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Surgery and critical care for anomalous coronary artery from the pulmonary artery

Published online by Cambridge University Press:  01 December 2010

Timothy A. Fehrenbacher ,
Michael E. Mitchell ,
Nancy S. Ghanayem  and
James S. Tweddell
Timothy A. Fehrenbacher
Affiliation:
Division of Cardiothoracic Surgery, Department of Surgery and the Section of Critical Care, Department of Pediatrics, Herma Heart Center, Children’s Hospital of Wisconsin, the Medical College of Wisconsin, Wisconsin, United States of America
Michael E. Mitchell
Affiliation:
Division of Cardiothoracic Surgery, Department of Surgery and the Section of Critical Care, Department of Pediatrics, Herma Heart Center, Children’s Hospital of Wisconsin, the Medical College of Wisconsin, Wisconsin, United States of America
Nancy S. Ghanayem
Affiliation:
Division of Cardiothoracic Surgery, Department of Surgery and the Section of Critical Care, Department of Pediatrics, Herma Heart Center, Children’s Hospital of Wisconsin, the Medical College of Wisconsin, Wisconsin, United States of America
James S. Tweddell*
Affiliation:
Division of Cardiothoracic Surgery, Department of Surgery and the Section of Critical Care, Department of Pediatrics, Herma Heart Center, Children’s Hospital of Wisconsin, the Medical College of Wisconsin, Wisconsin, United States of America
*
Correspondence to: J. S. Tweddell, MD, Children’s Hospital of Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, Wisconsin 53226, United States of America. Tel: 414 266 2638; Fax: 414 266 2075; E-mail: jtweddell@chw.org
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Abstract

Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that accounts for 0.25–0.50% of children with congenital cardiac disease and can cause myocardial dysfunction in young infants. In any infant presenting with ventricular dysfunction, the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery must be suspected and the origin of the coronary arteries must be confirmed. The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery is an indication for surgical repair. A two-coronary arterial system is the goal and is almost always achievable. The goal of surgical therapy is the creation of a two-coronary arterial system, which appears to provide better long-term survival and protection from left ventricular dysfunction and mitral valvar regurgitation than does simple ligation of the anomalous coronary artery. Direct reimplantation of the anomalous coronary artery is the procedure of choice. It is straightforward and borrows from well-practised techniques commonly used in other procedures such as the arterial switch operation. For the rare patient in whom direct reimplantation is not possible, strategies to lengthen the anomalous coronary artery, or baffle it within the pulmonary root, are available. Mitral valvar regurgitation is common at presentation, but following the establishment of a two-coronary arterial system and satisfactory myocardial perfusion, regurgitation of the mitral valve resolves in the vast majority. Therefore, mitral valvuloplasty at the time of initial surgery for anomalous origin of the left coronary artery from the pulmonary artery is not indicated. Post-operative care requires careful manipulation of inotropic support and reduction of afterload. Mechanical support, with either extracorporeal membrane oxygenation or left ventricular assist device, should be available for use if necessary.

Keywords

Congenital cardiac diseasepaediatric cardiac diseaseALCAPAanomalous origin of the left coronary artery from the pulmonary artery
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 35 - 43
Copyright
Copyright © Cambridge University Press 2010

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