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      Clinical and Morphologic Features of the Congenitally Unicuspid Acommissural Stenotic and Regurgitant Aortic Valve

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          Abstract

          Five adults, aged 30–75 years, are described with stenotic and regurgitant unicuspid acommissural aortic valves. Because none of these patients had clinical, echocardiographic or hemodynamic evidence of mitral valve disease, a case is made that these valves were congenitally malformed and not the result of an acquired condition.

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          Most cited references 8

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          Morphologic features of the normal and abnormal mitral valve.

          Anatomic and functional features of the normal and abnormal mitral valve are reviewed. Of 1,010 personally studied necropsy patients with severe (functional class III or IV, New York Heart Association) cardiac dysfunction from primary valvular heart disease, 434 (43%) had mitral stenosis (MS) with or without mitral regurgitation (MR): unassociated with aortic valve stenosis or regurgitation or with tricuspid valve stenosis in 189 (44%) patients, and associated with aortic stenosis in 152 (35%), with pure (no element of stenosis) aortic regurgitation in 65 (15%) patients, and with tricuspid valve stenosis with or without aortic valve stenosis in 28 (6%) patients. The origin of MS was rheumatic in all 434 patients. Of the 1,010 necropsy patients, 165 (16%) had pure MR (papillary muscle dysfunction excluded): unassociated with aortic valve stenosis or regurgitation or with tricuspid valve stenosis in 97 (59%) patients, and associated with pure aortic regurgitation in 45 (27%) and with aortic valve stenosis in 23 (14%) patients. When associated with dysfunction of the aortic valve, pure MR was usually rheumatic in origin, but when unassociated with aortic valve dysfunction it was usually nonrheumatic in origin. Review of operatively excised mitral valves in patients with pure MR unassociated with aortic valve dysfunction disclosed mitral valve prolapse (most likely an inherent congenital defect) as the most common cause of MR. Excluding the patients with MR from coronary heart disease (papillary muscle dysfunction), mitral prolapse was the cause of MR in 60 (88%) of the other 68 patients, and a rheumatic origin was responsible in only 3 of the 68 patients, all 68 of whom were greater than 30 years of age. Mitral anular calcification in persons aged greater than 65 years is usually associated with calcific deposits in the aortic valve cusps and in the coronary arteries. Because calcium in each of these 3 sites is common in older individuals residing in the Western World, it is most reasonable to view mitral anular calcification in older individuals as a manifestation of atherosclerosis. Mitral anular calcium appears to be extremely uncommon in persons with total serum cholesterol levels less than 150 mg/dl. Mitral anular calcium may produce mild MR and, if the deposits are heavy enough, MS.
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            Weights of operatively-excised stenotic unicuspid, bicuspid, and tricuspid aortic valves and their relation to age, sex, body mass index, and presence or absence of concomitant coronary artery bypass grafting.

            This study was designed to evaluate weights of operatively-excised stenotic aortic valves and to compare them with age, sex, body mass index, and presence or absence of concomitant coronary artery bypass grafting. Weights of operatively-excised stenotic aortic valves have not been previously reported. We weighed operatively-excised stenotic valves in 499 patients (aged 19 to 91 years, mean 70), none of whom had mitral valve replacement. The 499 aortic valves ranged in weight from 0.45 to 11.30 g (mean 2.67). The mean weights of the unicuspid and bicuspid valves were heavier than those of the tricuspid valves (4.36 vs 3.34 vs 2.04 g, p 30 kg/m(2) (2.62 vs 2.76 vs 2.57 g). Weights of operatively-excised stenotic aortic valves provide objective evidence of valvular stenosis.
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              The heart in systemic lupus erythematosus.

               W C Roberts,  S High (1999)
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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
                August 2007
                27 September 2006
                : 108
                : 2
                : 79-81
                Affiliations
                aDepartment of Internal Medicine, Division of Cardiology, bDepartment of Pathology, and cBaylor Heart & Vascular Institute, Baylor University Medical Center, Dallas, Tex., USA
                Article
                95912 Cardiology 2007;108:79–81
                10.1159/000095912
                17008775
                © 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: 14, Pages: 3
                Categories
                Original Research

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