Management of Women With Congenital or Inherited Cardiovascular Disease From Pre-Conception Through Pregnancy and Postpartum: JACC Focus Seminar 2/5

Abstract

Maternal morbidity and mortality continue to rise in the United States, with cardiovascular disease as the leading cause of maternal deaths. Congenital heart disease is now the most common cardiovascular condition encountered during pregnancy, and its prevalence will continue to grow. In tandem with these trends, maternal cardiovascular health is becoming increasingly complex. The identification of women at highest risk for cardiovascular complications is essential, and a team-based approach is recommended to optimize maternal and fetal outcomes. This document, the second of a 5-part series, will provide practical guidance from pre-conception through postpartum for cardiovascular conditions that are predominantly congenital or heritable in nature, including aortopathies, congenital heart disease, pulmonary hypertension, and valvular heart disease.

Keywords: aortopathy; cardio-obstetrics; congenital; pregnancy; pulmonary hypertension.

Figure 1.

Algorithm for preconception stress testing for women with CHD. Preconception exercise testing may be considered for risk stratification, in particular for women with congenital or valvular conditions. VO2 – Maximum oxygen consumption.

Figure 2.. Safety and Efficacy of Contraceptives in Women with CHD.

Long-acting reversible contraceptives (the IUD and subdermal implant) and permanent sterilization are safe and highly effective for all women with CHD. Caution should be advised when prescribing combined hormonal contraceptives to women with certain CHD conditions due to the increased risk of thromboembolism. ASD – Atrial septal defect.

Figure 3.. Delivery Planning in Women with CHD.

The multidisciplinary cardio-obstetrics team should collaborate to develop a delivery plan by the early third trimester. The delivery plan should include recommendations for anesthesia, monitoring, and timing/mode of delivery. CHD – Congenital Heart Disease. MFM – Maternal Fetal Medicine. OB – Obstetric. IV – intravenous. WHO – World Health Organization.

Figure 4.. Contributors to Pregnancy-Associated Right Heart Failure in Women with Pulmonary Hypertension.

Maternal cardiovascular morbidity and mortality due to PAH is most often related to the precipitation of right ventricular failure. Numerous physiologic changes in pregnancy and the postpartum period predispose to right heart failure, including tachycardia, volume overload, hypoxia and hypercoagulability.

Figure 5.. Contraception Recommendations for Women with Pulmonary Hypertension.

Highly effective contraception is recommended to prevent pregnancy in women with pulmonary hypertension. While progesterone only pills and medroxyprogesterone acetate injection are safe, they are less effective than the long acting reversible and permanent contraceptive options. IUD – Intrauterine Device

Figure 6.. Algorithm for the Management of Pregnancy in Women with Pulmonary Hypertension.

Management of pregnancy in a women with PAH requires meticulous multidisciplinary collaboration in a center experienced in managing pregnancy in this population of patients. Close monitoring before, during and after delivery is essential to reduce adverse maternal events. DVT – Deep Vein Thrombosis. ICU – Intensive Care Unit.

Figure 7.. Contraception Recommendations for Women with Valvular Heart Disease.

Long-acting reversible contraceptives (the IUD and subdermal implant) and permanent sterilization are safe and highly effective for all women with valvular heart disease. Caution should be advised when prescribing combined hormonal contraceptives to women with mechanical valves or atrial fibrillation due to the increased risk of thromboembolism.

Figure 8.. Delivery Planning in Women with Valvular Heart Disease.

The multidisciplinary cardio-obstetrics team should collaborate to develop a delivery plan for women with mWHO class III-IV conditions. The delivery plan should include recommendations for anesthesia, monitoring, and timing/mode of delivery. Extended postpartum monitoring should be considered for women with ≥ moderate valve lesions due to the increased risk of postpartum heart failure. mWHO – modified World Health Organization. AS – Aortic Stenosis. MS – Mitral Stenosis. ICU – Intensive Care Unit.

Figure 9.. Maternal and Fetal Factors to Consider in the Management of Prosthetic Valves in Pregnant Women.

Maternal physiologic changes must be considered in addition to fetal risks of warfarin exposure when considering optimal medication choice and dosing for women requiring anticoagulation during pregnancy. VKA – Vitamin K Antagonists.

Figure 10.. Management Algorithm for Pregnant Women with Mechanical Heart Valves.

Reproduced from the 2020 ACC/AHA Valvular Heart Disease Guidelines. Shared decision making should be employed to determine the optimal anticoagulant dosing regimen for each individual patient during pregnancy. Weekly INR or Xa levels should be monitored to ensure adequate anticoagulation. Warfarin and LMWH need to be discontinued in advance of delivery, with an intravenous UFH bridge to permit for regional anesthesia. *Dose-adjusted LMWH should be given at least 2 times per day, with close monitoring of anti-Xa levels. Target to Xa level of 0.8 to 1.2 U/mL, 4–6 hours after dose. Trough levels may aid in maintaining patient in therapeutic range. Continuous UFH should be adjusted to aPTT 2 times control. Green: Class I recommendation; Yellow: Class IIa recommendation; Orange: Class IIb recommendation.

Figure 11.. Hemodynamic Changes and Proposed Management Algorithm for Pregnant Women with Aortic Stenosis.

Aortic valve gradients are expected to increase in the setting of pregnancy, and may lead to adverse maternal outcomes. Serial imaging, heart rate control and volume management are useful in caring for pregnant women with MS. While most women may undergo vaginal delivery, some may need consideration of assisted second stage or Cesarean delivery depending on disease severity and clinical status. LV – Left ventricular.

Figure 12.. Hemodynamic Changes and Proposed management Algorithm for Pregnant Women with Mitral Stenosis.

Mitral valve gradients are expected to increase in the setting of pregnancy, and may lead to adverse maternal outcomes. Serial imaging, heart rate control, volume management and anticoagulation are useful in caring for pregnant women with MS. While most women may undergo vaginal delivery, some may need consideration of assisted second stage or Cesarean delivery depending on disease severity and clinical status.LA – Left atrial. MVA – Mitral Valve Area. MS – Mitral Stenosis. HR – Heart Rate. *Anticoagulation is indicated for atrial fibrillation, left atrial thrombosis, prior embolism, severe MS, spontaneous echo contrast in the LA, Left atrial volume index ≥60 mL/m2, or heart failure.

Central Illustration.. Multidisciplinary Cardio-Obstetrics Team Management for Women with Congenital Heart Disease.

CHD – Congenital Heart Disease. OB/GYN – Obstetrics and Gynecology. MFM – Maternal-Fetal Medicine. BNP – Brain Natriuretic Peptide. NT-proBNP – N-terminal-pro hormone BNP. Anatomical Classification: I - Simple complexity: Small atrial septal defect (ASD) or ventricular septal defect (VSD), mild pulmonary stenosis (PS), repaired ductus arteriosus, repaired secundum or sinus venosus ASD or VSD without residual shunt or chamber enlargement. II - Moderate complexity: Aorto-left ventricular fistula, anomalous pulmonary venous return, anomalous coronary artery arising from pulmonary artery, anomalous coronary artery arising from opposite sinus, partial or complete atrioventricular septal defect, congenital aortic or mitral valve disease, coarctation of the aorta, Ebstein anomaly, ostium primum ASD, ≥ moderate unrepaired ASD, ≥ moderate unrepaired patent ductus arteriosus, ≥ moderate PS, ≥ pulmonary regurgitation, peripheral PS, sinus of Valsalva fistula/aneurysm, sinus venosus defect, subvalvular or supravalvular aortic stenosis, Tetralogy of Fallot, VSD with associated anomalies and/or ≥ moderate shunt. III - Great complexity: Cyanotic congenital heart defect (unrepaired or palliated), double-outlet ventricle, Fontan procedure, interrupted aortic arch, mitral atresia, single ventricle, pulmonary atresia, transposition of the great arteries (any form), truncus arteriosus, other abnormalities of atrioventricular and ventriculoarterial connection (crisscross heart, isomerism, heterotaxy syndromes, ventricular inversion)(3) Physiological Stage: A – NYHA functional class I symptoms. No hemodynamic or anatomic sequelae. No arrhythmias. Normal exercise capacity. Normal renal/hepatic/pulmonary function. B – NYHA functional class II symptoms. Mild hemodynamic sequelae (mild aortic enlargement, mild ventricular enlargement, mild ventricular dysfunction). Mild valvular disease. Trivial or small shunt (not hemodynamically significant). Arrhythmia not requiring treatment. Abnormal objective cardiac limitation to exercise. C – NYHA functional class III symptoms. Significant (≥ moderate) valvular disease. ≥ moderate ventricular dysfunction (systemic, pulmonic, or both). Moderate aortic enlargement. Venous or arterial stenosis. Mild or moderate hypoxemia/cyanosis. Hemodynamically significant shunt. Arrhythmias controlled with treatment. Pulmonary hypertension (less than severe). End-organ dysfunction responsive to therapy.D – NYHA functional class IV symptoms. Severe aortic enlargement. Arrhythmias refractory to treatment. Severe hypoxiemia (almost always associated with cyanosis). Severe pulmonary hypertension. Eisenmenger syndrome. Refractory end-organ dysfunction.