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Identification, imaging, functional assessment and management of congenital coronary arterial abnormalities in children

Published online by Cambridge University Press:  26 November 2007

Alan H. Friedman ,
Mark A. Fogel ,
Paul Stephens Jr. ,
Jeffrey C. Hellinger ,
David G. Nykanen ,
James Tweddell ,
Timothy F. Feltes  and
Jonathan J. Rome
Alan H. Friedman*
Affiliation:
Section of Pediatric Cardiology, Yale University and the Yale New Haven Children’s Hospital, New Haven, Connecticut, United States of America
Mark A. Fogel
Affiliation:
Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Paul Stephens Jr.
Affiliation:
Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Jeffrey C. Hellinger
Affiliation:
Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
David G. Nykanen
Affiliation:
Congenital Heart Institute at Arnold Palmer Hospital and Miami Children’s Hospital, Miami, Florida, United States of America
James Tweddell
Affiliation:
Children’s Hospital of Wisconsin, Milwaukee, Wisconsin, United States of America
Timothy F. Feltes
Affiliation:
Ohio State University and Columbus Children’s Hospital, Columbus, Ohio, United States of America
Jonathan J. Rome
Affiliation:
Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: Alan Friedman, MD, Associate Professor of Pediatrics and Associate Chair of Pediatrics, Education, Yale University School of Medicine, 333 Cedar Street, LCI 302, New Haven, CT 06520-8064; Tel: 203 785-2022; Fax: 203 737 2786; E-mail: alan.friedman@yale.edu
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Abstract

The coronary arteries, the vessels through which both substrate and oxygen are provided to the cardiac muscle, normally arise from paired stems, right and left, each arising from a separate and distinct sinus of the aortic valve. The right coronary artery runs through the right atrioventricular groove, terminating in the majority of instances in the inferior interventricular groove. The main stem of the left coronary artery bifurcates into the anterior descending, or interventricular, and the circumflex branches. Origin of the anterior descending and circumflex arteries from separate orifices from the left sinus of Valsalva occurs in about 1% of the population, while it is also frequent to find the infundibular artery arising as a separate branch from the right sinus of Valsalva.

Anomalies of the coronary arteries can result from rudimentary persistence of an embryologic coronary arterial structure, failure of normal development or normal atrophy as part of development, or misplacement of connection of a an otherwise normal coronary artery. Anomalies, therefore, can be summarized in terms of abnormal origin or course, abnormal number of coronary arteries, lack of patency of the orifice of coronary artery, or abnormal connections of the arteries.

Anomalous origin of the left coronary artery from the pulmonary trunk occurs with an incidence of approximately 1 in 300,000 children. The degree of left ventricular dysfunction produced likely relates to the development of collateral vessels that arise from the right coronary artery, and provide flow into the left system. Anomalous origin of either the right or the left coronary artery from the opposite sinus of Valsalva can be relatively innocuous, but if the anomalous artery takes an interarterial course between the pulmonary trunk and the aorta, this can underlie sudden death, almost invariably during or immediately following strenuous exercise or competitive sporting events. Distal anomalies of the coronary arteries most commonly involve abnormal connections, or fistulas, between the right or left coronary arterial systems and a chamber or vessel.

We discuss the current techniques available for imaging these various lesions, along with their functional assessment, concluding with a summary of current strategies for management.

Keywords

Sudden deathmagnetic resonance imagingcomputerized tomography
Type
Original Article
Information
Cardiology in the Young , Volume 17 , Supplement S4 , September 2007 , pp. 56 - 67
Copyright
Copyright © Cambridge University Press 2007

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