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Left Coronary Artery Stenosis Causing Left Ventricular Dysfunction In Two Children With Supravalvular Aortic Stenosis

Left Coronary Artery Stenosis Causing Left Ventricular Dysfunction In Two Children With Supravalvular Aortic Stenosis

Authors :
Okan Yildiz
Firat H. Altin
Mehmet Gungor Kaya
Ersin Erek
Alper Güzeltaş
Isa Ozyilmaz
Source :
World Journal for Pediatric and Congenital Heart Surgery. 6:311-316
Publication Year :
2015
Publisher :
SAGE Publications, 2015.

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.

Details

ISSN :
2150136X and 21501351
Volume :
6
Database :
OpenAIRE
Journal :
World Journal for Pediatric and Congenital Heart Surgery
Accession number :
edsair.doi.dedup.....4f708dc92b30b984c515d53d9dba5bd1
Full Text :
https://doi.org/10.1177/2150135114563767