Cyanotic Congenital Heart Disease in Pregnancy: A Review of Pathophysiology and Management

The advancement of medical treatment and surgical technique, along with the invention of cardiopulmonary bypass, has allowed for long-term survival of patients with cyanotic congenital heart disease (CHD)-with many women with CHD now reaching child-bearing age and wishing to become pregnant. Pregnancy in these women is a major concern as the physiologic adaptations of pregnancy, including an increased circulating volume, increased cardiac output, reduced systemic vascular resistance, and decreased blood pressure, place a substantial load on the cardiovascular system. These changes are essential to meet the increased maternal and fetal metabolic demands and allow for sufficient placental circulation during gestation. However, in women with underlying structural heart conditions, they place an additional hemodynamic burden on the maternal body. Overall, with appropriate risk stratification, pre-conception counseling, and management by specialized cardiologists and high-risk obstetricians, most women with surgically corrected CHDs are expected to carry healthy pregnancies to term with optimization of both maternal and fetal risks. In this article, we describe the current understanding of 5 cyanotic CHDs-Tetralogy of Fallot, Transposition of the Great Arteries, Truncus Arteriosus, Ebstein's Anomaly, and Eisenmenger Syndrome-and explore the specific hemodynamic consequences, maternal and fetal risks, current guidelines, and outcomes of pregnancy in women with these conditions.