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      Decreased Left Ventricular Coronary Artery Density in Pulmonary Atresia and Intact Ventricular Septum

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          Abstract

          Background: The prognosis for pulmonary atresia and intact ventricular septum (PAIVS) has been poor. Our hypothesis is that intrinsic abnormal left ventricular (LV) intramyocardial circulation might be related to the poor outcomes of these patients. Methods:Neonatal heart specimens were examined microscopically in four groups of 6 cases each. Group I had PAIVS with ventriculocoronary artery connections (VCAC), group II had PAIVS without VCAC, group III had normal hearts, and group IV had LV hypertrophy. A projection microscope with grid overlay was used to count the LV intramyocardial coronary artery density (IMCAD), which was expressed as the number of profiles/mm<sup>2</sup>. Results: The LV IMCAD of groups I (0.40 ± 0.14/mm<sup>2</sup>) and II (0.45 ± 0.15/mm<sup>2</sup>) were significantly lower than those of groups III (0.77 ± 0.11/mm<sup>2</sup>) and IV (0.76 ± 0.09/mm<sup>2</sup>; all with p = 0.002). There was no significant difference between either groups I and II (p = 0.394) or groups III and IV (p = 0.818). Conclusions: This study demonstrates lower LV IMCAD in a widely heterogeneous spectrum of neonatal hearts with PAIVS, which might potentially predispose these patients to myocardial ischemia and in turn contribute to the poor prognosis of this disease.

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          Most cited references 13

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          Experience with one and a half ventricle repair.

          This article presents a 10-year experience with one and a half ventricle repair for right ventricular hypoplasia or dysfunction.
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            Long-term results in neonates with pulmonary atresia and intact ventricular septum.

            Our entire institutional experience with pulmonary atresia and intact ventricular septum (1965 through 1987) included 115 patients, 16 of whom died before surgical intervention. Fifty-six percent of surgical patients (n = 99) had angiographic evidence of right ventricle-coronary arterial connections. The early mortality in the surgical group was 27.2%, and the actuarial survival was 24.7% +/- 6% at 13 years postoperatively. Multivariate analysis indicated that the presence of ventriculocoronary connections (p = 0.037), a decreasing ratio between right ventricular and left ventricular pressure at the initial cardiac catheterization (p = 0.007), and lower weight at operation (p = 0.001) were incremental risk factors for postoperative death; the presence of Ebstein's anomaly was an additional risk factor in the overall experience (including patients not surgically treated) (p = 0.01). Nearly all long-term survivors underwent at least one reoperation, including right ventricular outflow tract reconstruction (n = 39) and thromboexclusion of the right ventricle (n = 9). The presence of severe stenosis or interruption of the proximal left anterior descending coronary artery system was a uniformly lethal risk factor for patients undergoing these procedures (p = 0.0003). We conclude that surgical procedures that successfully decompress the right ventricle will usually result in biventricular circulation in and long-term survival of patients with pulmonary atresia with intact ventricular septum not complicated by Ebstein's anomaly or extensive ventriculocoronary connections. Decompression or thromboexclusion of the right ventricle is contraindicated in patients with ventriculocoronary connections and a right ventricle-dependent coronary circulation.
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              The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum.

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                Author and article information

                Journal
                CRD
                Cardiology
                10.1159/issn.0008-6312
                Cardiology
                S. Karger AG
                0008-6312
                1421-9751
                2008
                December 2007
                10 July 2007
                : 109
                : 1
                : 10-14
                Affiliations
                aDepartment of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Chang Gung Children’s Hospital, Taoyuan, Taiwan, ROC; Departments of bPediatric Laboratory Medicine and Pathology, and cPediatric Cardiology, Hospital for Sick Children, and Departments of bLaboratory Medicine and Pathology, and cPediatrics, Faculty of Medicine, University of Toronto, Toronto, Ont., Canada
                Article
                105321 Cardiology 2008;109:10–14
                10.1159/000105321
                17627104
                © 2007 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Page count
                Figures: 1, Tables: 1, References: 24, Pages: 5
                Categories
                Original Research

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