title: Successful repair and postoperative management of tetralogy of Fallot in a patient with maple syrup urine disease.
abstract: 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.
authors.affiliation: Department of Surgery III, Nara Medical College, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Fukutomi
authors.forename: S
authors.initials: S
authors.lastname: Kitamura
authors.forename: K
authors.initials: K
authors.lastname: Kawachi
authors.forename: T
authors.initials: T
authors.lastname: Tsuji
authors.forename: K
authors.initials: K
authors.lastname: Hashimoto
authors.forename: A
authors.initials: A
authors.lastname: Yoshioka
mesh_terms: D002315:Cardiopulmonary Bypass; D002675:Child, Preschool; D004750:Enteral Nutrition; D005260:Female; D006801:Humans; D008375:Maple Syrup Urine Disease / Q000150:complications* / Q000628:therapy; D010288:Parenteral Nutrition; D010530:Peritoneal Dialysis; D011182:Postoperative Care; D011183:Postoperative Complications / Q000628:therapy*; D013771:Tetralogy of Fallot / Q000150:complications / Q000601:surgery*