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J Thorac Cardiovasc Surg 2011;142:868-874
© 2011 The American Association for Thoracic Surgery


Congenital Heart Disease

Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children

Vladimir Alexi-Meskishvili, MD, PhDa,*, Boris A. Nasseri, MDa, Sarah Nordmeyer, MDb, Boris Schmitt, MDb, Yu-Guo Weng, MDa, Wolfgang Böttcher, ECCPa, Michael Hübler, MDa, Felix Berger, MD, PhDc, Roland Hetzer, MD, PhDa

a Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
b Department of Pediatric Cardiology, Deutsches Herzzentrum Berlin, Berlin, Germany
c Congenital Heart Diseases, Deutsches Herzzentrum Berlin, Berlin, Germany

Received for publication December 28, 2010; revisions received March 22, 2011; accepted for publication April 8, 2011.

* Address for reprints: Vladimir Alexi-Meskishvili, MD, PhD, Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany. (Email: alexi{at}dhzb.de).

Objective: Although mortality after direct aortic reimplantation for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has significantly decreased, many questions remain unanswered.

Methods: Between 1986 and June 2010, we operated on 27 consecutive pediatric patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). All patients underwent reestablishment of a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta. Postoperative extracorporeal mechanical circulatory support was necessary in 7 cases. In all 7 patients, hemodynamic stability was achieved after 4 to 10 days of support. Mitral valve repair was performed in 9 patients with severe mitral valve incompetence and resulted in stable mitral valve function during follow-up as long as 19 years.

Results: There were no early or late deaths. During follow-up (3 months–17.5 years), both early and late improvement of myocardial function was observed in all patients. Reduced left ventricular regional function late after successful surgical correction of ALCAPA was related to the presence of left ventricular myocardial scar tissue, as detected by magnetic resonance imaging.

Conclusions: Despite the absence of early and late mortality, the long-term prognosis for patients after reimplantation of ALCAPA into the aorta is not clear. Scars and perfusion deficits of the left ventricle may not be detected by standard echocardiographic evaluation of global left ventricular function and therefore may be underestimated. We therefore recommend lifelong surveillance of these patients, including magnetic resonance imaging.





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