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Published on: November 28, 2018
Sporadic supravalvular stenosis in a young man
K M Ghodduci1, M Antic, K Tanaka
1Department of Radiology, UZ Brussel, Laerbeeklaan 101, B-1090 Brussels, Belgium. kghodduci@yahoo.com
Abstract:
Supravalvar aortic stenosis (SVAS) is a form of congenital left ventricular outflow tract (LVOT) obstruction that occurs as a localized or diffuse narrowing of the ascending aorta beyond the superior margin of the sinuses of Valsalva. SVAS is a rare progressive congenital heart defect with a higher risk of sudden cardiac death. Our patient was a 30-year-old man referred by his GP for an incidental finding of heart murmur. Echocardiography showed a hypertrophic left ventricle with an increased pressure gradient. CT and MRI angiography and eventual arteriography confirmed the diagnosis by showing a narrowing of the aorta at the sinotubular junction with significant accelerated flow. This stenosis was eventually surgically corrected with no complications.
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