Severe pulmonary hypertension in pregnancy following successful repair of ventricular septal defect in childhood.

BACKGROUND: Because of advances in surgical repair, an increasing number of women born with structural cardiac disease now live to reproductive age. Patients treated successfully in childhood are followed for varying periods of time, then may be lost to follow-up or told that no follow-up is necessary because their condition is stable. However, the hemodynamic changes that accompany pregnancy may result in cardiovascular decompensation, even after years of apparently good health. CASES: We have recently cared for two women who had undergone repair of congenital heart disease in childhood. Although they thought that their repair was complete and they had been asymptomatic until the pregnancy, both presented with symptoms and signs of severe pulmonary hypertension, subsequently confirmed on cardiac catheterization. One patient elected to terminate her pregnancy, and the other died in the immediate puerperium. CONCLUSION: Despite normal physical function and an absence of abnormal physical findings, a thorough cardiac evaluation including echocardiography should be considered for pregnant patients with a history of repaired congenital heart disease, especially if the original defect is known to lead to pulmonary hypertension.