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      Left coronary artery stenosis causing left ventricular dysfunction in two children with supravalvular aortic stenosis.

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          Abstract

          Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome (WBS) and other isolated elastin gene deletions. Cardiovascular manifestations associated with WBS are characterized by obstructive arterial lesions such as SVAS and pulmonary artery stenosis in addition to bicuspid aortic valve and mitral valve prolapse. However, coronary artery ostial stenosis may be associated with SVAS, and it increases the risk of sudden death and may complicate surgical management. In this report, we present our experience with two patients having SVAS and left coronary artery ostial stenosis with associated left ventricular dysfunction.

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          Author and article information

          Journal
          World J Pediatr Congenit Heart Surg
          World journal for pediatric & congenital heart surgery
          SAGE Publications
          2150-136X
          2150-1351
          Apr 2015
          : 6
          : 2
          Affiliations
          [1 ] Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey okanyildiz27@hotmail.com.
          [2 ] Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
          [3 ] Department of Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
          [4 ] Department of Pediatric Cardiology, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
          Article
          6/2/311
          10.1177/2150135114563767
          25870356
          f5b4370b-4fbd-451d-a99e-f3e75f16774f
          History

          left main coronary artery stenosis,myocardial ischemia,revascularization,supravalvar aortic stenosis

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