2017 年 1 巻 1 号 p. 79-82
Double-outlet right ventricle with intact ventricular septum is an extremely rare heart malformation. To our knowledge, only 42 cases have been reported, and there is no previous case report achieving Fontan completion. The diagnosis was made by echocardiography and contrast-enhanced cardiac computed tomography. Our patient had additional interesting anomalies such as sinusoidal communications. We describe a successful case of staged Fontan completion for double-outlet right ventricle with intact ventricular septum.