Modified Fontan operation for a single ventricle with a nonconfluent pulmonary artery]

A 8-year-old boy with a double inlet right ventricle with a non-confluent pulmonary artery was operated on with a modified Fontan operation. He had right isomerism, right aortic arch, bilateral superior caval veins, and left-sided inferior caval vein. Hepatic veins were separately drained to the right-side atrium. Left Blalock-Taussig shunt and right central shunt operations had been previously performed. Firstly, we had reconstructed the central pulmonary artery with a 16 mm porcine pericardial roll to unify the nonconfluent pulmonary arteries. Secondly, about 2 months after the first operation, we performed a modified Fontan operation. Systemic venous return from the inferior caval vein and the hepatic veins were drained to a reconstructed pericardial roll with an intraatrial Gore-Tex graft, and bilateral superior caval veins were also anastomosed to the roll. The structure of the pulmonary arterial system is one of the most important factors to determine the outcome of a modified Fontan operation. Even if the central pulmonary artery is absent, however, a modified Fontan operation is applicable for the patient whose peripheral pulmonary arteries have enough growth.