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      Congenital Absence of the Right Pulmonary Artery with Coronary Collaterals Supplying the Affected Lung: Effect on Coronary Perfusion

      a , a , b

      Cardiology

      S. Karger AG

      Pulmonary artery agenesis, Coronary shunt, Coronary perfusion

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          Abstract

          We describe a unique case of congenital absence of a right pulmonary artery presenting in a patient of advanced age and initially misdiagnosed as coronary artery disease. Perfusion of the affected lung was accomplished via anomalous collaterals from right and left circumflex coronary arteries which induced myocardial ischemia, as demonstrated by myocardial perfusion scan. To our knowledge there are only three reports in the international literature, describing unilateral pulmonary artery agenesis with the coronary artery supplying the abnormal lung. All these reports described that the existence of such vessels does not affect the myocardial perfusion. However, here we describe, to our knowledge, for the first time that in a patient with unilateral pulmonary artery agenesis, the existence of collaterals from the coronary arteries to the affected lung can actually have a negative effect in myocardial perfusion and can induce myocardial ischemia. In conclusion, clinicians should be aware of the possibility of undiagnosed cases of unilateral pulmonary artery agenesis presenting with chest pain in advanced age.

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          Most cited references 12

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          The varied manifestation of pulmonary artery agenesis in adulthood.

          Unilateral pulmonary artery agenesis (UPAA), a rare congenital anomaly that is frequently associated with other cardiovascular abnormalities, is usually diagnosed in childhood. Most patients who have no associated cardiac anomalies have only minor or absent symptoms and survive into adulthood. The conditions of such patients are frequently misdiagnosed in adulthood. In this report, we describe six patients with UPAA in whom the diagnosis was first established in adulthood. The varied clinical presentation of these patients is reviewed and the relative effectiveness of a variety of diagnostic tests is compared. During the period January 1987 through December 1990, six male patients, aged 17 to 20 years, were found to have UPAA at the time of their medical screening for enrollment into the armed forces. The diagnosis was based on history, clinical and imaging examinations, including chest radiography, ventilation-perfusion lung scan, digital subtraction angiography (DSA), computed tomography (CT), and magnetic resonance imaging (MRI). In four of the patients, the UPAA was on the left side and in two it was on the right. A right aortic arch was present in three patients and other cardiovascular anomalies were found in three. Pulmonary function studies showed a mild restrictive pattern in four. In contrast to previous reports, the ventilation scan showed a diminished "wash in" and "equilibrium" phase without a delayed "wash out" phase on the affected side in all patients. Selective bronchography through the fiberoptic bronchoscope revealed ipsilateral mixed-type bronchiectasis in two of four patients studied, a finding of clinical significance that has not been described previously. In all cases, the diagnosis was made by DSA. CT of the thorax (n = 6) and MRI (n = 4) were diagnostic in all cases in which they were performed, but added no significant information. UPAA is frequently misdiagnosed in adulthood and is often not considered in the differential diagnosis of the unilateral hyperlucent lung. Clinicians and radiologists should be aware of the possibility of undiagnosed cases in adults, with many atypical characteristics.
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            Absent right pulmonary artery with coronary collaterals supplying the affected lung.

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              Isolated Unilateral Absence of a Pulmonary Artery

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                Author and article information

                Journal
                CRD
                Cardiology
                10.1159/issn.0008-6312
                Cardiology
                S. Karger AG
                0008-6312
                1421-9751
                2007
                November 2007
                09 February 2007
                : 108
                : 4
                : 314-316
                Affiliations
                Departments of Cardiology, aGebze State Hospital and bIstanbul University, Cerrahpasa School of Medicine, Istanbul, Turkey
                Article
                99101 Cardiology 2007;108:314–316
                10.1159/000099101
                17290102
                © 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: 3, References: 19, Pages: 3
                Categories
                Case Report

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