The supravalvular aortic stenosis is a rare congenital heart defect being very
uncommon in adults. We present a case of supravalvular aortic stenosis in adult
associated with anomalies of the aortic arch vessels and aortic regurgitation, which
was submitted to aortic valve replacement and arterioplasty of the ascending aorta
with a good postoperative course.
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