Surgical repair of double-outlet right atrium

The case of a patient with abnormal position of the atrial septum resulting in a right atrium with two atrioventricular valves and a disconnected left atrium is presented. The left superior vena cava drained into the left atrium; the right superior vena cava and the coronary sinus were absent. The surgical technique for repair of this anomaly is described. A possible explanation of the embryopathogenesis of double-outlet right atrium is given. To our knowledge, this is the second case of double-outlet right atrium to be reported in the thoracic surgical literature.