A successful staged Fontan operation for a right atrial isomerism neonate having major aortopulmonary collateral arteries and extracardiac total anomalous pulmonary venous connection.

Right atrial isomerism and pulmonary atresia combined with major aortopulmonary collateral arteries are very rare. Surgical treatment becomes more challenging when an extracardiac total anomalous pulmonary venous connection (TAPVC) requiring surgical repair neonatally is also present. We describe a successful staged Fontan operation for a neonate with a single ventricle, right atrial isomerism, pulmonary atresia with major aortopulmonary collateral arteries, a small central pulmonary artery and a supracardiac TAPVC. Unifocalization of the pulmonary vasculature was achieved by ligating all dual-supply major aortopulmonary collateral arteries at their origins, concomitant with supracardiac TAPVC repair and modified Blalock-Taussig shunt placement at 15 days. The patient underwent patch augmentation of the small stenosed central pulmonary artery at 8 months and bilateral bidirectional Glenn shunt at 14 months. The Fontan operation was completed at 27 months using an intra-extracardiac conduit with fenestration. One year later, catheter examination revealed good Fontan circulation with acceptable central venous pressure, oxygen saturation and cardiac function. We also describe a new angiographic concept of central pulmonary arteriography to visualize more accurately the complex pulmonary circulation system with both major aortopulmonary collateral arteries and a central pulmonary artery.