title: Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle.
abstract: Surgical management of various forms of double-outlet right ventricle uses a variety of approaches depending on the underlying anatomic form. In this study, we sought to determine the risk factors of mortality and reoperation in those with double-outlet right ventricle undergoing biventricular repair, according to anatomic characteristics and initial surgical strategy.
  
  
  
  Between 1992 and 2013, 433 patients were included in the study. Double-outlet right ventricle was classified as double-outlet right ventricle with subaortic ventricular septal defect associated with subpulmonary obstruction in 33% of patients (n = 141), with subaortic ventricular septal defect without subpulmonary obstruction in 30% of patients (n = 130), with subpulmonary ventricular septal defect in 32% of patients (n = 139), and with noncommitted ventricular septal defect in 5% of patients (n = 23). Three types of repairs were performed: (1) intraventricular baffle repair, n = 149 (34%); (2) intraventricular baffle repair with right ventricular outflow tract reconstruction, n = 163 (38%); and (3) intraventricular baffle repair with arterial switch operation, n = 121 (28%).
  
  
  
  Thirty-day overall mortality was 7.4%. Early reoperation was needed in 6% of the cases. Early mortality was higher in the intraventricular baffle repair with arterial switch operation group (P = .01). Survival at 10 years was 86.2%, and freedom from reoperation at 10 years was 61.4%. At last follow-up (median, 5.7 years; 95% confidence interval, 4.5-6.6), mortality and reoperation rates were similar in the different surgical strategy groups. Late reoperation and late mortality were significantly higher in the double-outlet right ventricle with noncommitted ventricular septal defect group (P < .01). In multivariate analyses, risk factors for reoperation were concomitant surgical procedures (P = .03) and duration of cardiopulmonary bypass (P < .01). Risk factors for mortality were restrictive ventricular septal defect (P = .01), mitral cleft (P < .01), and associated coronary artery anomalies (P = .01).
  
  
  
  Those with the anatomic type of double-outlet right ventricle with noncommitted ventricular septal defect were at higher risk for reoperation and mortality. Intraventricular baffle repair with arterial switch operation was the surgical strategy in patients at higher risk of early death. Initial surgical strategy did not influence the late outcomes.
authors.affiliation: M3C-Necker Enfants Malades, AP-HP, Université Paris Descartes, Sorbonne Paris Cité, Paris, France. Electronic address: olivier.villemain@inserm.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Villemain
authors.affiliation: Centre Chirurgical Marie Lannelongue, M3C, Université Paris Sud, Le Plessis Robinson, France.
authors.forename: Emre
authors.initials: E
authors.lastname: Belli
authors.affiliation: M3C-Necker Enfants Malades, AP-HP, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
authors.forename: Magalie
authors.initials: M
authors.lastname: Ladouceur
authors.affiliation: Centre Chirurgical Marie Lannelongue, M3C, Université Paris Sud, Le Plessis Robinson, France.
authors.forename: Lucile
authors.initials: L
authors.lastname: Houyel
authors.affiliation: M3C-Necker Enfants Malades, AP-HP, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
authors.forename: Zakaria
authors.initials: Z
authors.lastname: Jalal
authors.affiliation: Pediatric Department, University Hospital of Bicetre, le Kremlin Bicetre, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Lambert
authors.affiliation: Centre Chirurgical Marie Lannelongue, M3C, Université Paris Sud, Le Plessis Robinson, France.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Ly
authors.affiliation: M3C-Necker Enfants Malades, AP-HP, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Vouhé
authors.affiliation: M3C-Necker Enfants Malades, AP-HP, Université Paris Descartes, Sorbonne Paris Cité, Paris, France.
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
mesh_terms: D063405:Anatomic Variation; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000145:classification / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D005260:Female; D005602:France; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012086:Reoperation / Q000706:statistics & numerical data; D015996:Survival Rate; D016896:Treatment Outcome
keywords: congenital heart disease; double-outlet right ventricle; outcome; pediatrics; surgery