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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
Supravalvar aortic stenosis (SVAS), a rare congenital heart defect, can cause left ventricular outflow tract obstruction. Surgical correction of this ascending aorta narrowing proved successful in a 30-year-old patient, resolving the stenosis without complications.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Vascular Surgery
Background:
- Supravalvar aortic stenosis (SVAS) is a rare congenital heart defect.
- It causes narrowing of the ascending aorta, leading to left ventricular outflow tract obstruction.
- SVAS carries an increased risk of sudden cardiac death.
Observation:
- A 30-year-old man presented with an incidental heart murmur.
- Echocardiography revealed left ventricular hypertrophy and an elevated pressure gradient.
- Advanced imaging (CT, MRI, arteriography) confirmed aortic narrowing at the sinotubular junction.
Findings:
- Diagnosis of supravalvar aortic stenosis was confirmed.
- Significant accelerated blood flow was noted due to the stenosis.
- The patient underwent successful surgical correction of the aortic stenosis.
Implications:
- Surgical intervention can effectively treat supravalvar aortic stenosis.
- Timely diagnosis and treatment can prevent severe cardiac events.
- This case highlights the importance of investigating heart murmurs, even when incidental.
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