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J Thorac Cardiovasc Surg 2007;134:967-973
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle

^a Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
^b Division of Cardiovascular Surgery, Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Received for publication September 17, 2006; revisions received April 29, 2007; accepted for publication May 22, 2007.

^_* Address for reprints: Brian W. McCrindle, MD, MPH, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8. (Email: brian.mccrindle{at}sickkids.ca).

Objective: We sought to define the prevalence of definitive end-states and their determinants in children with double-outlet right ventricle.

Methods: We performed a clinical record review of 393 children with double-outlet right ventricle presenting to our institution from 1980 to 2000.

Results: Double-outlet right ventricle classification was as follows: subaortic ventricular septal defect with or without pulmonary stenosis in 47%, subpulmonic ventricular septal defect in 23%, noncommitted ventricular septal defect in 26%, and doubly committed ventricular septal defect in 4%. Hypoplastic ventricles were present in 39%, pulmonary stenosis was present in 65%, and aortic arch obstruction was present in 24%. Biventricular repair was performed in 194 patients (55%) at a median age of 10 months (range: birth to 14.0 years), and the Fontan operation (n = 182; 23%) was performed at a median age of 3.7 years (range: 6 months to 14.9 years). Results improved over time (P < .001). Factors discriminating among end-states included younger patient age at presentation (P < .001), lower weight (P < .001), and adequacy of left-sided heart structures, especially the size of the left ventricle (P < .001), aortic arch (P < .001), and mitral valve (P = .004). For complex double-outlet right ventricle, Rastelli-type repair increased early reintervention risk (P = .04) and late post-repair mortality (P = .02), whereas the arterial switch operation increased early post-repair mortality (P = .02) with a benefit of improved late post-repair survival.

Conclusions: Biventricular repair, especially Rastelli-type reconstruction, is associated with higher late mortality and reintervention than is Fontan repair. The wisdom of extending biventricular repair to borderline anatomic candidates with hypoplastic left-sided structures or a nonsubaortic ventricular septal defect is questionable.