[Staged operation for right isomerism with total anomalous pulmonary venous return: report of a case]

A male infant with right isomerism, single ventricle, severe pulmonary stenosis (PS), and total anomalous pulmonary venous return (TAPVR) underwent successful staged Fontan operation. Pulmonary vein drained into right superior vena cava without a stenotic lesion. Common pulmonary venous chamber was also connected with the atrium by a thin bridging vein. Blalock-Taussig shunt was performed at 2-month-old. Because of progressive pulmonary congestion, an anastomosis between common pulmonary venous chamber and the atrium followed 4 days after the first shunt. Bidirectional cavopulmonary shunt was performed 7 months after the second operation. Because of progressive atrioventricular valve regurgitation, repeated bandings of the shunt were required to regulate the pulmonary flow. Total cavopulmonary connection was completed 9 months after the BCPS. Staged operation is a useful strategy to acquire an appropriate pulmonary blood flow for the isomeric heart with TAPVR and severe PS.