Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle.
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.
Bradley
Timothy J
TJ
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Karamlou
Tara
T
Kulik
Alex
A
Mitrovic
Bojana
B
Vigneswaran
Trisha
T
Jaffer
Salima
S
Glasgow
Patrick D
PD
Williams
William G
WG
Van Arsdell
Glen S
GS
McCrindle
Brian W
BW
D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D004310:Double Outlet Right Ventricle / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012044:Regression Analysis; D012086:Reoperation; D012307:Risk Factors; D018709:Statistics, Nonparametric; D016019:Survival Analysis