title: Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect.
abstract: BACKGROUND
  Double-outlet right ventricle encompasses a broad spectrum of anomalies. Heterotaxy syndrome, which is often associated with total anomalous pulmonary venous connection and complete atrioventricular canal defect, has been considered a risk factor for surgical repair of double-outlet right ventricle.
  
  
  METHODS
  From January 1992 to May 1999, medical records of 96 patients (50 males, 46 females) who had complex double-outlet right ventricle with heterotaxy and/or complete atrioventricular canal defect were reviewed (median age at initial surgery 3 months). Seventeen patients were neonates requiring surgery. Follow-up ranged from 1 day to 7.4 years (median, 16 months).
  
  
  RESULTS
  Sixty-eight patients had heterotaxy syndrome (27 with total anomalous pulmonary venous connection). Eighty-three had complete atrioventricular canal defect, 22 with moderate to severe atrioventricular valve regurgitation at the time of surgical repair. Eight patients had two-ventricle repair, and 88 patients were considered for single-ventricle management (bidirectional Glenn, 37; Fontan, 44). One patient had heart transplantation after bidirectional Glenn. There were 16 deaths including 10 early (<30 days postoperatively). Overall survival (95% confidence interval) estimated by the Kaplan-Meier method was 89% (83% to 96%) at 1 month, 84% (76% to 91%) at 1 year, and 81% (73% to 89%) at 5 years. Multivariate analysis revealed that neonatal presentation requiring surgery (p < 0.0001), moderate to severe atrioventricular valve regurgitation (p = 0.03), and pulmonary venous obstruction (p = 0.02) were risk factors for death.
  
  
  CONCLUSIONS
  Atrioventricular valve regurgitation, pulmonary venous obstruction, and neonatal presentation are risk factors for mortality in patients with complex double-outlet right ventricle. Early surgical intervention in symptomatic neonates and infants, including those with pulmonary venous obstruction, may reduce mortality and improve outcome after staged operation.
authors.affiliation: Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. koutakeuchi-circ@umin.ac.jp
authors.forename: Koh
authors.initials: K
authors.lastname: Takeuchi
authors.forename: Francis X
authors.initials: FX
authors.lastname: McGowan
authors.forename: Emile A
authors.initials: EA
authors.lastname: Bacha
authors.forename: John E
authors.initials: JE
authors.lastname: Mayer
authors.forename: David
authors.initials: D
authors.lastname: Zurakowski
authors.forename: Masaki
authors.initials: M
authors.lastname: Otaki
authors.forename: Pedro J
authors.initials: PJ
authors.lastname: del Nido
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000601:surgery; D006343:Heart Septal Defects / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015993:Life Tables; D008297:Male; D008944:Mitral Valve Insufficiency / Q000150:complications; D011183:Postoperative Complications / Q000401:mortality; D011667:Pulmonary Veins / Q000473:pathology; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D014262:Tricuspid Valve Insufficiency / Q000150:complications