title: Atrioventricular septal defect and tetralogy of Fallot: a 15-year experience.
abstract: OBJECTIVE
  Atrioventricular septal defect and tetralogy of Fallot is a relatively uncommon lesion in which there is a risk of right ventricular dysfunction related to inlet and outlet valve problems. For this reason, conservative management involving an initial palliative procedure is often chosen. The aim of this report is to retrospectively review our experience with this lesion.
  
  
  METHODS
  35 patients with atrioventricular septal defect and tetralogy of Fallot have been surgically managed at this institution between January 1980 and June 1995. Twenty-one (60%) of these patients underwent 28 initial palliative shunt procedures. Fourteen (40%) patients underwent primary definitive repair. The criteria for choosing one management strategy over another was based on a number of factors, including age at presentation, anatomy of the lesion, and severity of symptoms. Of the 21 patients who underwent an initial shunt procedure, 15 have undergone definitive operation. Of the 6 patients who did not undergo definitive operation, three died (two directly related to complications of the shunt procedure), two are awaiting operation, and one was lost to follow-up.
  
  
  RESULTS
  The primary indication for operation in all patients was cyanosis. Freedom from reoperation at 5 years after definitive operation was 65.1% for all patients; most reoperations were related to left atrioventricular valve regurgitation or residual leaks across the ventricular septal defect patch. The operative mortality at definitive operation was 10.3% (70% CL 4.5-20%) for all patients. The actuarial estimate of survival 7 years following definitive repair was 77.3% (70% CL 68.7-85.9%) for all patients. The actuarial estimate of survival at 7 years was 84.4% (70% CL 73.8-95%) in the patients undergoing primary repair and 65% (70% CL 52.4-77.6%) in patients initially palliated if the mortality of the palliative shunt procedure is included (P = 0.35).
  
  
  CONCLUSIONS
  Patients with atrioventricular septal defect and tetralogy of Fallot can be successfully managed with a variety of surgical strategies. Primary repair may be a reasonable option in carefully selected patients, as this eliminates the morbidity and mortality of an initial shunt procedure and the subsequent interval between initial palliation and definitive repair.
authors.affiliation: Cardiothoracic Unit, Great Ormond Street Hospital for Children, NHS Trust, London, UK.
authors.forename: R E
authors.initials: RE
authors.lastname: Delius
authors.forename: R V
authors.initials: RV
authors.lastname: Kumar
authors.forename: M J
authors.initials: MJ
authors.lastname: Elliott
authors.forename: J
authors.initials: J
authors.lastname: Stark
authors.forename: M R
authors.initials: MR
authors.lastname: de Leval
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery*; D000206:Actuarial Analysis; D006348:Cardiac Surgical Procedures / Q000379:methods; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006344:Heart Septal Defects, Atrial / Q000401:mortality / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000401:mortality / Q000601:surgery*; D006801:Humans; D008297:Male; D010166:Palliative Care; D011379:Prognosis; D012086:Reoperation; D012189:Retrospective Studies; D015996:Survival Rate; D013771:Tetralogy of Fallot / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome; D006113:United Kingdom