Sporadic supravalvular stenosis in a young man

Sporadic supravalvular stenosis in a young man

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...

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Bibliographic Details
Published in:JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR) Vol. 96; no. 1; pp. 27 - 29
Main Authors: Ghodduci, K M, Antic, M, Tanaka, K, Verdries, D, Kerkhove, D
Format: Journal Article
Language:English
Published: Belgium Ubiquity Press 01-01-2013
Subjects:
Adult
Aorta
Aorta - diagnostic imaging
Aorta - pathology
Aorta - surgery
Aortic Stenosis, Supravalvular - complications
Aortic Stenosis, Supravalvular - diagnosis
Aortic Stenosis, Supravalvular - surgery
Aortography - methods
Diagnosis, Differential
Heart Murmurs - complications
Humans
Magnetic Resonance Imaging - methods
Male
stenosis or obstruction
Tomography, X-Ray Computed - methods
Ultrasonography
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Summary: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.
ISSN:0302-7430
2514-8281
DOI:10.5334/jbr-btr.175