Pregnancy in Women with Congenital Heart Disease

Advances in cardiac surgical interventions in infancy and childhood have led to an increased number of women with congenital heart disease of childbearing age. For these women, individualized preconception counseling and pregnancy planning should be a vital component of their medical management, and presentation for obstetric care may even be an opportunity to re-establish cardiovascular care for patients who have been lost to follow-up. These patients have unique cardiovascular anatomy and physiology, which is dependent upon the surgical intervention they may have undergone during childhood or adolescence. These factors are associated with a variety of long-term complications, and the normal hemodynamic changes of pregnancy may unmask cardiac dysfunction and pose significant risk. Among three published risk assessment algorithms, the World Health Organization classification is the most sensitive in predicting maternal cardiovascular events in this population. Women with simple congenital heart defects generally tolerate pregnancy well and can be cared for in the community with careful monitoring. Conversely, women with complex congenital defects, with or without surgical repair and/or residual defects, should be managed in tertiary care centers under a multidisciplinary team of physicians experienced in adult congenital heart disease and high-risk obstetrics, who collaboratively participate in pregnancy planning, management, and care through childbirth and postpartum. Women who are cyanotic with oxygen saturation less than 85%, have significant pulmonary arterial hypertension of any cause, or have systemic ventricular dysfunction should be counseled to avoid pregnancy due to a very high risk of maternal and fetal mortality.