Left coronary artery stenosis causing left ventricular

World J Pediatr Congenit Heart Surg · Apr 2015

Case Reports

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

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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- Okan Yildiz, Firat H Altin, Mehmet Kaya, Isa Ozyılmaz, Alper Guzeltas, and Ersin Erek.
- Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey okanyildiz27@hotmail.com.
- World J Pediatr Congenit Heart Surg. 2015 Apr 1; 6 (2): 311-6.
AbstractCongenital 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. © The Author(s) 2014.

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

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