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      Unicuspid Aortic Valve Presenting with Decompensated Critical Aortic Stenosis

      case-report
      Author(s): , MD, , BS, RDCS (AE PE), , MD
      Publication date (Electronic):
      Journal: CASE : Cardiovascular Imaging Case Reports
      Publisher: Elsevier
      Keywords: Unicuspid, Aortic valve, Aortic stenosis, Unicommisural

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          Highlights

          • •

            UAV is a rare congenital anomaly that leads to severe symptomatic stenosis.

          • •

            Echocardiography plays a critical role in the evaluation of aortic stenosis.

          • •

            Correctly distinguishing between UAV and BAV is relevant in determining intervention.

          Graphical abstract

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          Most cited references15

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          Bicuspid aortic valve aortopathy in adults: Incidence, etiology, and clinical significance.

          Siddharth Prakash, Hector Michelena, Alessandro Della Corte … (2015)
          Bicuspid aortic valve is the most common congenital heart defect and is associated with an aortopathy manifested by dilatation of the ascending thoracic aorta. The clinical consequences of this aortopathy are the need for periodic monitoring of aortic diameters, elective prophylactic surgical aortic repair, and the occurrence of aortic dissection or rupture. This review describes the current knowledge of BAV aortopathy in adults, including incidence, pathophysiologic insights into its etiology, contemporary hypothesis-generating observations into its complications, and recommendations for monitoring and intervention.
          Article 0 comments Cited 38 times – based on 0 reviews     Review now
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            New insights in the association between bicuspid aortic valve phenotype, aortic configuration and valve haemodynamics.

            Efstratios I Charitos, Rebecca Hachmann, Hans-Hinrich Sievers … (2016)
            Patients with a bicuspid aortic valve (BAV) present with various phenotypes of the valve, the aortic root (AoR) and/or the ascending aorta (AAo) and various valve haemodynamics (vHs). The aim of the present study was to investigate the association between the above parameters.
            Article 0 comments Cited 20 times – based on 0 reviews     Review now
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              Incidence and echocardiographic features of congenital unicuspid aortic valve in an adult population.

              M. Mishra, Brian P. Griffin, Gian M. Novaro (2003)
              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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                Author and article information

                Journal
                Journal ID (nlm-ta): CASE (Phila)
                Journal ID (iso-abbrev): CASE (Phila)
                Title: CASE : Cardiovascular Imaging Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 2468-6441
                Publication date PMC-release: 18 June 2018
                Publication date Collection: August 2018
                Publication date (Electronic): 18 June 2018
                Volume: 2
                Issue: 4
                Pages: 129-134
                Affiliations
                [1]Division of Cardiology, Johns Hopkins Hospital, Baltimore, Maryland
                Article
                Publisher ID: S2468-6441(17)30196-2
                DOI: 10.1016/j.case.2018.04.002
                PMC ID: 6098169
                SO-VID: 26e72cab-5b00-4319-b834-3de0ec3c408b
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Categories
                Subject: Calcification Consequence

                Keywords: unicuspid,aortic valve,aortic stenosis,unicommisural
                Data availability:
                Keywords: unicuspid, aortic valve, aortic stenosis, unicommisural

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