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      Unicuspid Aortic Valve in Infant

      case-report

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          Abstract

          The unicuspid aortic valve (UAV) is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Here, we present a case of UAV with moderate aortic stenosis detected by transthoracic echocardiography in the infant.

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          Most cited references9

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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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            Congenital aortic stenosis resulting from a unicommisssural valve. Clinical and anatomic features in twenty-one adult patients.

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              Understanding the structure of the unicuspid and unicommissural aortic valve.

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                Author and article information

                Journal
                J Cardiovasc Ecogr
                J Cardiovasc Ecogr
                JCE
                Journal of Cardiovascular Echography
                Medknow Publications & Media Pvt Ltd (India )
                2211-4122
                2347-193X
                Jul-Sep 2015
                : 25
                : 3
                : 80-82
                Affiliations
                [1] Department of Pediatrics, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha, Maharashtra, India
                Author notes
                Address for correspondence Dr. Amar M. Taksande, Department of Pediatrics, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha - 442 102, Maharashtra, India. E-mail: amar.taksande@ 123456gmail.com
                Article
                JCE-25-80
                10.4103/2211-4122.166079
                5353412
                2e153746-03ca-477e-80ef-11592fe168d2
                Copyright: © 2015 Journal of Cardiovascular Echography

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Categories
                Case Report

                echocardiography,infant,unicuspid aortic valve
                echocardiography, infant, unicuspid aortic valve

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