title: Left coronary artery stenosis causing left ventricular dysfunction in two children with supravalvular aortic stenosis.
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.
authors: Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey okanyildiz27@hotmail.com.
authors: Okan
authors: O
authors: Yildiz
authors: Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
authors: Firat H
authors: FH
authors: Altin
authors: Department of Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
authors: Mehmet
authors: M
authors: Kaya
authors: Department of Pediatric Cardiology, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
authors: Isa
authors: I
authors: Ozyılmaz
authors: Department of Pediatric Cardiology, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
authors: Alper
authors: A
authors: Guzeltas
authors: Department of Pediatric Cardiovascular Surgery, Istanbul Mehmet AkifErsoy Thoracic & Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.
authors: Ersin
authors: E
authors: Erek
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000151:congenital* / Q000601:surgery; D001021:Aortic Valve / Q000002:abnormalities / Q000601:surgery; D000082882:Bicuspid Aortic Valve Disease; D002675:Child, Preschool; D023921:Coronary Stenosis / Q000150:complications* / Q000601:surgery; D005260:Female; D006349:Heart Valve Diseases / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities; D011666:Pulmonary Valve Stenosis / Q000151:congenital; D018487:Ventricular Dysfunction, Left / Q000209:etiology* / Q000601:surgery; D018980:Williams Syndrome / Q000150:complications
keywords: left main coronary artery stenosis; myocardial ischemia; revascularization; supravalvar aortic stenosis