A neonate with prenatally diagnosed large intracardiac rhabdomyomas and suspicion of tuberous sclerosis presented at birth with severe ductal-dependent obstruction at the tricuspid valve and an atrial septal defect (ASD). Biventricular repair at 9 days of life included tumor resection, repair of the posterior leaflet of the tricuspid valve with autologous pericardium, fenestrated ASD closure, and ductus ligation. After an uneventful postoperative recovery, follow-up echocardiography at two months showed excellent results with tricuspid valve competency and normal biventricular function. Overall prognosis will probably depend on issues pertaining to tuberous sclerosis.
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