title: Anatomic features and surgical strategies in double-outlet right ventricle.
abstract: BACKGROUND
  The objective of this study was to review anatomic features and surgical strategies in children with double-outlet right ventricle (DORV) and to assess risk factors for early mortality.
  
  
  RESULTS
  Records were reviewed of all children with DORV undergoing surgery between 1978 and 1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect (VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex) comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76 group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years.
  
  
  CONCLUSIONS
  Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a Fontan procedure is associated with a lower surgical mortality.
authors.affiliation: Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
authors.forename: S
authors.initials: S
authors.lastname: Kleinert
authors.forename: T
authors.initials: T
authors.lastname: Sano
authors.forename: R G
authors.initials: RG
authors.lastname: Weintraub
authors.forename: R B
authors.initials: RB
authors.lastname: Mee
authors.forename: T R
authors.initials: TR
authors.lastname: Karl
authors.forename: J L
authors.initials: JL
authors.lastname: Wilkinson
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000401:mortality / Q000473:pathology* / Q000601:surgery*; D018729:Fontan Procedure; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors