We describe an infant with the antenatal diagnosis of a large intracardiac rhabdomyoma causing tricuspid inflow obstruction. Postnatal cyanosis and arterial hypoxemia prompted commencement of intravenous prostaglandin E1 with immediate improvement in saturations. Clinical and genetic testing confirmed diagnosis of tuberous sclerosis. To secure ductal patency, two tandem coronary stents were implanted via an antegrade approach. Several episodes of long RP atrio-ventricular re-entrant tachycardia were observed. At 7½ months of age, the tumor was no longer obstructive to tricuspid inflow, ductal flow was highly restrictive and there was no recurrence of supraventricular tachycardia. ' 2007 Wiley-Liss, Inc.Key words: rhabdomyoma; tricuspid inflow obstruction; stent implantation; patent arterial duct; SVT CASE REPORT A female baby was born at term by vaginal delivery to a healthy 33-year-old mother who had one previous miscarriage. There was no family history of tuberous sclerosis (TS). Surveillance obstetrical ultrasound scans at 12, 18, and 22 weeks gestation were normal. At 36 weeks gestation, polyhydramnios, and a cardiac mass were detected. The fetal echocardiogram revealed multiple homogeneous well circumscribed tumors within the myocardial wall. The largest mass was attached to the tricuspid valve and measured 1.3 3 1 cm 2 ( Fig. 1A and B). There was tricuspid inflow acceleration to a peak velocity of 126 cm/s and abnormal inferior and superior vena caval Doppler flow patterns with increased A-wave reversal consistent with right ventricular inflow obstruction. Rhabdomyomas associated with TS were quoted as most likely diagnosis.No resuscitation was required at birth. Birth weight was 2.8 kg. Hyperoxia test at 1 hr of age demonstrated a preductal PaO 2 of 80 mmHg. At 3 hr of age, cyanosis with a transcutaneous oxygen saturation of 82% in room air prompted commencement of prostaglandin E1 (alprostadil) infusion with immediate improvement in oxygen saturations to 95%. Neonatal examination was unremarkable except an ejection systolic murmur at the left upper sternal border. No dysmorphic features or skin lesions were found.Electrocardiogram on admission demonstrated atrial enlargement, right ventricular hypertrophy, and no preexcitation. The postnatal echocardiogram confirmed presence of multiple rhabdomyomas in the right and left ventricular free wall, interventricular septum, and left atrial appendage. The largest tumor (Fig. 1C and D) was attached to the atrial surface of anterior tricuspid leaflet causing severe tricuspid inflow obstruction and right to left shunt across the patent foramen ovale. The rest of the cardiovascular anatomy was normal and there was a patent ductus arteriosus (PDA).