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Fistulous communications with the coronary arteries in the setting of hypoplastic ventricles

Published online by Cambridge University Press:  01 December 2010

Robert H. Anderson  and
Diane Spicer
Robert H. Anderson*
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Medical University of South Carolina, Charleston, South Carolina, United States of America Cardiac Unit, Institute of Child Health, University College, London, United Kingdom
Diane Spicer
Affiliation:
Division of Pediatric Cardiology, University of Florida, Gainesville, Florida, United States of America All Children’s Hospital, Congenital Institute of Florida, St. Petersburg, Florida, United States of America
*
Correspondence to: Professor R. H. Anderson, BSc, MD, FRCPath, 60 Earlsfield Road, London SW18 3DN, United Kingdom. Tel: 00 44 20 8870 4368; E-mail: r.anderson@ich.ucl.ac.uk
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Abstract

Neonates born with hypoplastic left heart syndrome now have a remarkably improved prognosis compared with the situation existing before the development of the Norwood sequence of operative procedures. Some of those born with hypoplastic right ventricles in the setting of pulmonary atresia with an intact ventricular septum, however, still have a relatively poor prognosis. In part this reflects the presence of fistulous communication between the cavity of the right hypoplastic right ventricle and the coronary arterial tree. Such fistulous communications are now increasingly recognised as being important in the setting of hypoplastic left heart syndrome. In this brief review, we describe the anatomy of the communications. Those found with hypoplastic right ventricles are seen most frequently when the cavity of the ventricle effectively represents only the inlet, this in turn reflecting mural overgrowth of the apical trabecular and outlet components during foetal development. This almost certainly reflects an earlier appearance of the pulmonary valvar lesion that promotes the cavitary hypoplasia. In those with hypoplastic left ventricles, the key feature differentiating those with fistulous communications is the presence of a patent mitral valve, since the left ventricle is typically no more than a virtual slit in postero-inferior ventricular wall in the setting of mitral valvar atresia or absence of the left atrioventricular connection.

Keywords

Coronary arterial anomaliespulmonary atresia with intact ventricular septumhypoplastic left heart syndrome
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 86 - 91
Copyright
Copyright © Cambridge University Press 2010

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