Preexisting and acquired cardiovascular disease (CVD) increases maternal and fetal morbidity and mortality during pregnancy.1,2,3,4 CVD complicates more than 1% of pregnancies, accounts for 20% of nonobstetric maternal death,2 and is the leading cause of indirect maternal mortality. Congenital heart disease (CHD) comprises more than 50% of CVD in pregnancy5,6; other common etiologies include rheumatic valve disease (more common in developing countries), connective tissue disease, and cardiomyopathies. Medical care begins in the preconception period with careful planning and anticipation of the possible complications that may occur during the antepartum, intrapartum, and postpartum periods. Risk-stratification models summarizing maternal and fetal outcomes have been developed to counsel women with CVD desiring pregnancy. Optimal patient care for the pregnant woman with CVD relies on understanding of the unique hemodynamic changes of pregnancy and the pathophysiology, signs and symptoms, and natural history specific to each heart condition that may impact pregnancy. A multidisciplinary team approach involving cardiologists, maternal fetal medicine specialists, and anesthesiologists in a center with experience is strongly advised for the care of pregnant women with heart disease.
Women with CVD should receive counseling regarding both maternal and fetal risks prior to conceiving a pregnancy. In addition, women with heart disease should be cared for in institutions with experience in treating CVD during pregnancy. There are extremely high-risk conditions in which pregnancy is not advised, including pulmonary arterial hypertension (PAH), congenital cyanotic lesions, severe systemic ventricular dysfunction (ejection fraction < 30%, New York Heart Association [NYHA] class > II), severe mitral stenosis, severe symptomatic aortic stenosis, significantly dilated aorta in connective tissue disorders in women with Marfan syndrome and above 5 cm in women with bicuspid aortic valve (BAV).1 Women with CVD considering pregnancy should undergo a complete workup, including a detailed medical and surgical history and a physical exam including oxygen saturations, electrocardiogram (ECG), appropriate cardiac imaging, and consideration of cardiopulmonary stress testing for further risk stratification.7
Genetic counseling is appropriate for patients with family members affected by congenital and inherited heart diseases (eg, hypertrophic cardiomyopathy, Marfan syndrome, long-QT syndrome). In general, CHD is present in about 0.8% of the population, yet for an affected individual, the risk of bearing children with CHD is increased to 2% to 6% regardless of the congenital diagnosis.8,9 Further recurrences of CHD in the same family are higher. A fetal echocardiogram is advised for parents with CHD between 18 and 22 weeks of gestation.10
Preconceptual counseling will allow for careful planning for any anticipated or potential events during pregnancy. All prepregnancy medications should be reviewed to ensure their safety in pregnancy. For example, angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers, and endothelin receptor blockers are teratogenic and contraindicated during pregnancy, and should be discontinued. Women requiring anticoagulation must be advised of the challenges of managing anticoagulation during pregnancy and individualized strategies should be ...