• Walid Ben Ali, Olivier Metton, François Roubertie, Philippe Pouard, Daniel Sidi, Olivier Raisky, and Pascal R Vouhé.
• Department of Pediatric Cardiac Surgery, University Paris Descartes and Sick Children Hospital, Paris, France.
• Eur J Cardiothorac Surg. 2009 Aug 1;36(2):244-8; discussion 248-9.

ObjectiveEvaluate the late results of a uniform approach to the surgical management of children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).MethodsBetween 1986 and 2007, 62 children with ALCAPA underwent surgery. The median age at operation was 16 months (range 10 days to 11 years). A uniform approach was applied, including (1) immediate surgery as soon as the diagnosis was established, (2) direct aortic reimplantation of the anomalous artery, when technically feasible (61/62, 98%), and (3) no concomitant mitral valve surgery, regardless of the severity of mitral regurgitation (59/62, 95%). The mean follow-up was 9.7 years (range 3 months to 21 years) and was 98% complete.ResultsThere were six hospital deaths (9.7%). Left ventricular assistance was used in four patients; two died of related complications. The poor left ventricular ejection fraction was an incremental risk factor for early mortality (p = 0.043); severity of mitral regurgitation was not. There were two late deaths, yielding an actuarial survival rate of 86% at 15 years. Five patients underwent reoperation (mitral valve repair in three, coronary procedure in two); the actuarial freedom from reoperation was 89% at 15 years. Left ventricular function recovered in all survivors. In the 50 late survivors who did not undergo mitral surgery at initial operation, the severity of mitral regurgitation decreased in 58%, remained unchanged in 40% (of which 3 patients underwent reoperation for mitral valve repair) and worsened in 2%; at last follow-up, mitral regurgitation was absent or trivial in 42%, mild in 50%, moderate in 8% and severe in 0%.Conclusions(1) Early mortality is related to the severity of preoperative left ventricular dysfunction; it may be reduced by a careful use of postoperative cardiac support techniques. (2) Late results are satisfactory and left ventricular function always recovers. (3) Mitral regurgitation improves along with left ventricular function, but recovery may be incomplete and need reoperation. The data suggest that mitral valve surgery is probably not indicated at initial surgery, except in selected cases with a low potential of recovery (severe regurgitation with relatively well-preserved left ventricular function).

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