz type I block.
  • Consider supine hypotensive syndrome in hypotension/syncope that develops when lying supine after the 20th week of pregnancy; Lay the patient on the left side.
ICD indication, ICD carriage, or history of ICD therapy

Approach

  1. Perform pre-pregnancy specialist cardiology evaluation in cases of ICD therapy history.
  2. Assess ventricular function, ischemic disease, cardiomyopathy, hereditary channelopathies, and family history of sudden cardiac death; Assess the need for electrophysiology consultation.
  3. Consider previous sustained VT/VF as high-risk phenotype; refer to a tertiary center with a low threshold and evaluate in a multidisciplinary committee; Risk stratify with mWHO and CARPREG II; previous arrhythmia is 3 points on CARPREG II.
  4. ICD indications do not change during pregnancy; Let cardiology, obstetrics, anesthesia and neonatology plan the timing of device operation, radiation reduction measures and emergency plan together.
  5. Perform routine prenatal ICD interrogation; Include device function and programming plan during labor (including electrocautery for cesarean section) in the written plan with the electrophysiology team.
  6. Determine the birth order emergency algorithm and team task distribution in writing.
  7. Conduct maternal rhythm management in conjunction with fetal monitoring.
  8. Determine the mode of delivery according to obstetric indications; Isolated arrhythmia alone is not an indication for cesarean section.
  9. Proactively monitor for recurrence during the first postpartum weeks; Schedule early check-ups before discharge, teach warning signs, and review antiarrhythmic therapy for breastfeeding compatibility.

Treatment

  • ICD implantation: if indicated, perform with a plan adapted to maternal–fetal safety.
  • On admission with ICD shock: interrogate the device, exclude triggers (heart failure, electrolyte disturbance, ischemia, infection); administer advanced life support and antiarrhythmic therapy in appropriate shock; In case of inappropriate shock (SVT, T wave oversensing), reprogram the device, if there is no programmer, put a magnet on the ICD.
  • If hemodynamically unstable — Synchronized electrical cardioversion: initial energy on biphasic device 200 J in AF and atrial flutter, 100 J in narrow QRS tachycardia and monomorphic VT (unsynchronized high-energy shock in polymorphous VT); pads are placed sterno-apically or antero-posteriorly, away from the breast tissue; Sedation is applied when possible (anesthesia is preferred if anesthesia service is available); Because the device often returns to asynchronous mode after the shock, synchronous mode is reactivated before each new shock; If synchronization is delayed and the situation is critical, non-synchronous shock is immediately applied.
  • In pulseless VT/VF — Defibrillation: manufacturer's recommended energy on a biphasic device (initial 120–200 J), highest device energy if unknown; subsequent shocks with the same or higher energy; 360 J in monophasic device; The pads are placed anterolaterally, and the lateral pad is placed under or next to the breast tissue.
  • If there is no pulse (cardiac arrest in pregnancy): immediately initiate high-quality cardiopulmonary resuscitation (CPR) and cardiac arrest algorithm; if the fundus is at or above the umbilicus, perform continuous manual left lateral uterine displacement with chest compression; Start resuscitative birth preparation at the diagnosis of arrest; If spontaneous circulation does not return with standard resuscitation, aim to complete the birth at the arrest site within the first 5 minutes.
  • Beta blocker required — Metoprolol (oral): immediate-release tartrate start at 25 mg twice daily, gradually increasing as tolerated, to a maximum of 200 mg daily, divided into 2–4 doses; Long-acting succinate is started at 25-50 mg once a day, gradually increasing as tolerated, to a maximum of 200 mg per day, divided into 1-2 doses.
  • Consider catheter ablation in refractory arrhythmia with individual risk–benefit assessment in an experienced center.

Note

  • In ARVC, do not discontinue beta-blocker therapy, including sotalol, during pregnancy or the postpartum period; reprogram the ICD appropriately.
  • Monitor fetal growth and fetal bradycardia on beta blocker therapy. If the mother used beta blockers, monitor the newborn for bradycardia and hypoglycemia.
  • Avoid amiodarone due to risk of fetal toxicity; Use only in life-threatening refractory arrhythmia.
  • Wearable cardioverter defibrillator may be considered in ICD indication due to a reversible cause.
  • Synchronized electrical cardioversion: The energy during pregnancy is the same as in non-pregnancy; The procedure is not delayed due to concerns about harm to the fetus; If the fetal viability limit is exceeded, the fetal heart rate is monitored after the procedure and it is preferred to perform the procedure in a center where emergency cesarean section can be performed.
  • Defibrillation: During pregnancy, the energy is the same as in non-pregnancy; if the fundus is at or above the umbilicus, manual left lateral uterine displacement is performed with chest compressions; Once cardiac arrest is diagnosed, external and internal fetal monitors are removed; Resuscitation and shock are not delayed for fetal assessment, fetal monitoring, or removal of monitors.
  • Metoprolol (oral): Do not start or increase the dose in decompensated heart failure; It is not used in second-third degree AV block, sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia and cardiogenic shock. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.

Hypertensive diseases

Hypertensive heart disease (left ventricular hypertrophy / diastolic dysfunction)
Clinical situation
Asymptomatic (left ventricular hypertrophy or diastolic dysfunction on ECHO)

Approach

  1. Define concomitant chronic hypertension, gestational hypertension, preeclampsia, or preeclampsia added to chronic hypertension; do not substitute cardiac involvement for this obstetric diagnosis.
  2. Measure BP with correct technique at each visit and systematically question symptoms; Perform baseline laboratory evaluation for preeclampsia; Monitor for signs of preeclampsia, renal function, and fetal growth.
  3. Evaluate left ventricular systolic function, mass index, wall thickness, diastolic function, left atrial volume, right ventricular function, and pulmonary pressures by ECG and ECHO; Do not delay cardiac evaluation if rapidly increasing dyspnea, orthopnea, paroxysmal nocturnal dyspnea, chest pain, syncope, marked palpitations, decreased oxygen saturation, or rales occur.
  4. If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  5. Determine the time of birth in cardiac involvement due to preeclampsia by evaluating the condition of the mother and the fetus together; cardiac findings can directly determine the time of birth.
  6. Closely monitor BP in the first week postpartum (may rise again in the first days, there is a risk of serious hypertension); If hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs, consider cardiology evaluation and ECHO.
  7. Plan long-term follow-up including BP control, weight management, glucose and lipid assessment, renal function and lifestyle counseling.

Treatment

  • Oral antihypertensive therapy: initiate or continue if systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg (on the basis of chronic, gestational hypertension or preeclampsia); Titrate to target BP <140/90 mmHg.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents during pregnancy.
  • Reconsider the choice of antihypertensive postpartum based on clinical presentation and compatibility with breastfeeding.
  • Even small fluid changes in diastolic dysfunction can lead to pulmonary edema; closely monitor fluid balance and avoid iatrogenic fluid overload.
  • Do not use BNP/NT-proBNP as a routine screening test.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
There is evidence of cardiac stimulation, no pulmonary edema

Approach

  1. Define concomitant chronic hypertension, gestational hypertension, preeclampsia, or preeclampsia added to chronic hypertension; do not substitute cardiac involvement for this obstetric diagnosis.
  2. Treat rapidly worsening breathlessness, orthopnea, paroxysmal nocturnal dyspnea, chest pain, syncope, marked palpitations, reduced oxygen saturation and crackles as warning signs requiring cardiac assessment; assess breathlessness together with BP, oxygen saturation, lung examination and urine output, and do not assume that it is physiological in preeclampsia.
  3. Schedule ECG and ECHO; assess left ventricular systolic and diastolic function, mass index, wall thickness, left atrial volume, right ventricular function, and pulmonary pressures; In case of suspicion of heart failure or pulmonary edema, request BNP/NT-proBNP and interpret it together with the clinical picture, examination, ECHO and laboratory (it does not make a diagnosis alone).
  4. Multidisciplinary evaluation with cardiology, maternal-fetal medicine, anesthesia and intensive care when necessary.
  5. Perform baseline laboratory evaluation for preeclampsia: complete blood count and platelet, creatinine, AST/ALT, bilirubin, and quantitative urine protein; Monitor fetal growth, renal function, and signs of preeclampsia.
  6. If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  7. Determine the time of birth in cardiac involvement due to preeclampsia by evaluating the condition of the mother and the fetus together; cardiac findings can directly determine the time of birth.
  8. Monitor BP closely during the first week postpartum; If hypertension persists or there is evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema, perform a cardiology evaluation and ECHO; Plan long-term follow-up including BP control, weight management, glucose and lipid assessment, kidney function and lifestyle counseling.

Treatment

  • Oral antihypertensive therapy: initiate or continue if systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg (on the basis of chronic, gestational hypertension or preeclampsia); Titrate to target BP <140/90 mmHg.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents during pregnancy.
  • Reconsider the choice of antihypertensive postpartum based on clinical presentation and compatibility with breastfeeding.
  • Even small fluid changes in diastolic dysfunction can lead to pulmonary edema; closely monitor fluid balance and avoid iatrogenic fluid overload.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Pulmonary edema or acute heart failure

Approach

  1. Define concomitant chronic hypertension, gestational hypertension, preeclampsia, or preeclampsia added to chronic hypertension; do not substitute cardiac involvement for this obstetric diagnosis.
  2. Do not manage as an outpatient; Multidisciplinary management with cardiology, maternal-fetal medicine, anesthesia and intensive care in a tertiary care center; Closely monitor oxygen saturation, lung examination, urine output, and fluid balance.
  3. Perform ECG and ECHO; assess left and right ventricular function, diastolic function, and pulmonary pressures; Request BNP/NT-proBNP and interpret it together with the clinical picture, examination, ECHO and laboratory (it does not make a diagnosis alone).
  4. Perform baseline laboratory evaluation for preeclampsia; If it is accompanied by preeclampsia, consider pulmonary edema as a serious finding.
  5. If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If BP remains above threshold or clinical deterioration occurs, seek urgent consultation with maternal-fetal medicine, anesthesiologist and intensivist.
  6. Monitor fetal growth and well-being; Perform delivery regardless of gestational age after stabilizing the mother in pulmonary edema due to preeclampsia; In pulmonary edema caused by another cause, determine the time of birth in a multidisciplinary manner based on the mother-fetus status and cardiac findings.
  7. Monitor BP closely during the first week postpartum; If hypertension persists or there is evidence of shortness of breath, chest pain, orthopnea or pulmonary edema, perform a cardiology evaluation and ECHO.
  8. Plan long-term follow-up including BP control, weight management, glucose and lipid assessment, renal function and lifestyle counseling.

Treatment

  • Implement fluid restriction.
  • Oxygen: If SpO2 is less than 90% or PaO2 is less than 60 mmHg, oxygen is given; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If necessary — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Nitroglycerin: Start with 5 µg/min IV infusion, increase every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • Rapid-acting antihypertensive (IV hydralazine or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If preeclampsia is also present, start magnesium sulfate alongside stabilization and the decision to deliver, without waiting for labor (if cesarean birth is planned before labor, start before surgery and continue throughout): a 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; continue the infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in pulmonary edema.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents during pregnancy.
  • Reconsider the choice of antihypertensive postpartum based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
Chronic hypertension
Period
Pre-pregnancy (pregnancy is planned)

Approach

  1. Describe the increased risk of preeclampsia added to pregnancy; State that antenatal follow-up will be planned more closely from the beginning.
  2. Make medication changes before pregnancy: switch to pregnancy-compatible agent and allow sufficient time to verify maternal stability on the new regimen.
  3. If assisted reproduction is planned, consult before treatment about the increased risk of hypertensive pregnancy disorders, including preeclampsia, and make an individual follow-up plan.

Treatment

  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Discontinue ACE inhibitor and angiotensin receptor blocker before pregnancy.
  • Avoid aldosterone antagonists, especially spironolactone.
  • Avoid atenolol.
  • If using statins, plan to discontinue before pregnancy; Make an individual decision with expert evaluation in selected very high-risk patients.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Pregnancy (no additional evidence of preeclampsia)

Approach

  1. Plan antenatal follow-up more closely from the beginning due to the added risk of preeclampsia: measure BP with correct technique at each visit, systematically question symptoms and monitor fetal growth.
  2. Perform baseline laboratory evaluation for preeclampsia: complete blood count and platelets, creatinine, AST/ALT, bilirubin, and quantitative urine protein.
  3. Suspect superimposed preeclampsia if new proteinuria, sudden increase in existing proteinuria, marked worsening of BP control, or serious findings occur.
  4. Consider ECHO for cardiac structure and function in chronic or resistant hypertension; Perform ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, desaturation or pulmonary edema.
  5. If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  6. Closely monitor BP in the first week postpartum (may rise again in the first days, there is a risk of serious hypertension); If hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs, consider cardiology evaluation and ECHO.
  7. Plan long-term follow-up including BP control, weight management, glucose and lipid assessment, renal function and lifestyle counseling.

Treatment

  • Oral antihypertensive therapy: initiate or maintain at threshold of 140/90 mmHg; Titrate dose to this threshold.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, spironolactone, and other RAAS-suppressing agents; If so, stop using it and switch to an agent compatible with pregnancy.
  • Avoid atenolol.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Postpartum

Approach

  1. Closely monitor BP in the first week postpartum (may rise again in the first days, there is a risk of serious hypertension); If hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs, consider cardiology evaluation and ECHO.
  2. Plan long-term follow-up including BP control, weight management, glucose and lipid assessment, renal function and lifestyle counseling.
  3. Assess the risk of postpartum VTE (hypertension and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
Gestational hypertension
Severity
Not serious (BP <160/110 mmHg, no proteinuria and organ dysfunction)
Week of pregnancy
37+0 weeks and beyond

Approach

  1. Monitor maternal and fetal growth closely for progression to preeclampsia; Systematically inquire about headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and sudden increase in edema.
  2. Evaluate for proteinuria (≥300 mg in 24-hour urine, spot protein/creatinine ratio ≥0.3, or 2+ on dipstick if quantitative measurement is not available) and organ dysfunction with complete blood count and platelets, creatinine, AST/ALT, and bilirubin.
  3. If thrombocytopenia, creatinine increase, transaminase elevation, pulmonary edema, new unexplained headache or visual symptoms develop, consider the diagnosis of preeclampsia even without proteinuria.
  4. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  5. Plan delivery at 37+0 weeks and later (labor induction is a suitable option); Determine the type of delivery according to the gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications.
  6. Closely monitor BP during the first week postpartum (may rise again in the first days); Assess the risk of VTE (hypertension and caesarean section are risk factors) and individualize thromboprophylaxis according to risk stratification.
  7. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.

Treatment

  • Oral antihypertensive therapy: start when systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg.
  • Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.
  • Nifedipine (long-acting, oral): Start at 30–60 mg once daily and increase at intervals of 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Methyldopa (oral): Start at 250 mg 2–3 times a day, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Before 37+0 weeks

Approach

  1. Monitor maternal and fetal growth closely for progression to preeclampsia; Systematically inquire about headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and sudden increase in edema.
  2. Evaluate for proteinuria (≥300 mg in 24-hour urine, spot protein/creatinine ratio ≥0.3, or 2+ on dipstick if quantitative measurement is not available) and organ dysfunction with complete blood count and platelets, creatinine, AST/ALT, and bilirubin.
  3. If thrombocytopenia, creatinine increase, transaminase elevation, pulmonary edema, new unexplained headache or visual symptoms develop, consider the diagnosis of preeclampsia even without proteinuria; Use the sFlt-1/PlGF ratio to assess the likelihood of preeclampsia and the short-term risk of progression in selected cases, but not to make or exclude the diagnosis.
  4. Consider outpatient follow-up only in selected patients who are asymptomatic, have stable maternal and fetal conditions, comply with frequent check-ups, have access to hospital, and have reliable home BP measurement.
  5. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals; If severe hypertension or organ dysfunction develops, reorganize management on the same basis as for preeclampsia.
  6. If no serious findings develop and the mother-fetus condition remains stable, plan the birth for 37+0 weeks and later; Determine the type of delivery according to the gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications.
  7. Closely monitor BP during the first week postpartum (may rise again in the first days); Assess the risk of VTE (hypertension and caesarean section are risk factors) and individualize thromboprophylaxis according to risk stratification.
  8. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.

Treatment

  • Oral antihypertensive therapy: start when systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg.
  • Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.
  • Nifedipine (long-acting, oral): Start at 30–60 mg once daily and increase at intervals of 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Methyldopa (oral): Start at 250 mg 2–3 times a day, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Severe hypertension (BP ≥160/110 mmHg)
Week of pregnancy
34+0 weeks and beyond

Approach

  1. Manage gestational hypertension with systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg without proteinuria or organ dysfunction on the same basis as preeclampsia; Re-evaluate the diagnosis of preeclampsia if proteinuria or organ dysfunction suggestive of preeclampsia develops.
  2. Do not manage as an outpatient; hospitalize and rapidly assess maternal condition; Evaluate the fetus with fetal biometry, amniotic fluid and fetal well-being tests appropriate to the gestational age.
  3. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  6. Perform delivery after stabilizing the mother; Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  7. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  8. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  9. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Oral antihypertensive therapy: initiate or continue for maintenance if BP is 140–159/90–109 mmHg or after acute severe hypertension is controlled; Titrate to target BP <140/90 mmHg.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: in severe cases, initiate labor without waiting, together with the decision to deliver and stabilization (if cesarean section is to be performed before the labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, then 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Before 34+0 weeks

Approach

  1. Manage gestational hypertension with systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg without proteinuria or organ dysfunction on the same basis as preeclampsia; Re-evaluate the diagnosis of preeclampsia if proteinuria or organ dysfunction suggestive of preeclampsia develops.
  2. Do not manage as an outpatient; Quickly evaluate the mother and fetus condition by hospitalizing them in a tertiary care center.
  3. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  6. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; Pay attention to fetal growth restriction and oligohydramnios in early-onset disease. If growth restriction is suspected or biometry is abnormal, add umbilical artery Doppler.
  7. Consider short-term expectant management only in carefully selected stable cases, under close monitoring in a tertiary center, for antenatal corticosteroid administration and fetal maturation.
  8. Stabilize the mother and deliver regardless of gestational age in case of any deterioration in the mother or the fetus, especially if there is one of the following: severe BP uncontrolled despite the maximum dose, treatment-resistant headache, epigastric/right upper quadrant pain unresponsive to repeated analgesics, visual impairment, motor deficit or altered consciousness, stroke, myocardial infarction, HELLP, new or worsening renal dysfunction, pulmonary edema, eclampsia, suspicion of placental abruption; unreassuring fetal test, fetal death, persistent reverse diastolic flow in the umbilical artery.
  9. Plan delivery at 34+0 weeks even if the mother and fetus remain stable during expectant management; do not use expectant management if the newborn is not expected to survive; do not delay indicated delivery to complete a course of corticosteroids.
  10. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  11. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  12. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  13. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Single course if birth is expected within 7 days, pregnancy is at 23+0–33+6 weeks (from 22+0 weeks if active neonatal care is planned) and no previous cycle — Antenatal corticosteroid: betamethasone 12 mg IM, 2 doses 24 hours apart or dexamethasone 6 mg IM, 4 doses 12 hours apart.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Oral antihypertensive therapy: initiate or continue for maintenance if BP is 140–159/90–109 mmHg or after acute severe hypertension is controlled; Titrate to target BP <140/90 mmHg.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate (seizure prophylaxis: start at initial evaluation, continue until completion of antenatal corticosteroid course (approximately 48 hours); discontinue if expectant management is to be continued, restart when labor is indicated) — 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Avoid continuing antepartum magnesium prophylaxis for more than 48 hours.
  • Below 32 weeks, magnesium sulfate also provides neonatal neuroprotection in preterm birth.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Preeclampsia
Serious finding
No serious findings
Week of pregnancy
37+0 weeks and beyond

Approach

  1. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  2. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  3. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age.
  4. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg before or after birth, confirm by measuring again within 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  5. Plan delivery at 37+0 weeks and later (labor induction is a suitable option); Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  6. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  7. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  8. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: start when systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg.
  • Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.
  • Nifedipine (long-acting, oral): Start at 30–60 mg once daily and increase at intervals of 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Methyldopa (oral): Start at 250 mg 2–3 times a day, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: in the absence of severe features, base use on clinical risk and local practice; if severe features develop, start magnesium sulfate alongside stabilization and the decision to deliver, without waiting for labor (if cesarean birth is planned before labor, start before surgery and continue throughout): a 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; continue the infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
34+0–36+6 weeks

Approach

  1. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  2. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  3. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; If growth restriction is suspected or biometry is impaired, add umbilical artery Doppler.
  4. Consider outpatient follow-up only in selected patients who are asymptomatic, have stable maternal and fetal conditions, comply with frequent check-ups, have access to hospital, and have reliable home BP measurement; If serious findings develop, discontinue outpatient monitoring and re-determine monitoring intensity, magnesium sulfate requirement, antihypertensive treatment and delivery time according to serious findings.
  5. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg before or after birth, confirm by measuring again within 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  6. Approach selectively at 34+0–36+6 weeks: if mother and fetus are stable, consider expectant management until 37+0 weeks with full counseling and close monitoring; planned preterm birth reduces adverse maternal outcomes but increases neonatal intensive care admission, individualize the decision; Reconsider the safety of expectant management in cases of unreassuring fetal testing, gross Doppler abnormality, significant growth restriction, or suspected placental abruption.
  7. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  8. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  9. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  10. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: start when systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg.
  • Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.
  • Nifedipine (long-acting, oral): Start at 30–60 mg once daily and increase at intervals of 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Methyldopa (oral): Start at 250 mg 2–3 times a day, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: in the absence of severe features, base use on clinical risk and local practice; if severe features develop, start magnesium sulfate alongside stabilization and the decision to deliver, without waiting for labor (if cesarean birth is planned before labor, start before surgery and continue throughout): a 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; continue the infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Before 34+0 weeks

Approach

  1. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  2. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  3. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; Pay attention to fetal growth restriction and oligohydramnios in early-onset disease. If growth restriction is suspected or biometry is abnormal, add umbilical artery Doppler.
  4. Consider outpatient follow-up only in selected patients who are asymptomatic, have stable maternal and fetal conditions, comply with frequent check-ups, have access to hospital, and have reliable home BP measurement; If serious findings develop, discontinue outpatient monitoring and re-determine monitoring intensity, magnesium sulfate requirement, antihypertensive treatment and delivery time according to serious findings.
  5. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg before or after birth, confirm by measuring again within 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  6. Individualize time of birth according to disease severity, maternal and fetal stability, and gestational age; If no serious findings develop, delivery is recommended at 37+0 weeks and later; the approach is selective at 34+0–36+6 weeks; Reconsider the safety of expectant management in cases of unreassuring fetal testing, gross Doppler abnormality, significant growth restriction, or suspected placental abruption.
  7. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  8. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  9. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  10. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: start when systolic BP ≥140 mmHg or diastolic BP ≥90 mmHg.
  • Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.
  • Nifedipine (long-acting, oral): Start at 30–60 mg once daily and increase at intervals of 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Methyldopa (oral): Start at 250 mg 2–3 times a day, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: unless serious findings, determine use based on clinical risk and facility practice; Seizure prophylaxis if serious findings develop: start with initial evaluation and continue until completion of antenatal corticosteroid course (approximately 48 hours); If expectant management is to be continued, discontinue, restart when indication for delivery occurs — 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Avoid continuing antepartum magnesium prophylaxis for more than 48 hours.
  • Below 32 weeks, magnesium sulfate also provides neonatal neuroprotection in preterm birth.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
  • Metoprolol (oral): Its metabolism increases in mid and late pregnancy; If the clinical response is insufficient, the dose and dosing frequency are adjusted.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
There are serious findings (BP ≥160/110 mmHg, platelets <100,000/mm³, creatinine >1.1 mg/dL or 2 times baseline (if there is no other renal disease), AST/ALT ≥2 times the upper limit of normal, unexplained persistent right upper quadrant/epigastric pain, pulmonary edema, new headache unresponsive to treatment or visual symptoms)
Week of pregnancy
34+0 weeks and beyond

Approach

  1. Do not manage as an outpatient; hospitalize and rapidly assess maternal condition; Evaluate the fetus with fetal biometry, amniotic fluid and fetal well-being tests appropriate to the gestational age.
  2. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  3. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  4. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  5. In known chronic kidney disease, do not apply the renal criterion solely using an absolute creatinine threshold; assess new deterioration from baseline together with other signs of preeclampsia.
  6. Perform delivery after stabilizing the mother; Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  7. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  8. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  9. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Oral antihypertensive therapy: initiate or continue for maintenance if BP is 140–159/90–109 mmHg or after acute severe hypertension is controlled; Titrate to target BP <140/90 mmHg.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: in severe cases, initiate labor without waiting, together with the decision to deliver and stabilization (if cesarean section is to be performed before the labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, then 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Do not use labetalol (IV or oral) if the serious finding is pulmonary edema or acute heart failure; Apply 'Acute severe hypertension › Pulmonary edema or heart failure is present' in the algorithm for fluid restriction, oxygen, IV nitroglycerin and, if necessary, IV furosemide.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Before 34+0 weeks

Approach

  1. Do not manage as an outpatient; Quickly evaluate the mother and fetus condition by hospitalizing them in a tertiary care center.
  2. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  3. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  4. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  5. In known chronic kidney disease, do not apply the renal criterion solely using an absolute creatinine threshold; assess new deterioration from baseline together with other signs of preeclampsia.
  6. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; Pay attention to fetal growth restriction and oligohydramnios in early-onset disease. If growth restriction is suspected or biometry is abnormal, add umbilical artery Doppler.
  7. Consider short-term expectant management only in carefully selected stable cases, under close monitoring in a tertiary center, for antenatal corticosteroid administration and fetal maturation.
  8. Stabilize the mother and deliver regardless of gestational age in case of any deterioration in the mother or the fetus, especially if there is one of the following: severe BP uncontrolled despite the maximum dose, treatment-resistant headache, epigastric/right upper quadrant pain unresponsive to repeated analgesics, visual impairment, motor deficit or altered consciousness, stroke, myocardial infarction, HELLP, new or worsening renal dysfunction, pulmonary edema, eclampsia, suspicion of placental abruption; unreassuring fetal test, fetal death, persistent reverse diastolic flow in the umbilical artery.
  9. Plan delivery at 34+0 weeks even if the mother and fetus remain stable during expectant management; do not use expectant management if the newborn is not expected to survive; do not delay indicated delivery to complete a course of corticosteroids.
  10. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  11. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  12. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  13. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Single course if birth is expected within 7 days, pregnancy is at 23+0–33+6 weeks (from 22+0 weeks if active neonatal care is planned) and no previous cycle — Antenatal corticosteroid: betamethasone 12 mg IM, 2 doses 24 hours apart or dexamethasone 6 mg IM, 4 doses 12 hours apart.
  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Oral antihypertensive therapy: initiate or continue for maintenance if BP is 140–159/90–109 mmHg or after acute severe hypertension is controlled; Titrate to target BP <140/90 mmHg.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate (seizure prophylaxis: start at initial evaluation, continue until completion of antenatal corticosteroid course (approximately 48 hours); discontinue if expectant management is to be continued, restart when labor is indicated) — 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Do not use labetalol (IV or oral) if the serious finding is pulmonary edema or acute heart failure; Apply 'Acute severe hypertension › Pulmonary edema or heart failure is present' in the algorithm for fluid restriction, oxygen, IV nitroglycerin and, if necessary, IV furosemide.
  • Avoid continuing antepartum magnesium prophylaxis for more than 48 hours.
  • Below 32 weeks, magnesium sulfate also provides neonatal neuroprotection in preterm birth.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Preeclampsia added to chronic hypertension
Serious finding
No serious findings
Week of pregnancy
37+0 weeks and beyond

Approach

  1. New proteinuria, sudden increase in existing proteinuria, significant deterioration in BP control, or the occurrence of serious findings are suggestive of superimposed preeclampsia; Be careful to distinguish pregnancy physiology from the effects of chronic disease in diagnosis.
  2. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  3. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  4. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age.
  5. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg before or after birth, confirm by measuring again within 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  6. Plan delivery at 37+0 weeks and later (labor induction is a suitable option); Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  7. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  8. Continue long-term monitoring of chronic hypertension; Monitor BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function.
  9. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: continue current therapy compatible with pregnancy; If not receiving treatment, start at the 140/90 mmHg threshold and titrate to that threshold.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: in the absence of severe features, base use on clinical risk and local practice; if severe features develop, start magnesium sulfate alongside stabilization and the decision to deliver, without waiting for labor (if cesarean birth is planned before labor, start before surgery and continue throughout): a 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; continue the infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers and other RAAS-suppressing agents, and atenolol.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
34+0–36+6 weeks

Approach

  1. New proteinuria, sudden increase in existing proteinuria, significant deterioration in BP control, or the occurrence of serious findings are suggestive of superimposed preeclampsia; Be careful to distinguish pregnancy physiology from the effects of chronic disease in diagnosis.
  2. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  3. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  4. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; If growth restriction is suspected or biometry is impaired, add umbilical artery Doppler.
  5. Consider outpatient follow-up only in selected patients who are asymptomatic, have stable maternal and fetal conditions, comply with frequent check-ups, have access to hospital, and have reliable home BP measurement; If serious findings develop, discontinue outpatient monitoring and re-determine monitoring intensity, magnesium sulfate requirement, antihypertensive treatment and delivery time according to serious findings.
  6. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg before or after birth, confirm by measuring again within 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  7. Approach selectively at 34+0–36+6 weeks: if mother and fetus are stable, consider expectant management until 37+0 weeks with full counseling and close monitoring; planned preterm birth reduces adverse maternal outcomes but increases neonatal intensive care admission, individualize the decision; Reconsider the safety of expectant management in cases of unreassuring fetal testing, gross Doppler abnormality, significant growth restriction, or suspected placental abruption.
  8. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  9. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  10. Continue long-term monitoring of chronic hypertension; Monitor BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function.
  11. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: continue current therapy compatible with pregnancy; If not receiving treatment, start at the 140/90 mmHg threshold and titrate to that threshold.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: in the absence of severe features, base use on clinical risk and local practice; if severe features develop, start magnesium sulfate alongside stabilization and the decision to deliver, without waiting for labor (if cesarean birth is planned before labor, start before surgery and continue throughout): a 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; continue the infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers and other RAAS-suppressing agents, and atenolol.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Before 34+0 weeks

Approach

  1. New proteinuria, sudden increase in existing proteinuria, significant deterioration in BP control, or the occurrence of serious findings are suggestive of superimposed preeclampsia; Be careful to distinguish pregnancy physiology from the effects of chronic disease in diagnosis.
  2. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  3. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  4. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; Pay attention to fetal growth restriction and oligohydramnios in early-onset disease. If growth restriction is suspected or biometry is abnormal, add umbilical artery Doppler.
  5. Consider outpatient follow-up only in selected patients who are asymptomatic, have stable maternal and fetal conditions, comply with frequent check-ups, have access to hospital, and have reliable home BP measurement; If serious findings develop, discontinue outpatient monitoring and re-determine monitoring intensity, magnesium sulfate requirement, antihypertensive treatment and delivery time according to serious findings.
  6. Measure BP with correct technique; If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg before or after birth, confirm by measuring again within 15 minutes; if persistent, initiate emergency treatment with IV labetalol, IV hydralazine, or oral immediate-release nifedipine as soon as possible, no later than 30–60 minutes; Apply the 'Acute severe hypertension' selection in the algorithm for doses and repeat intervals.
  7. Individualize time of birth according to disease severity, maternal and fetal stability, and gestational age; If no serious findings develop, delivery is recommended at 37+0 weeks and later; the approach is selective at 34+0–36+6 weeks; Reconsider the safety of expectant management in cases of unreassuring fetal testing, gross Doppler abnormality, significant growth restriction, or suspected placental abruption.
  8. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  9. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  10. Continue long-term monitoring of chronic hypertension; Monitor BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function.
  11. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: continue current therapy compatible with pregnancy; If not receiving treatment, start at the 140/90 mmHg threshold and titrate to that threshold.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Magnesium sulfate: unless serious findings, determine use based on clinical risk and facility practice; Seizure prophylaxis if serious findings develop: start with initial evaluation and continue until completion of antenatal corticosteroid course (approximately 48 hours); If expectant management is to be continued, discontinue, restart when indication for delivery occurs — 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Avoid ACE inhibitors, angiotensin receptor blockers and other RAAS-suppressing agents, and atenolol.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Avoid continuing antepartum magnesium prophylaxis for more than 48 hours.
  • Below 32 weeks, magnesium sulfate also provides neonatal neuroprotection in preterm birth.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
There are serious findings (BP ≥160/110 mmHg, platelets <100,000/mm³, creatinine >1.1 mg/dL or 2 times baseline (if there is no other renal disease), AST/ALT ≥2 times the upper limit of normal, unexplained persistent right upper quadrant/epigastric pain, pulmonary edema, new headache unresponsive to treatment or visual symptoms)
Week of pregnancy
34+0 weeks and beyond

Approach

  1. New proteinuria, sudden increase in existing proteinuria, significant deterioration in BP control, or the occurrence of serious findings are suggestive of superimposed preeclampsia; Be careful to distinguish pregnancy physiology from the effects of chronic disease in diagnosis.
  2. Do not manage as an outpatient; hospitalize and rapidly assess maternal condition; Evaluate the fetus with fetal biometry, amniotic fluid and fetal well-being tests appropriate to the gestational age.
  3. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  6. In known chronic kidney disease, do not apply the renal criterion solely using an absolute creatinine threshold; assess new deterioration from baseline together with other signs of preeclampsia.
  7. Perform delivery after stabilizing the mother; Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  8. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  9. Continue long-term monitoring of chronic hypertension; BP control, weight management, smoking cessation, monitor lipid profile, glucose metabolism and kidney function; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  10. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Oral antihypertensive therapy: continue current therapy compatible with pregnancy; If not receiving treatment, start at the 140/90 mmHg threshold and titrate to that threshold.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Magnesium sulfate: in severe cases, initiate labor without waiting, together with the decision to deliver and stabilization (if cesarean section is to be performed before the labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, then 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers and other RAAS-suppressing agents, and atenolol.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Do not use labetalol (IV or oral) if the serious finding is pulmonary edema or acute heart failure; Apply 'Acute severe hypertension › Pulmonary edema or heart failure is present' in the algorithm for fluid restriction, oxygen, IV nitroglycerin and, if necessary, IV furosemide.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Before 34+0 weeks

Approach

  1. New proteinuria, sudden increase in existing proteinuria, significant deterioration in BP control, or the occurrence of serious findings are suggestive of superimposed preeclampsia; Be careful to distinguish pregnancy physiology from the effects of chronic disease in diagnosis.
  2. Do not manage as an outpatient; Quickly evaluate the mother and fetus condition by hospitalizing them in a tertiary care center.
  3. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  6. In known chronic kidney disease, do not apply the renal criterion solely using an absolute creatinine threshold; assess new deterioration from baseline together with other signs of preeclampsia.
  7. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; Pay attention to fetal growth restriction and oligohydramnios in early-onset disease. If growth restriction is suspected or biometry is abnormal, add umbilical artery Doppler.
  8. Consider short-term expectant management only in carefully selected stable cases, under close monitoring in a tertiary center, for antenatal corticosteroid administration and fetal maturation.
  9. Stabilize the mother and deliver regardless of gestational age in case of any deterioration in the mother or the fetus, especially if there is one of the following: severe BP uncontrolled despite the maximum dose, treatment-resistant headache, epigastric/right upper quadrant pain unresponsive to repeated analgesics, visual impairment, motor deficit or altered consciousness, stroke, myocardial infarction, HELLP, new or worsening renal dysfunction, pulmonary edema, eclampsia, suspicion of placental abruption; unreassuring fetal test, fetal death, persistent reverse diastolic flow in the umbilical artery.
  10. Plan delivery at 34+0 weeks even if the mother and fetus remain stable during expectant management; do not use expectant management if the newborn is not expected to survive; do not delay indicated delivery to complete a course of corticosteroids.
  11. Determine the mode of delivery according to gestational age, cervical status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  12. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  13. Continue long-term monitoring of chronic hypertension; BP control, weight management, smoking cessation, monitor lipid profile, glucose metabolism and kidney function; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  14. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Single course if birth is expected within 7 days, pregnancy is at 23+0–33+6 weeks (from 22+0 weeks if active neonatal care is planned) and no previous cycle — Antenatal corticosteroid: betamethasone 12 mg IM, 2 doses 24 hours apart or dexamethasone 6 mg IM, 4 doses 12 hours apart.
  • Oral antihypertensive therapy: continue current therapy compatible with pregnancy; If not receiving treatment, start at the 140/90 mmHg threshold and titrate to that threshold.
  • First option — Labetalol (oral): start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • First option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • Alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.
  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Magnesium sulfate (seizure prophylaxis: start at initial evaluation, continue until completion of antenatal corticosteroid course (approximately 48 hours); discontinue if expectant management is to be continued, restart when labor is indicated) — 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers and other RAAS-suppressing agents, and atenolol.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Do not use labetalol (IV or oral) if the serious finding is pulmonary edema or acute heart failure; Apply 'Acute severe hypertension › Pulmonary edema or heart failure is present' in the algorithm for fluid restriction, oxygen, IV nitroglycerin and, if necessary, IV furosemide.
  • Avoid continuing antepartum magnesium prophylaxis for more than 48 hours.
  • Below 32 weeks, magnesium sulfate also provides neonatal neuroprotection in preterm birth.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Eclampsia
Period
Prenatal or labor

Approach

  1. Manage life-threatening obstetric emergency in hospital; Evaluate whether there is another neurological cause that could explain the seizure.
  2. Call for help during seizure (anesthesia and emergency team); protect the patient from injury, position the patient in the left lateral decubitus position, prevent aspiration and keep the airway open; Administer oxygen and monitor vital signs including SpO2. The seizure often stops spontaneously; Magnesium sulfate is given to prevent seizure recurrence. Proceed to delivery only after the mother is stabilized.
  3. In case of seizure lasting more than 20 minutes after bolus or more than two repetitions, immediately involve the anesthesia team for additional anticonvulsant, airway safety (intubation) and intensive care; Evaluate brain imaging.
  4. Verify BP with correct technique; Monitor closely for neurological signs, headache, visual disturbances, epigastric or right upper quadrant pain, shortness of breath, and oliguria.
  5. If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  6. Plan an ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decreased oxygen saturation, or signs of pulmonary edema; do not consider shortness of breath physiological in preeclampsia.
  7. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  8. Evaluate fetal well-being with tests appropriate to the gestational age; do not consider expectant management safe; after stabilizing the mother, give birth regardless of the gestational age; Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  9. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  10. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  11. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Magnesium sulfate: in eclampsia, start magnesium sulfate without waiting for labor; give a 4 g IV loading dose over 5–15 minutes, followed by a 1 g/hour IV maintenance infusion; continue the infusion for 24 hours after birth.
  • If seizures recur under magnesium infusion, give 2–4 g of magnesium sulfate as an additional bolus IV over at least 5 minutes.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Apply the 'Postnatal' selection of the relevant disease in the algorithm for maintenance antihypertensive therapy after delivery; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
Postpartum

Approach

  1. Manage life-threatening obstetric emergency in hospital; Evaluate whether there is another neurological cause that could explain the seizure.
  2. Call for help during seizure (anesthesia and emergency team); protect the patient from injury, place in the left lateral decubitus position, prevent aspiration and keep the airway open; Administer oxygen and monitor vital signs including SpO2. The seizure often stops spontaneously; Magnesium sulfate is given to prevent seizure recurrence.
  3. In case of seizure lasting more than 20 minutes after bolus or more than two repetitions, immediately involve the anesthesia team for additional anticonvulsant, airway safety (intubation) and intensive care; Evaluate brain imaging.
  4. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  6. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  7. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  8. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Magnesium sulfate: continue infusion started before birth at 1 g/hour IV for 24 hours after birth without reloading; If not given before birth, give a loading dose of 4 g IV over 5–15 minutes, followed by 1 g/hour IV for 24 hours.
  • If seizures recur under magnesium infusion, give 2–4 g of magnesium sulfate as an additional bolus IV over at least 5 minutes.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
HELLP syndrome
Period / week of pregnancy
Prenatal, 34+0 weeks and beyond

Approach

  1. Manage hemolysis, elevated liver enzymes, and thrombocytopenia as the severe variant of preeclampsia; do not exclude the diagnosis even in the absence of overt hypertension or classic proteinuria.
  2. Do not manage as an outpatient; hospitalize and rapidly assess maternal condition; evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Manage the non-term patient in a tertiary center with neonatal and obstetric intensive care.
  3. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, LDH, peripheral blood smear, coagulation tests, and quantitative urine protein; Consider acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis.
  6. If there is severe epigastric or right upper quadrant pain unresponsive to opioids, tenderness to liver palpation, shoulder or neck pain, pain on inspiration, hypotension, tachycardia, or greater than expected abdominal distension, even if liver enzymes are not very high, immediately monitor for liver bleeding (subcapsular hematoma or rupture): start with bedside FAST; formal USG, non-contrast MRI or CT if necessary.
  7. Do not use expectant management in HELLP syndrome; After stabilizing the mother, deliver the baby regardless of the gestational age; Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  8. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  9. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  10. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Magnesium sulfate: initiate labor without waiting, with the decision to deliver and stabilization (if cesarean section is to be performed before the labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Platelet transfusion: apply in active bleeding; If the platelet count is below 50,000/mm³ before cesarean section, apply to increase it to 50,000/mm³.
  • If liver hematoma is detected: stabilize hemodynamics, correct severe anemia and coagulopathy with the liver surgery experienced team, then deliver immediately by cesarean section; conservatively monitor the hematoma that is not ruptured and does not expand (USG at 48 hours, 1 week, and 6 weeks after birth); Perform surgical intervention on expanding or ruptured hematoma.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Apply the 'Postnatal' selection of the relevant disease in the algorithm for maintenance antihypertensive therapy after delivery; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • In preeclampsia/HELLP, response to platelet transfusion may be short-lived due to consumption; Monitor with repeat counts.
  • Note that liver failure may also rarely develop in HELLP syndrome.
Prenatal, before 34+0 weeks

Approach

  1. Manage hemolysis, elevated liver enzymes, and thrombocytopenia as the severe variant of preeclampsia; do not exclude the diagnosis even in the absence of overt hypertension or classic proteinuria.
  2. Do not manage as an outpatient; hospitalize and rapidly assess maternal and fetal status; Manage the non-term patient in a tertiary center with neonatal and obstetric intensive care.
  3. Measure BP with correct technique; If systolic BP is ≥160 mmHg and/or diastolic BP is ≥110 mmHg before and after birth, confirm by measuring again within 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, LDH, peripheral blood smear, coagulation tests, and quantitative urine protein; Consider acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis.
  6. If there is severe epigastric or right upper quadrant pain unresponsive to opioids, tenderness to liver palpation, shoulder or neck pain, pain on inspiration, hypotension, tachycardia, or greater than expected abdominal distension, even if liver enzymes are not very high, immediately monitor for liver bleeding (subcapsular hematoma or rupture): start with bedside FAST; formal USG, non-contrast MRI or CT if necessary.
  7. Evaluate the fetus with fetal biometry, amniotic fluid, and fetal well-being tests appropriate for gestational age; Plan NST or biophysical profile according to disease severity and gestational age; Pay attention to fetal growth restriction and oligohydramnios in early-onset disease. If growth restriction is suspected or biometry is abnormal, add umbilical artery Doppler.
  8. Do not use expectant management in HELLP syndrome; After stabilizing the mother, deliver the baby regardless of the gestational age; Determine the mode of delivery according to gestational age, cervix status, fetal presentation, fetal well-being and obstetric indications, regardless of the diagnosis of preeclampsia.
  9. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  10. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  11. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Single course if birth is expected within 7 days, pregnancy is at 23+0–33+6 weeks (from 22+0 weeks if active neonatal care is planned) and no previous cycle — Antenatal corticosteroid: betamethasone 12 mg IM, 2 doses 24 hours apart or dexamethasone 6 mg IM, 4 doses 12 hours apart.
  • Magnesium sulfate: initiate labor without waiting, with the decision to deliver and stabilization (if cesarean section is to be performed before the labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Platelet transfusion: apply in active bleeding; If the platelet count is below 50,000/mm³ before cesarean section, apply to increase it to 50,000/mm³.
  • If liver hematoma is detected: stabilize hemodynamics, correct severe anemia and coagulopathy with the liver surgery experienced team, then deliver immediately by cesarean section; conservatively monitor the hematoma that is not ruptured and does not expand (USG at 48 hours, 1 week, and 6 weeks after birth); Perform surgical intervention on expanding or ruptured hematoma.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Apply the 'Postnatal' selection of the relevant disease in the algorithm for maintenance antihypertensive therapy after delivery; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • In preeclampsia/HELLP, response to platelet transfusion may be short-lived due to consumption; Monitor with repeat counts.
  • Note that liver failure may also rarely develop in HELLP syndrome.
Postpartum

Approach

  1. Manage hemolysis, elevated liver enzymes, and thrombocytopenia as the severe variant of preeclampsia; do not exclude the diagnosis even in the absence of overt hypertension or classic proteinuria.
  2. Do not manage as an outpatient; Admit her to the hospital and evaluate the mother's condition quickly.
  3. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  4. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, LDH, peripheral blood smear, coagulation tests, and quantitative urine protein; Consider acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis.
  5. If there is severe epigastric or right upper quadrant pain unresponsive to opioids, tenderness to liver palpation, shoulder or neck pain, pain on inspiration, hypotension, tachycardia, or greater than expected abdominal distension, even if liver enzymes are not very high, immediately monitor for liver bleeding (subcapsular hematoma or rupture): start with bedside FAST; formal USG, non-contrast MRI or CT if necessary.
  6. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  7. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  8. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Magnesium sulfate: continue infusion started before birth at 1 g/hour IV for 24 hours after birth without reloading; If not given before birth, give a loading dose of 4 g IV over 5–15 minutes, followed by 1 g/hour IV for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.
  • Platelet transfusion: apply in active bleeding.
  • If liver hematoma is detected: stabilize hemodynamics, correct severe anemia and coagulopathy together with the liver surgery experienced team; conservatively monitor the hematoma that is not ruptured and does not expand (USG at 48 hours, 1 week, and 6 weeks after birth); Perform surgical intervention on expanding or ruptured hematoma.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • In preeclampsia/HELLP, response to platelet transfusion may be short-lived due to consumption; Monitor with repeat counts.
  • HELLP findings, subcapsular hematoma, and liver rupture may first appear after birth; Rarely, liver failure may also develop.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
Acute severe hypertension (BP ≥160/110 mmHg)
Period
Pregnancy (until birth)
Clinical situation
No pulmonary edema / heart failure, IV line open

Approach

  1. Verify systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg by remeasurement within 15 minutes using correct technique; if persistent, initiate rapid-acting treatment as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  2. Do not manage as an outpatient; Quickly evaluate the maternal condition in the hospital and evaluate fetal well-being with tests appropriate to the gestational age.
  3. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  4. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  5. Determine the underlying condition: if severe hypertension develops in gestational hypertension, adjust management on the same basis as for preeclampsia; Suspect preeclampsia superimposed on chronic hypertension.
  6. Expectant management may not be safe in resistant severe hypertension; Re-evaluate the time of birth according to maternal and fetal status.

Treatment

  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Nicardipine: start at 5 mg/hour IV via infusion pump; increase by 2.5 mg/hour every 5–15 minutes based on target BP; maximum 15 mg/hour. Stop infusion in unacceptable hypotension or tachycardia; When BP and pulse rate improve, restart at a lower rate of 3–5 mg/hour.
  • Magnesium sulfate in gestational hypertension with severe preeclampsia or severe hypertension: initiate labor without waiting, with the decision to deliver and stabilization (if cesarean section is to be performed before labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Nicardipine: do not use in severe aortic stenosis; closely monitor BP and pulse response in angina, heart failure, hepatic dysfunction or renal dysfunction.
No pulmonary edema / heart failure, no IV line

Approach

  1. Verify systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg by remeasurement within 15 minutes using correct technique; if persistent, initiate rapid-acting treatment as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  2. Do not delay emergency BP treatment with oral immediate-release nifedipine while waiting for IV access; establish IV access at the same time for magnesium sulfate and any IV treatments that may be needed.
  3. Do not manage as an outpatient; Quickly evaluate the maternal condition in the hospital and evaluate fetal well-being with tests appropriate to the gestational age.
  4. Systematically inquire about sudden increase in headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, altered consciousness, oliguria, and edema; Plan ECG and ECHO in cases of shortness of breath, chest pain, orthopnea, significant palpitations, decrease in oxygen saturation or signs of pulmonary edema. Do not accept shortness of breath as physiological in preeclampsia.
  5. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  6. Determine the underlying condition: if severe hypertension develops in gestational hypertension, adjust management on the same basis as for preeclampsia; Suspect preeclampsia superimposed on chronic hypertension.
  7. Expectant management may not be safe in resistant severe hypertension; Re-evaluate the time of birth according to maternal and fetal status.

Treatment

  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Magnesium sulfate in gestational hypertension with severe preeclampsia or severe hypertension: initiate labor without waiting, with the decision to deliver and stabilization (if cesarean section is to be performed before labor begins, start before surgery, continue throughout surgery): 4 g IV loading dose in 5–15 minutes, followed by 1 g/hour IV maintenance; Continue infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
There is pulmonary edema or heart failure

Approach

  1. Verify systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg by remeasurement within 15 minutes using correct technique; if persistent, initiate rapid-acting treatment as soon as possible, no later than 30–60 minutes; If BP remains above threshold or clinical deterioration occurs, seek urgent consultation with maternal-fetal medicine, anesthesiologist and intensivist.
  2. Do not manage as an outpatient; Multidisciplinary management with cardiology, maternal-fetal medicine, anesthesia and intensive care in a tertiary care center; Closely monitor oxygen saturation, lung examination, urine output, and fluid balance.
  3. Perform ECG and ECHO; assess left and right ventricular function, diastolic function, and pulmonary pressures; Request BNP/NT-proBNP and interpret it together with the clinical picture, examination, ECHO and laboratory (it does not make a diagnosis alone).
  4. Perform baseline laboratory evaluation for preeclampsia; If it is accompanied by preeclampsia, consider pulmonary edema as a serious finding.
  5. Evaluate fetal well-being with tests appropriate to the gestational age; Perform delivery regardless of gestational age after stabilizing the mother in pulmonary edema due to preeclampsia; In pulmonary edema caused by another cause, determine the time of birth in a multidisciplinary manner based on the mother-fetus status and cardiac findings.

Treatment

  • Implement fluid restriction.
  • Oxygen: If SpO2 is less than 90% or PaO2 is less than 60 mmHg, oxygen is given; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If necessary — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Nitroglycerin: Start with 5 µg/min IV infusion, increase every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • Nicardipine: start at 5 mg/hour IV via infusion pump; increase by 2.5 mg/hour every 5–15 minutes based on target BP; maximum 15 mg/hour. Stop infusion in unacceptable hypotension or tachycardia; When BP and pulse rate improve, restart at a lower rate of 3–5 mg/hour.
  • If preeclampsia is also present, start magnesium sulfate alongside stabilization and the decision to deliver, without waiting for labor (if cesarean birth is planned before labor, start before surgery and continue throughout): a 4 g IV loading dose over 5–15 minutes, followed by 1 g/hour IV maintenance; continue the infusion for 24 hours after birth.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in pulmonary edema.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension and fetal heart rate disturbances.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid ACE inhibitors, angiotensin receptor blockers, and other RAAS-suppressing agents.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Nicardipine: do not use in severe aortic stenosis; closely monitor BP and pulse response in angina, heart failure, hepatic dysfunction or renal dysfunction.
  • Furosemide (IV): Since excessive diuresis during pregnancy may reduce uterine blood flow and placental perfusion, it is used with caution and the dose is titrated only in cases of pulmonary congestion or volume overload.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
Postpartum
Clinical situation
No pulmonary edema / heart failure

Approach

  1. Confirm postpartum systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg by remeasurement within 15 minutes; if persistent, initiate rapid-acting treatment as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  2. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, monitor BP closely during the first week and describe in detail warning symptoms such as headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema before discharge.
  3. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  4. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  5. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  6. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Magnesium sulfate in gestational hypertension with severe preeclampsia or severe hypertension: continue the infusion started before birth at 1 g/hour IV without reloading for 24 hours after birth; If not given before birth, give a loading dose of 4 g IV over 5–15 minutes, followed by 1 g/hour IV for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
There is pulmonary edema or heart failure

Approach

  1. Confirm postpartum systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg by remeasurement within 15 minutes; if persistent, initiate rapid-acting treatment as soon as possible, no later than 30–60 minutes; If BP remains above threshold or clinical deterioration occurs, seek urgent consultation with maternal-fetal medicine, anesthesiologist and intensivist.
  2. Multidisciplinary management with cardiology, maternal-fetal medicine, anesthesia and intensive care in a tertiary care center; Closely monitor oxygen saturation, lung examination, urine output, and fluid balance.
  3. Perform cardiology evaluation and ECHO; In case of suspicion of heart failure or pulmonary edema, interpret BNP/NT-proBNP together with the clinical picture, examination and ECHO.
  4. Perform baseline laboratory evaluation for preeclampsia; If it is accompanied by preeclampsia, consider pulmonary edema as a serious finding.
  5. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Describe in detail any warning symptoms before discharge, such as headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  6. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  7. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Implement fluid restriction.
  • Oxygen: If SpO2 is less than 90% or PaO2 is less than 60 mmHg, oxygen is given; SpO2 is maintained at least 90%, preferably 95% or above; If SpO2 remains below 95% despite oxygen or respiratory distress (respiratory rate >25/min) persists, noninvasive positive pressure ventilation is applied; Intubation and invasive ventilation are applied in progressive respiratory failure despite oxygen or noninvasive support.
  • If necessary — Furosemide (IV): 40 mg IV, slow bolus over 1–2 minutes in patients who have not previously used diuretics; For those using oral loop diuretics, twice the daily oral dose of furosemide (or equivalent loop diuretic) IV; 2–3 boluses or continuous infusion per day; continuous infusion and high doses (≥160 mg) given at a rate of up to 4 mg/min; maximum 600 mg per day (up to 1000 mg may be considered in severe renal impairment).
  • Nitroglycerin: Start with 5 µg/min IV infusion, increase every 3–5 minutes to a maximum of 100 µg/min; It is given together with a diuretic.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • Magnesium sulfate if accompanied by preeclampsia: continue the infusion started before birth at 1 g/hour IV without reloading for 24 hours after birth; If not given before birth, give a loading dose of 4 g IV over 5–15 minutes, followed by 1 g/hour IV for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.

Note

  • Do not use labetalol in pulmonary edema.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Nitroglycerin: Start if systolic BP is above 110 mmHg.
Postpartum hypertension and long-term cardiovascular follow-up
Postpartum period
First 72 hours
Diagnosis during pregnancy
Severe preeclampsia, eclampsia or HELLP

Approach

  1. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  2. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture; Evaluate HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathy in the differential diagnosis in cases of thrombocytopenia, increased creatinine, elevated transaminases or hemolysis.
  3. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  4. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; add BP control, weight management, smoking cessation, lipid profile, glucose metabolism, and renal function to the long-term follow-up plan; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  5. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Magnesium sulfate: continue infusion started before birth at 1 g/hour IV for 24 hours after birth without reloading; If not given before birth, give a loading dose of 4 g IV over 5–15 minutes, followed by 1 g/hour IV for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
Preeclampsia without serious symptoms

Approach

  1. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  2. Request complete blood count and platelets, serum creatinine, AST/ALT, bilirubin, and quantitative urine protein; Add LDH, peripheral smear, coagulation tests and uric acid according to the clinical picture.
  3. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  4. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  5. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Magnesium sulfate: unless serious findings, determine postpartum use based on clinical risk and facility practice; If serious findings develop, administer 4 g IV load (in 5–15 minutes), followed by 1 g/hour IV for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Avoid iatrogenic fluid overload; Monitor fluid balance as closely as antihypertensive therapy.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
Gestational or chronic hypertension

Approach

  1. Closely monitor BP in the first week postpartum (may rise again in the first days, there is a risk of serious hypertension); If hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs, consider cardiology evaluation and ECHO.
  2. Preeclampsia may develop after birth; Monitor for new proteinuria, organ dysfunction, and warning signs.
  3. In gestational hypertension, if hypertension persists for more than 12 weeks, re-evaluate for chronic hypertension; Plan long-term follow-up including BP control, weight management, glucose and lipid assessment, kidney function and lifestyle counseling.
  4. Assess the risk of postpartum VTE (hypertension and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
There was no hypertension in pregnancy (hypertension or preeclampsia suspected for the first time after birth)

Approach

  1. Confirm new hypertension; inquire about symptoms and evaluate with complete blood count, platelets, creatinine, AST/ALT, bilirubin and quantitative urine protein; Note that preeclampsia can also develop with organ dysfunction without proteinuria.
  2. If there are serious findings, evaluate and monitor in hospital.
  3. If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Closely monitor BP, neurological signs, kidney and liver functions, and platelet count in the first 24–72 hours after birth (eclampsia may also develop after birth); Since BP may rise again, especially in the first week, describe warning symptoms in detail before discharge, such as headache, visual impairment, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  5. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  6. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  7. Assess the risk of postpartum VTE (preeclampsia/eclampsia, hypertension, and cesarean section are risk factors); Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Magnesium sulfate in severe preeclampsia: Give a 4 g IV loading dose over 5–15 minutes, followed by a 1 g/hour IV maintenance infusion for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
72 hours – 12 weeks
Diagnosis during pregnancy
Had hypertensive disorder in pregnancy

Approach

  1. BP may rise again, especially in the first week; Monitor BP closely during the first week and describe in detail warning signs before discharge, such as headache, visual disturbances, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  2. Hypertension may last up to 4 weeks; Monitor renal function and resolution of proteinuria (complete resolution of significant proteinuria may take weeks to months).
  3. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  4. Explain that the risks of hypertensive disorders in subsequent pregnancies as well as future chronic hypertension, coronary artery disease, stroke, heart failure, chronic kidney disease, type 2 diabetes, and metabolic syndrome are increased.
  5. Create a long-term follow-up plan: BP control, weight management, smoking cessation, lipid profile, glucose metabolism and renal function assessment.
  6. Note that the risk of VTE is highest in the first 6 weeks after birth; Individualize thromboprophylaxis according to risk stratification.

Treatment

  • In severe postpartum hypertension (≥160/110 mmHg), begin without delay: immediate-release oral nifedipine 10–20 mg, IV labetalol 20 mg (over 2 minutes), or IV/IM hydralazine 5 mg; For repeat intervals and upper dose limits, apply 'Acute severe hypertension › Postpartum' in the algorithm.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
There was no hypertension in pregnancy (hypertension or preeclampsia suspected for the first time after birth)

Approach

  1. Confirm new hypertension; inquire about symptoms and evaluate with complete blood count, platelets, creatinine, AST/ALT, bilirubin and quantitative urine protein; Note that preeclampsia can also develop with organ dysfunction without proteinuria.
  2. If there are serious findings, evaluate and monitor in hospital.
  3. If systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg, confirm by measuring again in 15 minutes; if persistent, initiate rapid-acting antihypertensive as soon as possible, no later than 30–60 minutes; If the BP threshold continues to be exceeded despite consecutive appropriate doses of the selected first-line agent, switch to another first-line agent and seek urgent consultation with a maternal-fetal medicine, anesthesiologist, or intensivist.
  4. Describe in detail any warning symptoms before discharge, such as headache, visual disturbance, epigastric or right upper quadrant pain, shortness of breath, chest pain, and sudden worsening of edema.
  5. Consider cardiology evaluation and ECHO if postpartum hypertension persists or evidence of shortness of breath, chest pain, orthopnea, or pulmonary edema occurs.
  6. If hypertension persists for more than 12 weeks, reassess for chronic hypertension; Include BP control, weight management, smoking cessation, lipid profile, glucose metabolism and kidney function in the long-term follow-up plan.
  7. Note that the risk of VTE is highest in the first 6 weeks after birth; Individualize thromboprophylaxis according to risk stratification.

Treatment

  • Rapid-acting antihypertensive (IV labetalol, IV hydralazine, or immediate-release oral nifedipine): administer only if systolic BP ≥160 mmHg and/or diastolic BP ≥110 mmHg confirmed by repeat measurement within 15 minutes; choose one according to contraindications and clinical picture.
  • IV labetalol: 20 mg IV over 2 minutes; If BP is above threshold, 40 mg after 10 minutes, then 80 mg every 10 minutes (each dose over 2 minutes); maximum 300 mg total; alternatively, 1–2 mg/min IV infusion.
  • Hydralazine: 5 mg IV or IM, followed by 5–10 mg IV every 20–40 minutes if necessary; maximum 20 mg total.
  • Immediate-release oral nifedipine: 10–20 mg; Repeat after 20 minutes if necessary; then 10–20 mg every 2–6 hours if necessary; maximum 180 mg per day.
  • If BP remains above threshold despite consecutive doses of one agent, switch to the other: IV labetalol after immediate-release nifedipine or hydralazine, IV hydralazine after IV labetalol; If there is no response, seek specialist support for second-line treatment.
  • Magnesium sulfate in severe preeclampsia: Give a 4 g IV loading dose over 5–15 minutes, followed by a 1 g/hour IV maintenance infusion for 24 hours.
  • Magnesium sulfate in renal dysfunction: if creatinine is 1.0–1.5 mg/dL or urine output is <30 mL/hour for more than 4 hours, keep the maintenance infusion after the loading dose at no more than 1 g/hour and measure serum magnesium every 4 hours; if the level exceeds 9.6 mg/dL (8 mEq/L), stop the infusion, measure every 2 hours, and restart at a lower rate when the level falls below 8.4 mg/dL (7 mEq/L); If creatinine clearance is <30 mL/min, apply intermittent doses according to serum level instead of continuous maintenance infusion and give a total of maximum 20 g in 48 hours.
  • In case of magnesium toxicity (loss of reflexes, respiratory depression, PR/QRS prolongation, AV block, bradycardia or hypotension), stop the infusion immediately, support breathing (intubation if necessary) and give 10% calcium gluconate 10 mL (1 g) IV over 3 minutes.
  • In uncomplicated postpartum hypertension — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing every 7–14 days; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • In uncomplicated postpartum hypertension — Labetalol (oral): Start at 100 mg twice daily, increasing by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If labetalol is not available in uncomplicated postpartum hypertension — Metoprolol (oral): start with immediate-release tartrate 50 mg twice daily, gradually increasing according to response; The usual dose during pregnancy is 100 mg twice a day; maximum 200 mg daily, divided into 2 doses; Long-acting succinate is started at 25–100 mg once a day, up to 200 mg once a day.

Note

  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Note that high or frequent doses of hydralazine increase the risk of maternal hypotension.
  • Do not puncture or sublingual administration of immediate-release nifedipine capsule.
  • Choose postpartum antihypertensive based on clinical presentation and compatibility with breastfeeding; nifedipine, labetalol and metoprolol are compatible with breastfeeding.
  • Magnesium sulfate: monitor patellar reflex, respiratory rate, SpO2, and hourly urine output; In case of reflex loss or respiratory depression, stop the infusion and measure serum magnesium. Magnesium is not appropriate in myasthenia gravis, hypocalcemia, moderate to severe renal failure, cardiac ischemia, myocarditis, or heart block (except for first-degree block or the presence of a functioning pacemaker); Select alternative agent with specialist for seizure prevention/treatment.
  • Individualize the threshold for starting oral therapy and the BP target in non-severe postpartum hypertension based on repeated measurements and the clinical picture.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Metoprolol (oral): Not used in second-third degree AV block and sick sinus syndrome (if there is no functional pacemaker), sinus bradycardia, cardiogenic shock and overt or decompensated heart failure; If the treatment is to be discontinued, the dose should be reduced over 1-2 weeks. Since metabolism decreases after birth, the need is re-evaluated.
Hypertension lasting more than 12 weeks

Approach

  1. Re-evaluate for chronic hypertension and ensure long-term BP control.
  2. Create a long-term follow-up plan: BP control, weight management, smoking cessation, lipid profile, glucose metabolism and renal function evaluation; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  3. Explain that the risks of hypertensive disorders in subsequent pregnancies as well as future chronic hypertension, coronary artery disease, stroke, heart failure, chronic kidney disease, type 2 diabetes, and metabolic syndrome are increased.
  4. Plan counseling, BP and metabolic risk assessment before next pregnancy; Explain that there is an increased risk of preeclampsia during pregnancy.

Treatment

  • If hypertension is persistent, maintain antihypertensive therapy and titrate according to the adult hypertension approach.
  • Recommend weight management, smoking cessation and lifestyle modification.
  • If pregnancy is planned, first option — Labetalol (oral): Start at 100 mg twice daily, increase by 100 mg (twice daily) every 2–3 days; if necessary, given 3 times a day; maximum 2400 mg per day.
  • If pregnancy is planned, first option — Nifedipine (long-acting, oral): Start at 30–60 mg once daily, increasing at 7–14 day intervals; The usual dose is 30–90 mg once a day, maximum 120 mg daily.
  • If pregnancy is planned, alternative — Methyldopa (oral): Start at 250 mg 2–3 times daily, increasing every 2 days at the earliest; up to 3000 mg daily in divided doses.

Note

  • If breastfeeding, choose agent compatible with breastfeeding (nifedipine, labetalol, or metoprolol).
  • If pregnancy is planned, discontinue ACE inhibitor and angiotensin receptor blocker before pregnancy.
  • Do not use labetalol in asthma, pre-existing myocardial disease, decompensated heart function, heart block, or bradycardia.
  • Labetalol (oral): Avoid in asthma, chronic obstructive pulmonary disease, heart failure, bradycardia (<60/min), or advanced first-degree heart block.
  • Nifedipine (long-acting, oral): Not given sublingually; The tablet is swallowed whole, not broken, split or chewed; Some patients tolerate better dividing the daily dose into 2 separate applications.
  • Methyldopa (oral): It can be stopped within 2 days after birth and switched to another medication.
Long term (after 12 weeks, BP normal)

Approach

  1. Create a long-term follow-up plan: BP control, weight management, smoking cessation, lipid profile, glucose metabolism and renal function evaluation; In cases of early-onset, severe, recurrent preeclampsia or complicated by fetal growth restriction, long-term cardiovascular monitoring should be especially stringent.
  2. Explain that the risks of hypertensive disorders in subsequent pregnancies as well as future chronic hypertension, coronary artery disease, stroke, heart failure, chronic kidney disease, type 2 diabetes, and metabolic syndrome are increased.
  3. Plan preconception counseling, BP and metabolic risk assessment, and close monitoring from early pregnancy for subsequent pregnancy; Plan low-dose aspirin prophylaxis in suitable candidates.

Treatment

  • Recommend weight management, smoking cessation and lifestyle modification.
Preeclampsia prevention (high-risk women)
Risk situation
Preeclampsia in previous pregnancy

Approach

  1. Provide pre-pregnancy counseling and assess BP and metabolic risk; Explain that the risk of hypertensive disorders in subsequent pregnancy increases significantly, especially after early onset or severe preeclampsia; Plan close monitoring from early pregnancy.
  2. Consider concomitant risk factors: chronic hypertension, pregestational diabetes, chronic kidney disease, multiple pregnancy, antiphospholipid syndrome, SLE, obesity, advanced maternal age, assisted reproduction.
  3. Measure BP with correct technique and inquire about symptoms at each antenatal visit; The absence of risk factors does not exclude preeclampsia.

Treatment

  • Aspirin 75–150 mg/day at bedtime: if there is at least one high-risk factor or two moderate-risk factors for preeclampsia, give from week 12 until week 36/37; discontinue at week 36/37 if preeclampsia prevention is the sole indication.

Note

  • Do not give low-dose aspirin in asthma with a history of allergy to aspirin or other salicylates, NSAID hypersensitivity, nasal polyps, or aspirin-induced bronchospasm. Individualize the decision in cases of history of gastrointestinal bleeding, active peptic ulcer, other foci of gastrointestinal or genitourinary bleeding, or severe liver dysfunction.
Chronic hypertension, pregestational diabetes, chronic kidney disease or multiple pregnancy

Approach

  1. Consider these conditions as important preeclampsia risk factors, even if there is no previous history of preeclampsia; Measure BP with the correct technique and inquire about symptoms at each antenatal visit.

Treatment

  • Aspirin 75–150 mg/day at bedtime: if there is at least one high-risk factor or two moderate-risk factors for preeclampsia, give from week 12 until week 36/37; discontinue at week 36/37 if preeclampsia prevention is the sole indication.

Note

  • Do not give low-dose aspirin in asthma with a history of allergy to aspirin or other salicylates, NSAID hypersensitivity, nasal polyps, or aspirin-induced bronchospasm. Individualize the decision in cases of history of gastrointestinal bleeding, active peptic ulcer, other foci of gastrointestinal or genitourinary bleeding, or severe liver dysfunction.
  • Preeclampsia risk class of multiple pregnancy can be evaluated differently; Individualize the aspirin decision with accompanying risk factors.
Systemic lupus erythematosus (SLE)

Approach

  1. Plan pregnancy when the disease is clinically inactive for at least 6 months.
  2. Be careful to distinguish lupus flare from pregnancy physiology and other pregnancy complications.
  3. Measure BP with correct technique and inquire about symptoms at each antenatal visit; The absence of risk factors does not exclude preeclampsia.

Treatment

  • Low-dose aspirin: 81–162 mg/day; Start at week 12, continue until week 36; administer regardless of antiphospholipid antibody status.
  • A history of APS-associated arterial or venous thrombosis (thrombotic APS) requires therapeutic doses of LMWH throughout pregnancy in addition to low-dose aspirin; Apply the antiphospholipid syndrome selection of the thrombosis group in the algorithm for dose and discontinuation and reinitiation around delivery; Individually consider continuing aspirin after week 36.

Note

  • Do not give low-dose aspirin in asthma with a history of allergy to aspirin or other salicylates, NSAID hypersensitivity, nasal polyps, or aspirin-induced bronchospasm. Individualize the decision in cases of history of gastrointestinal bleeding, active peptic ulcer, other foci of gastrointestinal or genitourinary bleeding, or severe liver dysfunction.
Antiphospholipid antibody positivity (no history of thrombosis)
APS status
No clinical criteria for APS (aPL positivity only)

Approach

  1. Assess the clinical criteria for obstetric APS: at least one unexplained fetal death in a morphologically normal fetus at or after 10+0 weeks; at least one preterm birth before the 34th week due to severe preeclampsia or placental insufficiency; or at least 3 consecutive unexplained spontaneous miscarriages before the 10th week. Laboratory criterion: lupus anticoagulant, medium-high-titer anticardiolipin IgG/IgM (>40 GPL/MPL or >99th percentile), or anti-β2 glycoprotein I IgG/IgM >99th percentile, positive on two occasions at least 12 weeks apart.
  2. Consider lupus anticoagulant positivity and triple aPL positivity as indicators of high risk for adverse pregnancy outcome.
  3. Measure BP with correct technique and inquire about symptoms at each antenatal visit; The absence of risk factors does not exclude preeclampsia.

Treatment

  • Low-dose aspirin: 81–162 mg/day; Start at week 12 and continue until week 36.

Note

  • Do not give low-dose aspirin in asthma with a history of allergy to aspirin or other salicylates, NSAID hypersensitivity, nasal polyps, or aspirin-induced bronchospasm. Individualize the decision in cases of history of gastrointestinal bleeding, active peptic ulcer, other foci of gastrointestinal or genitourinary bleeding, or severe liver dysfunction.
Obstetric APS

Approach

  1. Consider lupus anticoagulant positivity and triple aPL positivity as indicators of high risk for adverse pregnancy outcome.
  2. Measure BP with correct technique and inquire about symptoms at each antenatal visit; The absence of risk factors does not exclude preeclampsia.
  3. Discontinue LMWH before planned delivery.

Treatment

  • Low-dose aspirin: 81–162 mg/day; usually start before pregnancy.
  • When intrauterine pregnancy is confirmed — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • Postpartum LMWH: restart 4–6 hours after vaginal delivery, 6–12 hours after cesarean section if there is no significant bleeding and hemostasis is achieved; if neuraxial intervention was performed, start at least 12 hours after the procedure and at least 4 hours after epidural catheter removal; Use whichever of these intervals ends latest; It usually lasts 6 weeks.

Note

  • Do not perform neuraxial intervention until at least 12 hours after the last prophylactic dose of LMWH.
  • Do not give low-dose aspirin in asthma with a history of allergy to aspirin or other salicylates, NSAID hypersensitivity, nasal polyps, or aspirin-induced bronchospasm. Individualize the decision in cases of history of gastrointestinal bleeding, active peptic ulcer, other foci of gastrointestinal or genitourinary bleeding, or severe liver dysfunction.
  • For prophylaxis above 40 mg/day (intermediate dose), evidence is insufficient to define the waiting interval before neuraxial procedures (12–24 hours). Plan timing with the anesthesia team; do not automatically apply the 12-hour rule.
  • Aspirin (and other NSAIDs) along with prophylactic LMWH while the epidural catheter is in place may increase the risk of bleeding; Plan catheter removal and resumption of medications with the anesthesia team: remove the catheter at least 12 hours after the last LMWH dose, give the next LMWH dose at least 4 hours after removal.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.

Thromboembolic conditions

VTE thromboprophylaxis during pregnancy
VTE risk status
No additional risk factors other than pregnancy

Approach

  1. Consider pregnancy alone as a VTE risk factor; Re-evaluate the risk throughout pregnancy and after birth, and individualize the prophylaxis decision according to risk classification.
  2. Question antepartum risk factors throughout pregnancy: multiple pregnancy, varicose veins, assisted reproduction, inflammatory bowel disease, urinary tract infection, diabetes, hospitalization for more than 3 days due to non-partum reasons, BMI >30 kg/m², maternal age >35, hyperemesis gravidarum, smoking.
  3. Inquire about general risk factors: hereditary thrombophilia, antiphospholipid syndrome, previous VTE.
  4. After birth, reassess postpartum risk factors: cesarean section, medical comorbidity, preterm birth, stillbirth, hypertension, smoking, preeclampsia or eclampsia, postpartum infection, transfusion.
  5. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Routine (universal) thromboprophylaxis is not required.
There is an additional risk factor
Period
Antepartum

Approach

  1. Identify those most likely to benefit from prophylaxis: hereditary or acquired thrombophilia, previous VTE, obesity, long-term immobilization, hypertensive disorder, autoimmune disease, assisted reproductive pregnancy.
  2. Individualize thromboprophylaxis according to risk classification, not universally; Reassess VTE risk throughout pregnancy and after birth.
  3. Request hereditary thrombophilia testing if there is a personal history of VTE, if it has not been performed previously, and if the result would change antepartum or postpartum management.
  4. Determine the interval for stopping anticoagulation before delivery according to thrombotic risk, anticoagulant type and dose, and whether delivery will be induced or by cesarean section.
  5. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.
  6. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery in the last weeks of pregnancy, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (if aPTT is prolonged, the dose is reduced).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; Heparins do not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Limit the use of fondaparinux in pregnancy to severe heparin reactions such as HIT.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Postpartum

Approach

  1. Evaluate postpartum risk factors: cesarean section, medical comorbidity, preterm birth, stillbirth, hypertension, smoking, preeclampsia or eclampsia, postpartum infection, transfusion.
  2. Note that the risk of VTE is highest in the first 6 weeks after birth; Reassess risk after birth and individualize prophylaxis according to risk classification.
  3. Request hereditary thrombophilia testing if there is a personal history of VTE, if it has not been performed previously, and if the result would change antepartum or postpartum management.

Treatment

  • Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (SC, prophylactic): 5000 IU SC every 8–12 hours after birth.
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, initiate or restart anticoagulant 4–6 hours after vaginal delivery and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women receiving antepartum prophylaxis with prior VTE, high-risk thrombophilia, or thrombophilia together with a family history, continue prophylaxis for 6 weeks postpartum; For prophylaxis initiated solely because of postpartum risk factors, apply for at least 10 days and extend it to up to 6 weeks if the risk factor persists for more than 10 days.

Note

  • Avoid estrogen-containing combined hormonal contraception in the event of previous thromboembolism or high-risk prothrombotic status; Progestin-only methods are safer from a thrombotic perspective.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Deep vein thrombosis (DVT)
Diagnostic phase
Suspicion – low or intermediate clinical probability

Approach

  1. Assess for unilateral leg pain and swelling, erythema, warmth, and tenderness; right-sided or bilateral symptoms do not exclude the diagnosis.
  2. Request D-dimer: no validated pregnancy-specific threshold, use threshold of 500 ng/mL; with low–moderate clinical probability, negative D-dimer may exclude DVT and obviate the need for CUS; In this case, continue clinical monitoring and re-evaluate immediately if symptoms progress.
  3. Note that the D-dimer strategy is particularly useful in the 1st and 2nd trimesters and in the setting where immediate access to CUS is limited, and its benefit diminishes as pregnancy progresses.
  4. If D-dimer is not negative, perform CUS; If possible, evaluate the entire leg and iliac veins, examine in the left lateral decubitus position in advanced pregnancy; If CUS is positive, the diagnosis has been made.
  5. Generally do not perform additional evaluation if negative CUS is consistent with low–intermediate clinical probability; If moderate suspicion persists or examination is limited to proximal veins only, consider serial CUS with a low threshold.
  6. Note that pelvic vein thrombosis may be missed on compression ultrasonography (CUS).
  7. Maintain clinical monitoring throughout pregnancy.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; If CUS is positive, start immediately; if diagnostic imaging will be delayed, give the first dose until VTE is excluded.
  • If creatinine clearance <30 mL/min under the same baseline condition, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • In the same starting condition, if there is a history of HIT or active HIT, at the treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.

Note

  • Do not use VKA (warfarin) and DOAC (dabigatran, rivaroxaban, apixaban, edoxaban) during pregnancy.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Suspicion – high clinical probability

Approach

  1. Assess for unilateral leg pain and swelling, erythema, warmth, and tenderness; right-sided or bilateral symptoms do not exclude the diagnosis.
  2. Perform proximal vein compression ultrasonography (CUS).
  3. D-dimer exclusion strategy is supported only in low–moderate clinical probability; In this case, do not base the diagnosis on D-dimer.
  4. Note that pelvic vein thrombosis may be missed on compression ultrasonography (CUS).
  5. Diagnosis can also be made by decreased flow on femoral–iliac vein Doppler or filling defect on MR venography; ascending contrast or CT venography is rarely used.
  6. Maintain clinical monitoring throughout pregnancy.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; If DVT is detected on CUS or other imaging, start immediately; if diagnostic imaging will be delayed, give the first dose until VTE is excluded.
  • If creatinine clearance <30 mL/min under the same baseline condition, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • In the same starting condition, if there is a history of HIT or active HIT, at the treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.

Note

  • Do not use VKA (warfarin) and DOAC (dabigatran, rivaroxaban, apixaban, edoxaban) during pregnancy.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
CUS negative
Clinical evaluation
Consistent with low–intermediate clinical probability; There is no suspicion and the examination is not limited to the proximal veins.

Approach

  1. In cases of negative CUS compatible with clinical evaluation, additional evaluation is generally not performed unless suspicion persists and the examination is not limited to proximal veins only.
  2. Maintain clinical monitoring throughout pregnancy.
  3. Note that pelvic vein thrombosis may be missed on compression ultrasonography (CUS).

Treatment

  • No specific drug treatment is required.
Clinical suspicion persists or examination is limited to proximal veins only

Approach

  1. If clinical suspicion persists, repeat CUS on days 3 and 7.
  2. Note that pelvic vein thrombosis may be missed on compression ultrasonography (CUS).
  3. If iliac vein thrombosis is suspected (swelling in the whole leg, back or hip pain), perform Doppler ultrasonography of the iliac vein; If the result does not support thrombosis, perform MR venography.
  4. Maintain clinical monitoring throughout pregnancy.

Treatment

  • Therapeutic dose LMWH (enoxaparin): start immediately if DVT is detected on repeat imaging; In cases of suspected iliac vein thrombosis, start while waiting for the results; 1 mg/kg SC every 12 hours based on early pregnancy weight.
  • If creatinine clearance <30 mL/min under the same baseline condition, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • In the same starting condition, if there is a history of HIT or active HIT, at the treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.

Note

  • Do not use VKA (warfarin) and DOAC (dabigatran, rivaroxaban, apixaban, edoxaban) during pregnancy.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
DVT confirmed (CUS or other appropriate venous imaging)

Approach

  1. Consider thrombosis detected on positive compression ultrasonography (CUS) or other appropriate venous imaging as the diagnosis and initiate treatment immediately.
  2. In cases of suspected concomitant PE, positive proximal CUS generally eliminates the need for confirmatory chest imaging.
  3. Do not screen for hereditary thrombophilia at the time of acute VTE diagnosis; acute thrombosis can lower antithrombin and protein C levels, and pregnancy can lower protein S levels, leading to misleading results.
  4. Determine the interval for stopping anticoagulation before delivery according to thrombotic risk, anticoagulant type and dose, and whether delivery will be induced or by cesarean section.
  5. Wait at least 24 hours after the last therapeutic dose of LMWH until planned delivery or neuraxial intervention.
  6. If UFH is used, perform neuraxial intervention after aPTT returns to normal; may last approximately 6 hours after therapeutic IV infusion and up to 24 hours after subcutaneous administration.
  7. If subcutaneous UFH 5,000 units is used two or three times daily, wait at least 4–6 hours after the last dose for neuraxial intervention or evaluate coagulation; If 7,500–10,000 units are used twice daily, wait at least 12 hours and assess coagulation; If >10,000 units per dose or >20,000 units per day, wait at least 24 hours and verify aPTT is normal.
  8. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; Anti-Xa monitoring is rarely required; if done, measure peak anti-Xa level 4–6 hours after dose, target approximately 0.6–1.2 U/mL.
  • If creatinine clearance is <30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery in the last weeks of pregnancy, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If there is a history of HIT or active HIT in thrombosis requiring treatment, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • Maintain therapeutic anticoagulation throughout pregnancy and for at least 6 weeks postpartum, for a total treatment duration of at least 3 months; If there is a permanent indication, continue longer.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; Heparins do not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Limit the use of fondaparinux in pregnancy to severe heparin reactions such as HIT.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Suspicion of pulmonary embolism (PE)
Diagnostic phase
Hemodynamic instability (shock or permanent hypotension)

Approach

  1. Immediately call the experienced multidisciplinary team (senior obstetrician, cardiology, anesthesia, radiology) and begin maternal resuscitation.
  2. Evaluate with bedside ECHO and, if possible, CT pulmonary angiography (CTPA) within 1 hour; If CTPA cannot be performed, use right ventricular dysfunction on ECHO in the decision on reperfusion.
  3. Perform cardiopulmonary resuscitation (CPR) with left lateral tilt in cardiac arrest; If resuscitation is unsuccessful in pregnancy beyond the 20th week, perform perimortem cesarean section within 5 minutes.

Treatment

  • IV UFH as initial anticoagulant — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/hour infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • Systemic thrombolysis by multidisciplinary decision if high-risk PE is confirmed or is too severe to await confirmation — Alteplase: 100 mg IV, infused over 2 hours; In extreme hemodynamic instability such as cardiac arrest, accelerated regimen is 0.6 mg/kg IV over 15 minutes (maximum 50 mg); At the end of the infusion, when aPTT drops to 2 times normal or below, UFH is started or continued without giving a loading dose.
  • Catheter reperfusion can be applied instead of systemic thrombolysis; If thrombolysis is not appropriate or unsuccessful, perform surgical embolectomy.

Note

  • Apply thrombolytic or interventional therapy in the peripartum period only in high-risk PE with multidisciplinary decision.
  • Do not use VKA (warfarin) and DOAC during pregnancy.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Alteplase: Pregnancy and the first week postpartum are relative contraindications; It is only given with a multidisciplinary decision in life-threatening PE in the peripartum period.
Initial evaluation (prior to imaging)

Approach

  1. Consider sudden onset of dyspnea or chest pain in favor of acute PE; Slowly developing shortness of breath suggests physiological dyspnea of ​​pregnancy, but the subacute picture can also be seen in chronic VTE or acute embolism in a woman with pre-existing cardiopulmonary disease.
  2. Obtain a chest X-ray for every pregnant woman with suspected PE; indicates alternative diagnoses (pneumonia, pneumothorax, pulmonary edema, cardiomegaly) and guides subsequent imaging.
  3. Do not routinely order arterial blood gases; they are neither sensitive nor specific, and normal PaO₂, PaCO₂ and alveolar–arterial gradient do not exclude PE.
  4. Positive proximal compression ultrasonography (CUS) establishes the diagnosis of DVT and usually eliminates the need for confirmatory chest imaging; CUS alone does not diagnose PE.
  5. Perform ECHO to distinguish heart failure, including PPCM, from PE in slowly progressive dyspnea and orthopnea.
  6. If underlying lung disease (e.g. lymphangioleiomyomatosis) is suspected, differentiate with chest CT.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; If DVT is detected in the proximal CUS, start immediately; if diagnostic imaging will be delayed, give the first dose until VTE is excluded.
  • If creatinine clearance <30 mL/min under the same baseline condition, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • In the same starting condition, if there is a history of HIT or active HIT, at the treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.

Note

  • Do not use VKA (warfarin) and DOAC (dabigatran, rivaroxaban, apixaban, edoxaban) during pregnancy; Use heparin, which does not cross the placenta, as an anticoagulant.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Chest radiography is normal

Approach

  1. Perform ventilation/perfusion (V/Q) scintigraphy or CT pulmonary angiography (CTPA); In normal radiography, the negative predictive value of V/Q scintigraphy is high and ambiguous results are less common.
  2. Use CTPA as primary imaging modality; It also detects an alternative diagnosis in 12–13% of cases.
  3. Accept normal perfusion on V/Q scintigraphy as a finding that excludes PE, and a high-probability result as confirming PE; consider a very-low-probability result exclusionary only when combined with low clinical probability; low- and intermediate-probability results are nondiagnostic.
  4. Positive proximal compression ultrasonography (CUS) establishes the diagnosis of DVT and usually eliminates the need for confirmatory chest imaging; CUS alone does not diagnose PE.
  5. Do not routinely use MR pulmonary angiography or conventional contrast-enhanced pulmonary angiography; they have not been adequately validated in pregnancy.
  6. Do not routinely add echocardiography to the assessment of suspected PE; an intracardiac or pulmonary artery thrombus is rarely visualized.
  7. Do not routinely order arterial blood gases; they are neither sensitive nor specific, and normal PaO₂, PaCO₂ and alveolar–arterial gradient do not exclude PE.
  8. If V/Q or CTPA is normal or nondiagnostic and clinical suspicion persists, perform alternative or repeat imaging.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; If DVT is detected in the proximal CUS, start immediately; if diagnostic imaging will be delayed, give the first dose until VTE is excluded; Continue the initiated anticoagulation until PE is definitively excluded.
  • If creatinine clearance <30 mL/min under the same baseline condition, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • In the same starting condition, if there is a history of HIT or active HIT, at the treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.

Note

  • Do not use VKA (warfarin) and DOAC (dabigatran, rivaroxaban, apixaban, edoxaban) during pregnancy; Use heparin, which does not cross the placenta, as an anticoagulant.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Chest radiograph abnormal

Approach

  1. Consider alternative diagnoses on the radiograph, such as pneumonia, pneumothorax, pulmonary edema, or cardiomegaly.
  2. If suspicion of PE persists, perform CT pulmonary angiography; Prefer CTPA to V/Q scintigraphy in abnormal chest radiographs.
  3. Note that CT pulmonary angiography (CTPA) also detects an alternative diagnosis in 12–13% of cases.
  4. Perform ECHO to distinguish heart failure, including PPCM, from PE in slowly progressive dyspnea and orthopnea.
  5. If underlying lung disease (e.g. lymphangioleiomyomatosis) is suspected, differentiate with chest CT.
  6. Positive proximal compression ultrasonography (CUS) establishes the diagnosis of DVT and usually eliminates the need for confirmatory chest imaging; CUS alone does not diagnose PE.
  7. Do not routinely order arterial blood gases; they are neither sensitive nor specific, and normal PaO₂, PaCO₂ and alveolar–arterial gradient do not exclude PE.
  8. If V/Q or CTPA is normal or nondiagnostic and clinical suspicion persists, perform alternative or repeat imaging.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; If DVT is detected in the proximal CUS, start immediately; if diagnostic imaging will be delayed, give the first dose until VTE is excluded; Continue the initiated anticoagulation until PE is definitively excluded.
  • If creatinine clearance <30 mL/min under the same baseline condition, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after challenge, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • In the same starting condition, if there is a history of HIT or active HIT, at the treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.

Note

  • Do not use VKA (warfarin) and DOAC (dabigatran, rivaroxaban, apixaban, edoxaban) during pregnancy; Use heparin, which does not cross the placenta, as an anticoagulant.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
PE confirmed (CTPA or diagnostic V/Q)

Approach

  1. Consider PE confirmed by CT pulmonary angiography (CTPA) or diagnostic ventilation/perfusion (V/Q) examination as VTE requiring treatment.
  2. Do not screen for hereditary thrombophilia at the time of acute VTE diagnosis; acute thrombosis can lower antithrombin and protein C levels, and pregnancy can lower protein S levels, leading to misleading results.
  3. Determine the interval for stopping anticoagulation before delivery according to thrombotic risk, anticoagulant type and dose, and whether delivery will be induced or by cesarean section.
  4. Wait at least 24 hours after the last therapeutic dose of LMWH until planned delivery or neuraxial intervention.
  5. If UFH is used, perform neuraxial intervention after aPTT returns to normal; may last approximately 6 hours after therapeutic IV infusion and up to 24 hours after subcutaneous administration.
  6. If subcutaneous UFH 5,000 units is used two or three times daily, wait at least 4–6 hours after the last dose for neuraxial intervention or evaluate coagulation; If 7,500–10,000 units are used twice daily, wait at least 12 hours and assess coagulation; If >10,000 units per dose or >20,000 units per day, wait at least 24 hours and verify aPTT is normal.
  7. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; Anti-Xa monitoring is rarely required; if done, measure peak anti-Xa level 4–6 hours after dose, target approximately 0.6–1.2 U/mL.
  • If creatinine clearance is <30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery in the last weeks of pregnancy, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If there is a history of HIT or active HIT in thrombosis requiring treatment, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • Maintain therapeutic anticoagulation throughout pregnancy and for at least 6 weeks postpartum, for a total treatment duration of at least 3 months; If there is a permanent indication, continue longer.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; Heparins do not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Limit the use of fondaparinux in pregnancy to severe heparin reactions such as HIT.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Hereditary thrombophilia
Clinical situation
Test indication under evaluation

Approach

  1. Do not perform routine hereditary thrombophilia screening in unselected pregnant women.
  2. Order the test only if its result would change antepartum or postpartum management.
  3. Consider a woman with a personal history of VTE a candidate for testing, especially if she has not been tested before.
  4. Consider a woman with a first-degree relative known to have high-risk hereditary thrombophilia as a candidate for testing.
  5. Do not perform routine thrombophilia screening after stillbirth; evaluate for antiphospholipid syndrome.
  6. Do not order homocysteine, MTHFR polymorphisms (C677T, A1298C), PAI-1 polymorphism or factor VIII levels as part of a thrombophilia assessment.

Treatment

  • Testing decision alone does not require specific drug treatment.

Note

  • Interpret protein C, protein S, and antithrombin results while taking into account the effects of acute thrombosis and pregnancy; do not diagnose hereditary deficiency based on a low protein S result during pregnancy.
High risk: antithrombin deficiency, homozygous FVL or prothrombin G20210A, combined heterozygosity

Approach

  1. Classify as hereditary thrombophilia with high VTE risk; The risk is significantly higher than for heterozygous factor V Leiden (FVL) or prothrombin mutation and protein C/S deficiency.
  2. Inquire about personal or first-degree relative history of VTE; significantly increases the risk.
  3. Reassess VTE risk throughout pregnancy and postpartum; Thrombophilia is in the group that benefits most from prophylaxis, individualize prophylaxis according to risk classification.
  4. Association with placenta-related complications is not consistent; If present, it is limited and largely seen in high-risk thrombophilias.
  5. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.
  6. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If antithrombin deficiency and previous VTE are present, individualize LMWH under hematology supervision at an increased dose, 50%, 75%, or all of the therapeutic dose; continue throughout pregnancy and for 6 weeks postpartum or until return to long-term anticoagulation.
  • If creatinine clearance is <30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery in the last weeks of pregnancy, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (if aPTT is prolonged, the dose is reduced).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; Heparins do not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Low risk: heterozygous FVL, heterozygous prothrombin G20210A, protein C or S deficiency

Approach

  1. Classify as low VTE risk hereditary thrombophilia.
  2. Inquire about personal or first-degree relative history of VTE; significantly increases the risk.
  3. Do not give routine antepartum prophylaxis if there is no personal history of VTE; Determine postpartum prophylaxis based on VTE history in a first-degree relative and additional risk factors.
  4. Reassess VTE risk throughout pregnancy and postpartum; Thrombophilia is in the group that benefits most from prophylaxis, individualize prophylaxis according to risk classification.
  5. No consistent association with placenta-related complications has been demonstrated; The limited effect reported is largely confined to high-risk types.
  6. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.
  7. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • If there is a personal history of VTE, throughout pregnancy and 6 weeks postpartum — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min or if rapid reversal may be required for labor, neuraxial anesthesia, or emergency surgery in the last weeks of pregnancy, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (if aPTT is prolonged, the dose is reduced).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; Heparins do not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Antiphospholipid syndrome (APS)
APS type
Thrombotic APS (history of venous or arterial thrombosis)

Approach

  1. Confirm the diagnosis with persistent antiphospholipid antibody (aPL) positivity on two tests at least 12 weeks apart: positive lupus anticoagulant (LA), or moderate-to-high-titer (>40 GPL/MPL or >99th percentile) IgG/IgM anticardiolipin, or IgG/IgM anti-β2 glycoprotein I above the 99th percentile; do not count isolated low-titer positivity alone as an APS laboratory criterion.
  2. Consider LA positivity as a major predictor of poor pregnancy outcome and triple aPL positivity as a predictor of worse obstetric outcome.
  3. Do not routinely use biomarkers such as low C4; validation is required.
  4. Determine the interval for stopping anticoagulation before delivery according to thrombotic risk, anticoagulant type and dose, and whether delivery will be induced or by cesarean section.
  5. Wait at least 24 hours after the last therapeutic dose of LMWH until planned delivery or neuraxial intervention.
  6. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.
  7. Plan postpartum anticoagulation duration according to persistent thrombotic indication.

Treatment

  • Therapeutic dose LMWH (enoxaparin; in pregnancy, instead of pre-pregnancy chronic anticoagulant): 1 mg/kg SC every 12 hours based on early pregnancy weight; Anti-Xa monitoring is rarely required; if done, measure peak anti-Xa level 4–6 hours after dose, target approximately 0.6–1.2 U/mL.
  • Low-dose aspirin (81–162 mg/day): continue or initiate; Consider continuation after week 36 in patients with a history of APS-related thrombotic events such as stroke or myocardial infarction.
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If baseline aPTT is prolonged (e.g., lupus anticoagulant), adjust UFH dose with anti-Xa instead of aPTT; target anti-Xa 0.3–0.7 IU/mL.
  • If there is a history of HIT or active HIT in therapeutic anticoagulation, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • If the indication for chronic anticoagulation before pregnancy continues, do not discontinue the treatment just because the 6 weeks after birth are completed.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Obstetric APS (no history of thrombosis)

Approach

  1. Verify at least one of the following obstetric criteria after other causes have been excluded: at least three consecutive unexplained pregnancy losses before the 10th week; At least one unexplained death in a morphologically normal fetus at 10+0 weeks or later; birth before the 34th week due to severe preeclampsia, eclampsia, or placental insufficiency.
  2. Confirm the laboratory criterion on two tests at least 12 weeks apart: positive lupus anticoagulant (LA), or moderate-to-high-titer (>40 GPL/MPL or >99th percentile) IgG/IgM anticardiolipin, or IgG/IgM anti-β2 glycoprotein I above the 99th percentile; do not count isolated low-titer positivity alone as an APS laboratory criterion.
  3. Consider LA positivity as a major predictor of poor pregnancy outcome and triple aPL positivity as a predictor of worse obstetric outcome.
  4. Do not routinely use biomarkers such as low C4; validation is required.
  5. Wait at least 12 hours after the last prophylactic dose of LMWH until planned delivery or neuraxial intervention.
  6. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Low-dose aspirin (81–162 mg/day): usually start before pregnancy.
  • Add when intrauterine pregnancy is confirmed — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • Continue LMWH for 6 weeks postpartum in women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Systemic lupus erythematosus (SLE)
aPL / APS status
Antiphospholipid antibody (aPL) negative

Approach

  1. Plan pregnancy when the disease is clinically inactive for at least 6 months.
  2. Note that it is difficult to distinguish a lupus flare from the physiological changes of pregnancy and other pregnancy complications.
  3. State in counseling that there is an increased risk of maternal VTE, hypertensive disorder, preterm birth, unplanned cesarean section, postpartum hemorrhage, acute renal failure, fetal loss, and fetal growth restriction (FGR).
  4. Autoimmune disease is in the group that benefits most from thromboprophylaxis; Evaluate VTE risk individually throughout pregnancy and after birth.
  5. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.

Treatment

  • Low-dose aspirin (81–162 mg/day): start at week 12 and continue until week 36 to reduce the risk of preeclampsia and FGR; In SLE, it can be given regardless of aPL status.
  • If the risk of VTE is increased — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; If anticoagulant is required, use heparin, which does not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
aPL positive, no history of thrombosis
Obstetric APS criteria
Not met

Approach

  1. Plan pregnancy when the disease is clinically inactive for at least 6 months.
  2. Note that it is difficult to distinguish a lupus flare from the physiological changes of pregnancy and other pregnancy complications.
  3. Note that antiphospholipid antibodies increase the risk of thrombosis through activation of endothelium, platelets, monocytes and complement.
  4. State in counseling that there is an increased risk of maternal VTE, hypertensive disorder, preterm birth, unplanned cesarean section, postpartum hemorrhage, acute renal failure, fetal loss, and fetal growth restriction (FGR).
  5. Autoimmune disease is in the group that benefits most from thromboprophylaxis; Evaluate VTE risk individually throughout pregnancy and after birth.
  6. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.

Treatment

  • Low-dose aspirin (81–162 mg/day): Start at week 12, continue through week 36; It is also recommended in aPL positive women.
  • If the risk of VTE is increased — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; If anticoagulant is required, use heparin, which does not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Meets (obstetric APS)

Approach

  1. Plan pregnancy when the disease is clinically inactive for at least 6 months.
  2. Note that it is difficult to distinguish a lupus flare from the physiological changes of pregnancy and other pregnancy complications.
  3. Verify at least one of the following obstetric criteria after other causes have been excluded: at least three consecutive unexplained pregnancy losses before the 10th week; At least one unexplained death in a morphologically normal fetus at 10+0 weeks or later; birth before the 34th week due to severe preeclampsia, eclampsia, or placental insufficiency.
  4. Confirm the laboratory criterion on two tests at least 12 weeks apart: positive lupus anticoagulant (LA), or moderate-to-high-titer (>40 GPL/MPL or >99th percentile) IgG/IgM anticardiolipin, or IgG/IgM anti-β2 glycoprotein I above the 99th percentile; do not count isolated low-titer positivity alone as an APS laboratory criterion.
  5. Note that LA positivity and triple aPL positivity predict poor pregnancy outcome.
  6. Note that concomitant APS significantly increases the risk of arterial and venous thrombosis.
  7. State in counseling that there is an increased risk of maternal VTE, hypertensive disorder, preterm birth, unplanned cesarean section, postpartum hemorrhage, acute renal failure, fetal loss, and fetal growth restriction (FGR).
  8. Wait at least 12 hours after the last prophylactic dose of LMWH until planned delivery or neuraxial intervention.

Treatment

  • Low-dose aspirin (81–162 mg/day): usually start before pregnancy as it is accompanied by obstetric APS.
  • Add when intrauterine pregnancy is confirmed — Enoxaparin (prophylactic): 40 mg SC once daily for under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (dose is reduced if aPTT is prolonged).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (stop all heparin) or in thrombosis requiring treatment with a history of HIT, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • Continue LMWH for 6 weeks postpartum in women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Accompanied by thrombotic APS (e.g. history of VTE, stroke, MI)

Approach

  1. Plan pregnancy when the disease is clinically inactive for at least 6 months.
  2. Note that it is difficult to distinguish a lupus flare from the physiological changes of pregnancy and other pregnancy complications.
  3. Note that concomitant APS significantly increases the risk of arterial and venous thrombosis.
  4. State in counseling that there is an increased risk of maternal VTE, hypertensive disorder, preterm birth, unplanned cesarean section, postpartum hemorrhage, acute renal failure, fetal loss, and fetal growth restriction (FGR).
  5. Determine the interval for stopping anticoagulation before delivery according to thrombotic risk, anticoagulant type and dose, and whether delivery will be induced or by cesarean section.
  6. Wait at least 24 hours after the last therapeutic dose of LMWH until planned delivery or neuraxial intervention.
  7. Plan postpartum anticoagulation duration according to persistent thrombotic indication.

Treatment

  • Therapeutic dose LMWH (enoxaparin; in pregnancy, instead of pre-pregnancy chronic anticoagulant): 1 mg/kg SC every 12 hours based on early pregnancy weight.
  • Low-dose aspirin (81–162 mg/day): continue or initiate; Consider continuation after week 36 in patients with a history of APS-related thrombotic events such as stroke or myocardial infarction.
  • If creatinine clearance is <30 mL/min, instead of LMWH — UFH (IV, therapeutic): 80 IU/kg IV loading followed by 18 IU/kg/h infusion; aPTT is measured 4–6 hours after the loading dose, 6 hours after each dose change, and at least daily within the therapeutic range; target aPTT ratio 1.5–2.5 times the average control.
  • If baseline aPTT is prolonged (e.g., lupus anticoagulant), adjust UFH dose with anti-Xa instead of aPTT; target anti-Xa 0.3–0.7 IU/mL.
  • If there is a history of HIT or active HIT in therapeutic anticoagulation, treatment dose instead of LMWH — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • If the indication for chronic anticoagulation before pregnancy continues, do not discontinue the treatment just because the 6 weeks after birth are completed.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Thromboembolic risk in heart disease
Heart disease
Fontan circulation, cyanotic or complex repaired CHD

Approach

  1. Consider thrombotic risk particularly high.
  2. Individualize anticoagulation plan based on pre-pregnancy lesion, previous history of thrombosis, oxygen saturation, and ventricular function; Clearly discuss the need for maternal protection, fetal safety and monitoring.
  3. Monitor with the Multidisciplinary Pregnancy Heart Team; Apply careful risk stratification and close monitoring.
  4. Classify uncomplicated Fontan circulation as mWHO III, Fontan circulation with major complications and Eisenmenger syndrome as mWHO IV.
  5. Recommend avoiding pregnancy in patients with major complications of Fontan circulation and Eisenmenger syndrome.
  6. If pregnancy has occurred, discuss the maternal risk, the conditions for continuing in a specialist center, and the option of terminating it under appropriate conditions with the Pregnancy Heart Team in the early period.
  7. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.

Treatment

  • In uncomplicated Fontan circulation, consider low-dose aspirin by joint decision according to complication status: 75–100 mg/day orally.
  • In uncomplicated Fontan circulation, with the same consensus, instead of or in addition to aspirin — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • Only in patients with cyanotic or complex repaired CHD who are assessed as having a high risk of VTE — Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If there is an indication for therapeutic anticoagulation, such as previous thromboembolism, atrial fibrillation or intracardiac thrombus, therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight.
  • At therapeutic dosing, instead of LMWH if creatinine clearance is <30 mL/min or rapid reversal may be needed in the final weeks of pregnancy — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • In prophylaxis, instead of LMWH if creatinine clearance is <30 mL/min or rapid reversal may be required in the last weeks of pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (discontinue all heparin) or with a history of HIT and when therapeutic anticoagulation is required, therapeutic dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; If anticoagulant is required, use heparin, which does not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • In cyanotic pregnant women, heparin may increase the natural hemostasis disorder and lead to heavy bleeding.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
DCM, severe ventricular dysfunction or heart failure (non-PPCM)

Approach

  1. Consider low cardiac output and the tendency for intracardiac thrombi due to stasis.
  2. Individualize anticoagulation plan prior to pregnancy; Clearly discuss the need for maternal protection, fetal safety and monitoring.
  3. Monitor with the Multidisciplinary Pregnancy Heart Team; Apply careful risk stratification and close monitoring.
  4. Classify cardiomyopathy with LVEF <30% or NYHA III–IV symptoms as mWHO IV; Recommend avoiding pregnancy.
  5. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.

Treatment

  • If there is documented intracardiac thrombus or LVEF <35%, therapeutic dose LMWH (enoxaparin): 1 mg/kg SC based on early pregnancy weight every 12 hours.
  • If there is no documented thrombus and LVEF ≥35%, consider thromboprophylaxis individually — Enoxaparin (prophylactic): 40 mg SC once daily for weight under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • At therapeutic dosing, instead of LMWH if creatinine clearance is <30 mL/min or rapid reversal may be needed in the final weeks of pregnancy — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • In prophylaxis, instead of LMWH if creatinine clearance is <30 mL/min or rapid reversal may be required in the last weeks of pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (discontinue all heparin) or with a history of HIT and when therapeutic anticoagulation is required, therapeutic dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; If anticoagulant is required, use heparin, which does not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Pulmonary hypertension

Approach

  1. Consider increased thromboembolic risk, including stroke and pulmonary embolism.
  2. Make the anticoagulation plan according to individual risk classification before pregnancy; determine the type and dose by weighing the risk of bleeding (especially in Eisenmenger syndrome) and VTE at each stage of pregnancy; Clearly discuss the need for maternal protection, fetal safety and monitoring.
  3. Monitor with the Multidisciplinary Pregnancy Heart Team; Apply careful risk stratification and close monitoring.
  4. Classify significant pulmonary arterial hypertension (PAH) as mWHO IV; If pregnancy occurs, discuss the termination option, and if it persists, monitor it in a specialist center experienced in pulmonary hypertension.
  5. If LMWH is used, wait at least 12 hours after the last prophylactic dose until planned delivery or neuraxial intervention and at least 24 hours after the intermediate or therapeutic dose.

Treatment

  • Enoxaparin (prophylactic): 40 mg SC once a day under 100 kg; 60 mg SC for 100–129 kg, 80 mg SC for 130–169 kg once daily (can be divided into 2 doses); 0.6 mg/kg/day SC for 170 kg and above (can be divided into 2 doses).
  • If therapeutic anticoagulation is indicated, therapeutic dose LMWH (enoxaparin): 1 mg/kg SC every 12 hours based on early pregnancy weight; Stop 24 hours before each type of birth.
  • At therapeutic dosing, instead of LMWH if creatinine clearance is <30 mL/min or rapid reversal may be needed in the final weeks of pregnancy — UFH (IV, therapeutic): 80 IU/kg IV loading dose, followed by 18 IU/kg/h infusion; measure aPTT 4–6 hours after the loading dose, 6 hours after each dose change, and at least once daily once in the therapeutic range; target aPTT ratio is 1.5–2.5 times the mean control.
  • In prophylaxis, instead of LMWH if creatinine clearance is <30 mL/min or rapid reversal may be required in the last weeks of pregnancy — UFH (SC, prophylactic): 5000–7500 IU in the 1st trimester, 7500–10 000 IU in the 2nd trimester, 10 000 IU SC every 12 hours in the 3rd trimester (If aPTT is prolonged, the dose is reduced).
  • Instead of LMWH for prophylaxis in a history of HIT — Fondaparinux: 2.5 mg SC once a day for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • In active HIT (discontinue all heparin) or with a history of HIT and when therapeutic anticoagulation is required, therapeutic dose instead of LMWH — Fondaparinux: 2.5 mg SC once daily for prophylaxis; In active HIT or thrombosis requiring treatment, 5 mg under 50 kg, 7.5 mg in 50–100 kg, 10 mg SC once a day over 100 kg.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal birth and 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.

Note

  • Do not use VKA (warfarin) and DOAC during pregnancy; If anticoagulant is required, use heparin, which does not cross the placenta.
  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • Avoid estrogen-containing combined hormonal contraception; Progestin-only methods are safer from a thrombotic perspective.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
  • Enoxaparin (prophylactic): If the daily dose exceeds 40 mg, the timing of neuraxial intervention is planned as for intermediate dose prophylaxis: there is insufficient data to recommend a specific waiting period between 12 and 24 hours, the 12-hour rule is not automatically applied. If creatinine clearance is below 30 mL/min, the prophylactic dose is 30 mg SC once a day.
  • UFH (IV, therapeutic): No loading dose is given after thrombolysis, infusion is started at 18 IU/kg/hour; A loading dose is not given when switching from LMWH, and the infusion is started 1–2 hours before the time of the next LMWH dose. Before neuraxial intervention, the infusion is stopped 4–6 hours and coagulation is evaluated.
  • UFH (SC, prophylactic): before neuraxial procedures or catheter removal, wait 4–6 hours after the last dose of 5000 IU given 2–3 times daily, or assess coagulation; for 7500–10,000 IU twice daily, wait 12 hours after the last dose and assess coagulation; if a single dose exceeds 10,000 IU or the total daily dose exceeds 20,000 IU, wait at least 24 hours after the last dose and confirm that aPTT is normal. The next SC UFH dose may be given 1 hour after the procedure or catheter removal. If UFH has been used for more than 4 days, check the platelet count before the procedure or catheter removal.
  • Fondaparinux: Contraindicated with creatinine clearance below 30 mL/min; Prophylactic use is contraindicated in adults with a body weight below 50 kg, the risk of bleeding increases with low weight; elimination half-life is 17–21 hours; The optimal time between administration and neuraxial intervention is unknown; LMWH's 12/24 hour waiting intervals do not apply to fondaparinux; Benefits and risks are weighed before neuraxial intervention.
Anticoagulant around birth and neuraxial anesthesia
Anticoagulant used
LMWH – prophylactic dose
Mode of birth
Vaginal birth

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue anticoagulant usually 12–24 hours before planned delivery to limit maternal bleeding and neuraxial hematoma; Determine the interval according to thrombotic risk, type and dose of anticoagulant, and whether the birth will be by induction or cesarean section.
  3. Take advantage of UFH as rapid reversal may be required in the last weeks of pregnancy; LMWH is preferred except for the last weeks.
  4. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Wait at least 12 hours after the last prophylactic dose of LMWH until planned delivery or neuraxial intervention.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal delivery.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
Caesarean section

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue anticoagulant usually 12–24 hours before planned delivery to limit maternal bleeding and neuraxial hematoma; Determine the interval according to thrombotic risk, type and dose of anticoagulant, and whether the birth will be by induction or cesarean section.
  3. Take advantage of UFH as rapid reversal may be required in the last weeks of pregnancy; LMWH is preferred except for the last weeks.
  4. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Wait at least 12 hours after the last prophylactic dose of LMWH until planned delivery or neuraxial intervention.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
  • If enoxaparin exceeds 40 mg per day (e.g., 60 mg per day or 40 mg every 12 hours), plan neuraxial intervention and epidural catheter timing with the anesthesia team; do not automatically apply the 12-hour interval used for the standard 40 mg regimen.
LMWH – intermediate or therapeutic dose
Mode of birth
Vaginal birth

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue anticoagulant usually 12–24 hours before planned delivery to limit maternal bleeding and neuraxial hematoma; Determine the interval according to thrombotic risk, type and dose of anticoagulant, and whether the birth will be by induction or cesarean section.
  3. Take advantage of UFH as rapid reversal may be required in the last weeks of pregnancy; LMWH is preferred except for the last weeks.
  4. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Wait at least 24 hours after the last intermediate or therapeutic dose of LMWH until planned delivery or neuraxial intervention.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal delivery.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.
  • In women who have experienced acute VTE during pregnancy, continue postpartum for at least 6 weeks and a total treatment duration of at least 3 months; If there is an indication for permanent/chronic anticoagulation, do not discontinue the treatment at the 6th week and continue according to the underlying indication.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
Caesarean section

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue anticoagulant usually 12–24 hours before planned delivery to limit maternal bleeding and neuraxial hematoma; Determine the interval according to thrombotic risk, type and dose of anticoagulant, and whether the birth will be by induction or cesarean section.
  3. Take advantage of UFH as rapid reversal may be required in the last weeks of pregnancy; LMWH is preferred except for the last weeks.
  4. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.

Treatment

  • Wait at least 24 hours after the last intermediate or therapeutic dose of LMWH until planned delivery or neuraxial intervention.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 6–12 hours after cesarean section.
  • If neuraxial block was performed, initiate standard prophylactic dose LMWH at least 12 hours after intervention, intermediate or therapeutic dose LMWH at least 24 hours after intervention, and in any case at least 4 hours after epidural catheter withdrawal; Remove the epidural catheter at least 12 hours after the last standard prophylactic dose and at least 24 hours after the intermediate or therapeutic dose.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.
  • In women who have experienced acute VTE during pregnancy, continue postpartum for at least 6 weeks and a total treatment duration of at least 3 months; If there is an indication for permanent/chronic anticoagulation, do not discontinue the treatment at the 6th week and continue according to the underlying indication.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
UFH – therapeutic IV infusion
Mode of birth
Vaginal birth

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue therapeutic IV UFH infusion 4–6 hours before planned delivery or neuraxial intervention; Confirm aPTT has returned to normal prior to intervention.
  3. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.
  4. If UFH use exceeds four days, check platelet count before neuraxial intervention or epidural catheter withdrawal.

Treatment

  • Perform neuraxial intervention after aPTT returns to normal; Normalization usually takes approximately 6 hours after the therapeutic IV infusion is discontinued.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal delivery.
  • If neuraxial block was performed and there is no evidence of postpartum hemorrhage, initiate UFH at least 1 hour after the procedure and epidural catheter removal.
  • If the procedure was traumatic, initiate therapeutic anticoagulation at least 24 hours after catheter removal.
  • If switching to LMWH after birth, start the standard prophylactic dose at least 12 hours after the intervention, the intermediate or therapeutic dose at least 24 hours after the procedure, and in any case at least 4 hours after the epidural catheter is removed.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.
  • In women who have experienced acute VTE during pregnancy, continue postpartum for at least 6 weeks and a total treatment duration of at least 3 months; If there is an indication for permanent/chronic anticoagulation, do not discontinue the treatment at the 6th week and continue according to the underlying indication.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
Caesarean section

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue therapeutic IV UFH infusion 4–6 hours before planned delivery or neuraxial intervention; Confirm aPTT has returned to normal prior to intervention.
  3. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.
  4. If UFH use exceeds four days, check platelet count before neuraxial intervention or epidural catheter withdrawal.

Treatment

  • Perform neuraxial intervention after aPTT returns to normal; Normalization usually takes approximately 6 hours after the therapeutic IV infusion is discontinued.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 6–12 hours after cesarean section.
  • If neuraxial block was performed and there is no evidence of postpartum hemorrhage, initiate UFH at least 1 hour after the procedure and epidural catheter removal.
  • If the procedure was traumatic, initiate therapeutic anticoagulation at least 24 hours after catheter removal.
  • If switching to LMWH after birth, start the standard prophylactic dose at least 12 hours after the intervention, the intermediate or therapeutic dose at least 24 hours after the procedure, and in any case at least 4 hours after the epidural catheter is removed.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.
  • In women who have experienced acute VTE during pregnancy, continue postpartum for at least 6 weeks and a total treatment duration of at least 3 months; If there is an indication for permanent/chronic anticoagulation, do not discontinue the treatment at the 6th week and continue according to the underlying indication.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
UFH – subcutaneous (prophylactic or therapeutic)
Mode of birth
Vaginal birth

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue UFH according to dosage before planned delivery to limit maternal bleeding and neuraxial hematoma; Determine the interval according to thrombotic risk and whether the birth will be by induction or cesarean section.
  3. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.
  4. If UFH use exceeds four days, check platelet count before neuraxial intervention or epidural catheter withdrawal.

Treatment

  • Perform neuraxial intervention after aPTT returns to normal; Normalization may take up to 24 hours after subcutaneous administration.
  • If subcutaneous UFH 5,000 units is used two or three times daily, wait at least 4–6 hours after the last dose for neuraxial intervention or evaluate coagulation; If 7,500–10,000 units are used twice daily, wait at least 12 hours and assess coagulation; If >10,000 units per dose or >20,000 units per day, wait at least 24 hours and verify aPTT is normal.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 4–6 hours after vaginal delivery.
  • If neuraxial block was performed and there is no evidence of postpartum hemorrhage, initiate UFH at least 1 hour after intervention and epidural catheter removal; If low-dose SC UFH is used, remove the catheter at least 4–6 hours after the last dose.
  • If the procedure was traumatic, initiate therapeutic anticoagulation at least 24 hours after catheter removal.
  • If switching to LMWH after birth, start the standard prophylactic dose at least 12 hours after the intervention, the intermediate or therapeutic dose at least 24 hours after the procedure, and in any case at least 4 hours after the epidural catheter is removed.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.
  • In women who have experienced acute VTE during pregnancy, continue postpartum for at least 6 weeks and a total treatment duration of at least 3 months; If there is an indication for permanent/chronic anticoagulation, do not discontinue the treatment at the 6th week and continue according to the underlying indication.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
Caesarean section

Approach

  1. Predetermine, document, and explain the perinatal anticoagulant plan to the patient with the obstetrics, anesthesia, and hematology teams.
  2. Discontinue UFH according to dosage before planned delivery to limit maternal bleeding and neuraxial hematoma; Determine the interval according to thrombotic risk and whether the birth will be by induction or cesarean section.
  3. Keep in mind that the risk of VTE is highest in the first 6 weeks after birth.
  4. If UFH use exceeds four days, check platelet count before neuraxial intervention or epidural catheter withdrawal.

Treatment

  • Perform neuraxial intervention after aPTT returns to normal; Normalization may take up to 24 hours after subcutaneous administration.
  • If subcutaneous UFH 5,000 units is used two or three times daily, wait at least 4–6 hours after the last dose for neuraxial intervention or evaluate coagulation; If 7,500–10,000 units are used twice daily, wait at least 12 hours and assess coagulation; If >10,000 units per dose or >20,000 units per day, wait at least 24 hours and verify aPTT is normal.
  • If there is no significant bleeding or significant risk of bleeding, restart anticoagulant 6–12 hours after cesarean section.
  • If neuraxial block was performed and there is no evidence of postpartum hemorrhage, initiate UFH at least 1 hour after intervention and epidural catheter removal; If low-dose SC UFH is used, remove the catheter at least 4–6 hours after the last dose.
  • If the procedure was traumatic, initiate therapeutic anticoagulation at least 24 hours after catheter removal.
  • If switching to LMWH after birth, start the standard prophylactic dose at least 12 hours after the intervention, the intermediate or therapeutic dose at least 24 hours after the procedure, and in any case at least 4 hours after the epidural catheter is removed.
  • In women who have not had VTE during pregnancy and who do not have an indication for long-term anticoagulant use, continue anticoagulant use for 6 weeks postpartum.
  • In women who have experienced acute VTE during pregnancy, continue postpartum for at least 6 weeks and a total treatment duration of at least 3 months; If there is an indication for permanent/chronic anticoagulation, do not discontinue the treatment at the 6th week and continue according to the underlying indication.

Note

  • Do not use DOACs during breastfeeding; they can pass into breast milk and pose a risk to the baby.
Abbreviations: ACE: angiotensin converting enzyme · AF: atrial fibrillation · ALT: alanine aminotransferase · aPL: antiphospholipid antibodies · APS: antiphospholipid syndrome · aPTT: activated partial thromboplastin time · ARB: angiotensin receptor blocker · ARNI: angiotensin receptor–neprilysin inhibitor · ARVC: arrhythmogenic right ventricular cardiomyopathy · ASD: atrial septal defect · ASI: aortic size index · AST: aspartate aminotransferase · AV: atrioventricular · BAV: bicuspid aortic valve · BNP: B-type natriuretic peptide · CT: computed tomography · CTPA: computed tomography pulmonary angiography · C4: complement C4 · CARPREG: cardiac risk score during pregnancy (Cardiac Disease in Pregnancy) · DCM: dilated cardiomyopathy · LMWH: low molecular weight heparin · DOAC: direct acting oral anticoagulant · DVT: deep vein thrombosis · ECMO: extracorporeal membrane oxygenation · ECG: electrocardiography · ECHO: echocardiography · FAST: focused bedside ultrasonography in trauma · FGR: fetal growth restriction · FVL: factor V Leiden · GPL: IgG phospholipid unit · HELLP: hemolysis, elevated liver enzymes and low platelet syndrome · HIT: heparin-induced thrombocytopenia · HCM: hypertrophic cardiomyopathy · ICD: implantable cardioverter-defibrillator · IM: intramuscular · INR: international normalized rate · IO: intraosseous · IU: international unit · IV: intravenous · BP: blood pressure · CHD: congenital heart disease · CPR: cardiopulmonary resuscitation · CPVT: catecholaminergic polymorphic ventricular tachycardia · CUS: compression ultrasonography · LA: lupus anticoagulant · LDH: lactate dehydrogenase · LQT2: long QT syndrome type 2 · LV: left ventricle · LVAD: left ventricular assist device · LVEF: left ventricular ejection fraction · LVOT: left ventricular outflow tract · MCA: middle cerebral artery · MI: myocardial infarction · MPL: IgM phospholipid unit · MRI: magnetic resonance imaging · MTHFR: methylenetetrahydrofolate reductase · mWHO: modified World Health Organization maternal cardiovascular risk classification · NSAID: nonsteroidal anti-inflammatory drug · NST: non-stress test · NT-proBNP: N-terminal pro-B type natriuretic peptide · NYHA: New York Heart Association functional classification · PAH: pulmonary arterial hypertension · PAI-1: plasminogen activator inhibitor-1 · PCC: prothrombin complex concentrate · PDA: patent ductus arteriosus · PE: pulmonary embolism · PPCM: peripartum cardiomyopathy · PR: PR interval in ECG · QRS: QRS complex (ventricular depolarization) on ECG · QT: QT interval on ECG · QTc: heart rate-corrected QT interval · RAAS: renin–angiotensin–aldosterone system · S3: third heart sound · SC: subcutaneous · sFlt-1: soluble fms-like tyrosine kinase-1 · SLE: systemic lupus erythematosus · SpO2: peripheral oxygen saturation · SVT: supraventricular tachycardia · TGA: transposition of the great arteries · UFH: unfractionated heparin · USG: ultrasonography · V/Q: ventilation/perfusion scintigraphy · VA-ECMO: venoarterial extracorporeal membrane oxygenation · VF: ventricular fibrillation · VKA: Vitamin K antagonist · BMI: body mass index · VSD: ventricular septal defect · VT: ventricular tachycardia · VTE: venous thromboembolism · WPW: Wolff–Parkinson–White syndrome · ZAHARA: pregnancy risk score in congenital heart disease