title: Sporadic supravalvular stenosis in a young man.
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.
authors.affiliation: Department of Radiology, UZ Brussel, Laerbeeklaan 101, B-1090 Brussels, Belgium. kghodduci@yahoo.com
authors.forename: K M
authors.initials: KM
authors.lastname: Ghodduci
authors.forename: M
authors.initials: M
authors.lastname: Antic
authors.forename: K
authors.initials: K
authors.lastname: Tanaka
authors.forename: D
authors.initials: D
authors.lastname: Verdries
authors.forename: D
authors.initials: D
authors.lastname: Kerkhove
mesh_terms: D000328:Adult; D001011:Aorta / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D021921:Aortic Stenosis, Supravalvular / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D001027:Aortography / Q000379:methods; D003937:Diagnosis, Differential; D006337:Heart Murmurs / Q000150:complications; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D014057:Tomography, X-Ray Computed / Q000379:methods; D014463:Ultrasonography