Endocardial fibroelastosis: an unusual cause of pulmonary hypertension in pregnancy.

Pulmonary hypertension due to endocardial fibroelastosis is usually diagnosed during infancy and childhood and is almost uniformly lethal when severe. Since females with this disorder rarely reach reproductive age, no cases of successful pregnancy in the presence of this severe cardiopulmonary disease have been reported. A 23-year-old Caucasian primigravida with a history of congenital endocardial fibroelastosis and severe pulmonary hypertension presented at 20 weeks' gestation. Following cardiac catheterization, the pregnancy was managed with bed rest, oral theophylline and digoxin, and low-flow oxygen therapy. After spontaneous onset of labor at 35 weeks, invasive hemodynamic monitoring and epidural anesthesia were initiated. Worsening of maternal pulmonary artery pressures postpartum was relieved by intravenous nitroglycerin infusion. Recent advances in medical care have resulted in more women with endocardial fibroelastosis reaching reproductive age. Successful pregnancy outcome is possible using established techniques of modern obstetric care.