A 13-year-old girl, who had undergone the total correction of partial atrioventricular septal defect at the age of 4 years, was admitted with severe tricuspid regurgitation in echocardiography. She had received one-and-a-half ventricle repair during follow-up. Her right ventricle showed global akinesia, and the ejection fraction of the left ventricle was 25% with paradoxical interventricular septal motion. We performed right ventricular exclusion adjunct to the Fontan procedure. She is doing well two years after the operation without complications.