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Congenital supravalvular aortic stenosis is congenital narrowing of the ascending aorta that
may be localized or diffuse, originating at superior margin of the sinuses of Valsalva just
above the level of the coronary arteries.
We have experienced a case of congenital supravalvular aortic stenosis who had characte-
ristic face and mental retardation associated with aortic aneurysm.
We have presented this case and reviewed the literature.
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