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Pulmonary atresia with intact ventricular septum: management of the coronary arterial anomalies

Published online by Cambridge University Press:  19 August 2008

Umesh Dyamenahalli ,
Brian D. Hanna  and
Geoffrey P. Sharratt
Umesh Dyamenahalli
Affiliation:
Departments of Pediatrics and Cardiology, Izaac Walton Killam Hospital for Children and Dalhousie University, Halifax, Nova Scotia, Canada
Brian D. Hanna
Affiliation:
Departments of Pediatrics and Cardiology, Izaac Walton Killam Hospital for Children and Dalhousie University, Halifax, Nova Scotia, Canada
Geoffrey P. Sharratt*
Affiliation:
Departments of Pediatrics and Cardiology, Izaac Walton Killam Hospital for Children and Dalhousie University, Halifax, Nova Scotia, Canada
*
G.P. Sharratt, Department of Cardiology, IWK Childrens Hospital, 5850 University Avenue, Halifax, N.S. Canada B3J 3G9 Tel: 902 428 8524. Fax: 902 425 2481
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Abstract

Over a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.

Keywords

Pulmonary atresia with intact septumcoronary arterial anomaliescoil embolizationcongenital heart diseasecardiac catheterizationcoronary arterial orificial atresia
Type
Original Articles
Information
Cardiology in the Young , Volume 7 , Issue 1 , January 1997 , pp. 80 - 87
Copyright
Copyright © Cambridge University Press 1997

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