Successful prenatal digoxin therapy for Ebstein's anomaly with hydrops fetalis. A case report

Ebstein anomaly is a rare tricuspid valve anomaly. Some fetuses with Ebstein's anomaly have concurrent severe cardiac function impairment, which results in hydrops fetalis. Most of these fetuses are inevitably terminally ill. No reports have demonstrated the potential prenatal therapy for fetuses under such conditions.

Ebstein's anomaly and hydrops fetalis were detected at 28 weeks' gestation. Tricuspid regurgitation with congestive heart failure was observed. From 28 to 34 weeks' gestation, intrauterine therapy with digoxin, 0.75 mg/d, was administered. The fetal hydrops status improved gradually, while the tricuspid valve regurgitation persisted. At 36 weeks' gestation the fetus was delivered normally. During the neonatal phase, digoxin was continued and gradually tapered off. The tricuspid valve regurgitation and cardiomegaly gradually improved.

The favorable outcome in this case supports the positive effect of prenatal digoxin therapy for Ebstein's anomaly with hydrops fetalis. In such conditions, upon the appearance of hydrops and congestive cardiac failure, immediate digoxin therapy may be useful. This successful trial encouraged us to manage such fetuses more aggressively.