Anomalous origin of the left coronary artery from the pulmonary artery: late results with special attention to the mitral valve.
OBJECTIVE
Evaluate 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).


METHODS
Between 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.


RESULTS
There 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).
Department of Pediatric Cardiac Surgery, University Paris Descartes and Sick Children Hospital, Paris, France.
Walid
W
Ben Ali
Olivier
O
Metton
François
F
Roubertie
Philippe
P
Pouard
Daniel
D
Sidi
Olivier
O
Raisky
Pascal R
PR
Vouhé
D002648:Child; D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D004812:Epidemiologic Methods; D005260:Female; D006353:Heart-Assist Devices; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008944:Mitral Valve Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D011379:Prognosis; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery; D012086:Reoperation; D013318:Stroke Volume; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000209:etiology / Q000628:therapy; D016277:Ventricular Function, Left