title: Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot.
abstract: OBJECTIVE
  The objective was to explore the best management algorithm for atrioventricular septal defect in conjunction with tetralogy of Fallot.
  
  
  METHODS
  We reviewed the cases of 38 children referred to our division (March 1981-August 1997) who had atrioventricular septal defect associated with tetralogy of Fallot; 32 (84%) had Down syndrome. Twenty-one received initial palliation with a systemic-to-pulmonary artery shunt; of these, 2 (9.5%) died before complete repair. Thirty-one children underwent complete repair; 14 of these (45%) had undergone initial palliation (mean age at shunt 20 +/- 24 months). Right ventricular outflow obstruction was relieved by a transannular patch in 22 (71%); 14 (64% of 22) had a monocuspid valve inserted. Four required an infundibular patch.
  
  
  RESULTS
  Two children (6.4%) died early after repair; 1 had undergone previous palliation. Patients with palliation underwent repair at an older age (78 vs 36 months), required longer ventilatory support (8 vs 4 days) and inotropic support (8 vs 4 days), and had longer intensive care stays (11 vs 6 days) and hospital stays (24 vs 15 days). Eleven children (35%) underwent reoperation, 7 (58%) for right ventricular outflow reconstruction and pulmonary arterioplasty. Reoperation was more frequent in the palliation group than in the primary operation group (64% vs 12%). The single late death was related to a reoperation in the palliation group.
  
  
  CONCLUSIONS
  Atrioventricular septal defect with tetralogy of Fallot can be repaired with a low mortality rate. Initial palliation with a shunt resulted in a more complex postoperative course and a higher reoperative rate. Primary repair is superior to initial palliation with later repair.
authors.affiliation: Division of Cardiovascular Surgery, Department of Surgery, Hospital for Sick Children and the Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: H K
authors.initials: HK
authors.lastname: Najm
authors.forename: G S
authors.initials: GS
authors.lastname: Van Arsdell
authors.forename: S
authors.initials: S
authors.lastname: Watzka
authors.forename: L
authors.initials: L
authors.lastname: Hornberger
authors.forename: J G
authors.initials: JG
authors.lastname: Coles
authors.forename: W G
authors.initials: WG
authors.lastname: Williams
mesh_terms: D006348:Cardiac Surgical Procedures; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D008297:Male; D010166:Palliative Care; D012086:Reoperation; D012189:Retrospective Studies; D015996:Survival Rate; D013771:Tetralogy of Fallot / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome