title: Complete atrioventricular septal defects: the influence of associated cardiac anomalies on surgical management and outcome.
abstract: OBJECTIVE
  Major associated cardiac anomalies are known to increase the risk of repair of complete atrioventricular septal defects (CAVSDs). The purpose of this study was to examine the effects of such anomalies on the current surgical management of CAVSDs and their influence on outcome following repair.
  
  
  METHODS
  We performed a retrospective review of a 100 consecutive non-isomeric patients undergoing repair of CAVSD at our institution, between January 1989 and December 1994; patients with partial or intermediate defects were excluded. Complete atrioventricular septal defect patients with other major cardiac abnormalities (complex) were then compared to those with isolated CAVSDs.
  
  
  RESULTS
  There were 15 patients (15%) with associated anomalies; 3 had tetralogy of Fallot, 1 patient had pulmonary atresia, 6 had hypoplastic left or right ventricle, 1 had tetralogy of Fallot and hyperplastic right ventricle, 2 patients had double outlet right ventricle, 1 had hypoplastic aortic arch and 1 patient had aortic coarctation. The median age at operation was similar for both groups (4.2 months), while the median weight was not significantly different for isolated CAVSDs compared to complex (4.2 months vs 3.4 months, P = 0.89), but there was a higher incidence of trisomy 21 (70/85, 82% vs 8/15, 53.3%, P = 0.01). Two of the 85 isolated CAVSD patients (2.3%) had undergone palliative pulmonary artery banding, while 5 of the 15 complex patients (33.3%) had either banding or Blalock-Taussig shunts performed. The technique of CAVSD repair was identical in each group. All complex patients had standard repair of their associated anomalies. Hospital mortality was higher in the complex group (3/15, 20% vs 2/85, 2.3%, P = 0.004); all early deaths in the complex group occurred in patients with a hypoplastic ventricle. Reoperation for left atrioventricular valve regurgitation was required in six isolated CAVSD patients (7.1%) and in one complex (6.6%).
  
  
  CONCLUSIONS
  In the absence of significant ventricular hypoplasia, the early results of surgical repair in patients with CAVSDs and associated cardiovascular anomalies are similar to those achieved in patients with isolated CAVSD.
authors.affiliation: Heart Unit, Birmingham Children's Hospital, Ladywood, UK.
authors.forename: J M
authors.initials: JM
authors.lastname: Redmond
authors.forename: E D
authors.initials: ED
authors.lastname: Silove
authors.forename: J V
authors.initials: JV
authors.lastname: De Giovanni
authors.forename: J G
authors.initials: JG
authors.lastname: Wright
authors.forename: N
authors.initials: N
authors.lastname: Sreeram
authors.forename: W J
authors.initials: WJ
authors.lastname: Brawn
authors.forename: B
authors.initials: B
authors.lastname: Sethia
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities; D001017:Aortic Coarctation / Q000150:complications; D002648:Child; D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000150:complications; D004314:Down Syndrome / Q000150:complications; D004694:Endocardial Cushion Defects / Q000150:complications* / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications; D007223:Infant; D008297:Male; D008722:Methods; D011651:Pulmonary Artery / Q000002:abnormalities; D012086:Reoperation; D012189:Retrospective Studies; D015996:Survival Rate; D013771:Tetralogy of Fallot / Q000150:complications; D016896:Treatment Outcome