Clinical results of arterial switch operation for double-outlet right ventricle with subpulmonary VSD

Objective: An arterial switch operation is considered a good alternative fo r the repair of double-outlet right ventricle (DORV) with atrioventricular concordance connection and subpulmonary ventricular septal defect (VSD) whe n intraventricular rerouting is not feasible. The clinical results of an ar terial switch operation with ventricular septal defect closure for this ano maly were studied. Methods. Between 1986 and 1997, 27 patients ranging from 10 days to 5 years of age (mean 0.4 years) underwent an arterial switch op eration with ventricular septal defect closure for the correction of double outlet right ventricle with subpulmonary VSD. The 50% rule was used to def ine double-outlet right ventricle. Arch anomalies were associated in nine c ases, and were corrected either previously or simultaneously. A subarterial muscle resection was performed in 14 without any subsequent stenosis of th e ventricular outflow tract. The relationship of the great arteries was mos tly anteroposterior in 15 and mostly side by side in 12. The left coronary artery (main trunk or circumflex artery) courses behind the pulmonary arter y in 15/27 (six/15 in the anteroposterior relation and ten/12 in the side b y side relation). The Lecompte maneuver was used to reconstruct the pulmona ry artery in all but five cases with a side by side relationship of the gre at arteries. Results: There was one operative death (3.7%) and three late d eaths. The actuarial survival rate was 83 +/- 8% at 9 years. Right ventricu lar outflow tract obstruction including peripheral pulmonary stenosis devel oped in seven cases operated on in the early era. The reoperation free rate was 46 +/- 20% at 9 years. Conclusion: Although double-outlet right ventri cle with subpulmonary VSD has complex features, including an aortic arch ob struction and coronary artery anomalies, an optimal definitive surgical rep air using an arterial switch operation can be performed safely with a thoro ugh understanding of this variable anomaly. The prevention of right ventric ular outflow tract obstruction at the time of an arterial switch operation may thus help improve the rate of late morbidity. (C) 1999 Elsevier Science B.V. All rights reserved.