In 3 patients previously undergoing one and one-half ventricular repair, right ventricular dysfunction progressed for more than 10 years. Their clinical features resembled those seen in patients undergoing the atriopulmonary Fontan procedure, and reoperation was carried out for conversion to total cavopulmonary connection. Hemodynamics improved subsequent to the circulatory renewal. In 2 patients having atrial arrhythmia before conversion, the resected right atrial wall illustrated grossly abnormal histopathology. These patients suffered from persistent sinus nodal dysfunction and eventually needed pacemaker implantation. The third patient died of sepsis 4 months later.