Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome

Williams syndrome (WS) is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPAS) are common vascular abnormalities in WS....

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Bibliographic Details
Published inThe Korean journal of thoracic and cardiovascular surgery Vol. 49; no. 2; pp. 115 - 118
Main Authors Lee, Jong Uk, Jang, Woo Sung, Lee, Young Ok, Cho, Joon Yong
Format Journal Article
LanguageKorean
Published 2016
Subjects
Stenosis
Aorta
Congenital heart disease
Pulmonary artery
Williams syndrome
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Summary:Williams syndrome (WS) is a developmental disorder characterized by vascular abnormalities such as thickening of the vascular media layer in medium- and large-sized arteries. Supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPAS) are common vascular abnormalities in WS. The natural course of SVAS and PPAS is variable, and the timing of surgery or intervention is determined according to the progression of vascular stenosis. In our patient, SVAS and PPAS showed rapid concurrent progression within two weeks after birth. We report the early manifestation of SVAS and PPAS in the neonatal period and describe the surgical treatment for stenosis relief.
Bibliography:KISTI1.1003/JNL.JAKO201612460480380
ISSN:2233-601X
2093-6516