One-stage unifocalization followed by staged Fontan operation.

We report a successful case of staged Fontan operation for single ventricle, pulmonary atresia, major aortopulmonary collaterals and absent central pulmonary artery. The one-stage unifocalization, concomitant with central pulmonary artery creation by autologous pericardium and modified Blalock-Taussig shunt at 1 month of age as the first palliation, and the Glenn operation and pulmonary arterial augmentation with superior vena caval patch at 9 months of age as the second palliation were performed. Extracardiac conduit Fontan operation was completed at 22 months of age. The pulmonary artery continued developing with increasing pulmonary arterial index and stable pulmonary arterial pressure throughout the clinical course. The one-stage unifocalization in early infancy and the repeated surgical intervention to the pulmonary artery without prosthetic material provided excellent pulmonary arterial growth and Fontan completion.