title: Surgical outcome of double-outlet right ventricle with subpulmonary VSD.
abstract: BACKGROUND
  Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial. We reviewed our 7-year experience with patients who had this anatomic configuration.
  
  
  METHODS
  Between January 1992 and January 1999, 20 patients underwent an arterial switch operation (ASO group), and 12 underwent a bidirectional Glenn procedure followed by a modified Fontan in 10 (Glenn/Fontan). Mean follow-up was 23 +/- 18 months.
  
  
  RESULTS
  An initial palliative operation was done in 19 patients (9 in the ASO group, 10 in the Glenn/Fontan group). There were no deaths in the Glenn/Fontan group. Four patients in the ASO group died within 33 days postoperatively. Two of them had a single coronary artery, 1 had a straddling mitral valve, 1 had a hypoplastic aortic arch, and 1 had multiple ventricular septal defects. Three patients had reoperation for subaortic stenosis (n = 2) or pulmonary stenosis (n = 1) after the ASO. Four patients (3 in the ASO group, 1 in the Glenn/Fontan) required a pacemaker for postoperative complete atrioventricular block. Actuarial survival at 5 years for the entire group was 87% (70% confidence interval, 81% to 93%).
  
  
  CONCLUSIONS
  The ASO remains our preferred treatment for infants with double-outlet right ventricle and subpulmonary ventricular septal defect. However, associated anatomic defects are important risk factors.
authors.affiliation: Department of Cardiac Surgery, The Children's Hospital, Boston, Massachusetts 02115, USA.
authors.forename: K
authors.initials: K
authors.lastname: Takeuchi
authors.forename: F X
authors.initials: FX
authors.lastname: McGowan
authors.forename: A M
authors.initials: AM
authors.lastname: Moran
authors.forename: D
authors.initials: D
authors.lastname: Zurakowski
authors.forename: J E
authors.initials: JE
authors.lastname: Mayer
authors.forename: R A
authors.initials: RA
authors.lastname: Jonas
authors.forename: P J
authors.initials: PJ
authors.lastname: del Nido
mesh_terms: D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000150:complications / Q000401:mortality / Q000601:surgery*; D018729:Fontan Procedure / Q000379:methods; D018610:Heart Bypass, Right / Q000401:mortality; D006345:Heart Septal Defects, Ventricular / Q000150:complications*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis