Successful repair and postoperative management of tetralogy of Fallot in a patient with maple syrup urine disease.

A case of tetralogy of Fallot with maple syrup urine disease (MSUD) treated surgically is reported. This 2-year-old female patient developed neurologic deterioration after intracardiac repair. Postoperative management, which included peritoneal dialysis, intravenous hyperalimentation, and early intestinal alimentation with branched-chain amino acid-free milk was successful. At the present time, two years after the operation, she is doing quite well with a special diet for MSUD. This is the first report on a patient with MSUD who underwent open heart surgery using a cardiopulmonary bypass, stressing the importance of postoperative management.