Prenatal echocardiographic diagnosis of Ebstein's anomaly with pulmonary atresia

Ebstein's anomaly with pulmonary atresia was diagnosed in utero for the first time by fetal echocardiography. The fetus, examined at 35 weeks' gestation, had no extracardiac sonographic evidence of heart failure, but the heart filled the anterior portion of the chest. Two-dimensional echocardiography revealed a huge right atrium. The tricuspid valve, instead of originating from the anulus, was demonstrated as originating from the wall of the right ventricle, thus dividing the ventricle into two parts: the true ventricle and the "atrialized portion" of the right ventricle. These features corresponded with Ebstein's anomaly. Short episodes of supraventricular tachycardia (250 bpm) corresponded with the tachyarrhythmias frequently associated with Ebstein's anomaly. Pulmonary atresia was suspected, as movement of the pulmonary valve could not be demonstrated. The baby was delivered at 40 weeks' gestation and died 3 days later. Postnatal cardiac catheterization and angiocardiography and postmortem examination confirmed the prenatal diagnosis.