SEVERE PULMONARY-HYPERTENSION IN PREGNANCY FOLLOWING SUCCESSFUL REPAIR OF VENTRICULAR SEPTAL-DEFECT IN CHILDHOOD

Background: Because of advances in surgical repair, an increasing numb er of women born with structural cardiac disease now live to reproduct ive age. Patients treated successfully in childhood are followed for v arying 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 cardiovasc ular decompensation, even after years of apparently good health. Cases : We have recently cared for two women who had undergone repair of con genital heart disease in childhood. Although they thought that their r epair was complete and they had been asymptomatic until the pregnancy, both presented with symptoms and signs of severe pulmonary hypertensi on, subsequently confirmed on cardiac catheterization. One patient ele cted to terminate her pregnancy, and the other died in the immediate p uerperium. Conclusion: Despite normal physical function and an absence of abnormal physical findings, a thorough cardiac evaluation includin g echocardiography should be considered for pregnant patients with a h istory of repaired congenital heart disease, especially if the origina l defect is known to lead to pulmonary hypertension.