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      The unicuspid aortic valve

      research-article
      , MD, PHD, , MD,, , MD, PHD, , MD,
      Cardiovascular Journal of Africa
      Clinics Cardive Publishing
      aortic dilatation, echocardiography, unicuspid aortic valve

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          Summary

          The unicuspid aortic valve is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Other congenital disorders may accompany this phenomenon and aortic dilatation and left ventricular hypertrophy are frequent complications. We present a case report of a young, symptomatic patient with a unicuspid aortic valve, complicated by dilatation of the aortic root and ascending aorta, with left ventricular hypertrophy. The patient recovered fully after a Bentall procedure.

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          Most cited references12

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          Incidence and echocardiographic features of congenital unicuspid aortic valve in an adult population.

          Although well described in pediatric populations, series of congenital unicuspid aortic valves in adults are limited and mainly arise from surgical reports. Also, the incidence of this rare congenital anomaly has never been estimated in an adult echocardiographic population. Between January 1990 and May 2002, the authors' echocardiographic database was systematically reviewed to identify adult patients with congenital unicuspid aortic valve. Echocardiographic studies were reviewed off-line to define morphologic characteristics and evaluate ascending aortic dimensions. Twenty-one adult patients with congenital unicuspid aortic valve were identified, resulting in an estimated incidence in the referral population of approximately 0.02%. Aortic stenosis was the predominant associated hemodynamic lesion; on average, this required surgical intervention during the third decade of life. All valves were unicommissural with a posteriorly positioned commissural attachment. Of particular clinical importance, coexisting ascending aortic dilatation was found in almost half of the cohort (48%), despite the younger patient age. Adult congenital unicuspid aortic valve is a rare cardiac anomaly, even in a large referral population. On average, patients will present for cardiac surgery in their third decade of life. Aortic dilatation in association with unicuspid aortic valves should be well recognized and routinely assessed in the initial evaluation and follow up of these patients.
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            Implications of a congenitally abnormal valve: a study of 1025 consecutively excised aortic valves.

            An increasing proportion of patients with congenitally abnormal aortic valves (AV) present for AV replacement. To review morphological changes in a large contemporary patient population undergoing AV replacement. A detailed review was conducted for all 1025 patients who underwent AV replacement from 2002 to 2005, including the clinical indication for surgery, the type of native AV disease, the pathological changes observed in each valve and the need for related surgery. Tricuspid (TAV), bicuspid (BAV) and unicuspid (UAV) aortic valves were observed in 64.5%, 31.9% and 3.0% of all patients respectively. A decreased number of cusps was associated with increasing predilection for male gender (83.9%, 73.4%, 59.2% for UAV, BAV, TAV respectively), a younger patient age at surgery (41.6 (14.3), 61.3 (12.8), 67.5 (12.9) years), and an increased occurrence of pathological changes in the cusps, including calcification of both the cusp and the base, ossification and ulceration. UAV and BAV were also associated with increasing replacement of the ascending aorta due to dilatation and aneurysm formation (54.8, 38.8%, 16.6%). The incidence of infective endocarditis and rheumatic heart disease was 3.8% and 11.2% of all excised valves respectively. UAV and BAV were increasingly likely to affect men, fail at an earlier age, and show an increasing incidence of pathological changes in the cusps and ascending aorta than TAV. These results suggest that TAV, BAV and UAV may represent a phenotypic continuum of a similar disease process.
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              Congenital aortic stenosis resulting from a unicommisssural valve. Clinical and anatomic features in twenty-one adult patients.

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                Author and article information

                Contributors
                Journal
                Cardiovasc J Afr
                Cardiovasc J Afr
                TBC
                Cardiovascular Journal of Africa
                Clinics Cardive Publishing
                1995-1892
                1680-0745
                April 2010
                : 21
                : 2
                : 113-114
                Affiliations
                Department of Cardiac and Thoracic Surgery, The Chaim Sheba Medical Center, Tel Hashomer, Israel
                Department of Cardiac and Thoracic Surgery, The Chaim Sheba Medical Center, Tel Hashomer, Israel
                Department of Cardiothoracic Surgery, Jinling Hospital, School of Clinical Medicine, Nanjing University, Nanjing, Jiangsu Province, People’s Republic of China
                Department of Cardiothoracic Surgery, Jinling Hospital, School of Clinical Medicine, Nanjing University, Nanjing, Jiangsu Province, People’s Republic of China
                Article
                5566075
                20532437
                2573e1b5-ee20-45fa-96a9-fbc651dde005
                Copyright © 2015 Clinics Cardive Publishing

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 20 July 2009
                : 13 October 2009
                Categories
                Cardiovascular Topics

                aortic dilatation,echocardiography,unicuspid aortic valve

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