Left Coronary Artery Stenosis Causing Left Ventricular Dysfunction In Two Children With Supravalvular Aortic Stenosis
World Journal for Pediatric and Congenital Heart Surgery, cilt.6, sa.2, ss.311-316, 2015 (Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 6 Sayı: 2
- Basım Tarihi: 2015
- Doi Numarası: 10.1177/2150135114563767
- Dergi Adı: World Journal for Pediatric and Congenital Heart Surgery
- Derginin Tarandığı İndeksler: Scopus
- Sayfa Sayıları: ss.311-316
- Anahtar Kelimeler: supravalvar aortic stenosis, left main coronary artery stenosis, myocardial ischemia, revascularization
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
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.