• Wlodzimierz Kuroczynski, Christoph Kampmann, Nalan Kayhan, Markus Heinemann, Diethard Pruefer, and Christian-Friedrich Vahl.
• Department of Cardiothoracic and Vascular Surgery, Johannes Gutenberg-University, Langenbeck-Str. 1, 55131 Mainz, Germany. kuroczynski@uni-mainz.de
• Clin Res Cardiol. 2008 Apr 1;97(4):266-71.

ObjectivesChildren with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) are at risk for myocardial infarction and death. This retrospective study shows the mid-term follow-up after the use of aortic implantation and alternative methods to achieve coronary transfer.MethodsSince 1990 seven consecutive children underwent primary repair of ALCAPA. Age at operation ranged from 2 to 71 months (median 11 months). Operative techniques included ligation (n = 1), intrapulmonary tunnel (n = 1), and aortic implantation (n = 5). One patient with severe mitral valve incompetence underwent additional mitral valve replacement. A 4-month-old patient was successfully treated after the operation with a left heart assist device.ResultsOne death in the series occurred at 2 weeks after intrapulmonary tunneling. The mid-term results were evaluated in the six survivors with a follow-up mean of 98 months (ranged 58-168). In all surviving patients with two-vessel coronary blood supply, left ventricular end-diastolic volume and left ventricular ejection fraction returned to near normal values 2-12 months postoperatively. The mitral valve incompetence decreased in all patients with a native mitral valve. One patient with coronary ligation showed severe mitral valve regurgitation and received additional mitral valve replacement concomitantly. Six years after primary valve replacement of a 21 mm SJM (Saint Jude Medical) a change of the mechanical valve to a 27 mm valve was necessary because of development of severe stenosis due to growth.ConclusionsIt is always preferable to establish an antegrade flow of oxygenated blood through the coronary arteries and to create a two-coronary artery system. Mitral valve annuloplasty or replacement may be necessary for patients with severe mitral valve incompetence.

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