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KMID : 0364020160490020115
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Korean Journal of Thoracic and Cardiovascular Surgery
2016 Volume.49 No. 2 p.115 ~ p.118
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Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome
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Lee Jong-Uk
Jang Woo-Sung
Lee Young-Ok
Cho Joon-Yong
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Abstract
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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.
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KEYWORD
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Congenital heart disease, Stenosis, Aorta, Pulmonary artery, Williams syndrome
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