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KMID : 0364019900230061251
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Korean Journal of Thoracic and Cardiovascular Surgery
1990 Volume.23 No. 6 p.1251 ~ p.1255
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Williams Syndrome
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김영삼/Kim, Young Sam
강정수/김철훈/박영환/한영숙/Kang, Jung Soo/Kim, Chul Hoon/Park, Young Hwan/Han, Young Sook
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Abstract
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Supravalvular aortic stenosis is a congenital narrowing of the ascending aorta that may be localized or diffuse, originating at the superior margin of the sinus of Valsalva just above the level of the orifices of the coronary arteries. It is helpful to classify patients according to Heir clinical presentation into
A) nonfa nilial : sporadic case, normal facies and intelligence ;
B) familial : normal facies and intelligence ;
C) nonfamilial syndrome with abnormal facial appearance and mental retardation.
Recently, we experienced a boy with type C characteristics of this syndrome and surgical correction which was consisted of a vertical incision across narrowed segment of the aorta, with the insertion of a prosthetic, Goretex patch, was performed successfully.
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KEYWORD
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