Korean J Thorac Cardiovasc Surg.  2016 Apr;49(2):115-118. 10.5090/kjtcs.2016.49.1.115.

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

• Lee JU
• Jang WS
• Lee YO
• Cho JY

• KMID: 2161807
• DOI: http://doi.org/10.5090/kjtcs.2016.49.1.115

Abstract

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.

Keyword

Congenital heart disease; Stenosis; Aorta; Pulmonary artery; Williams syndrome

MeSH Terms

Aorta
Aortic Stenosis, Supravalvular
Arteries
Constriction, Pathologic*
Heart Defects, Congenital
Humans
Parturition
Pulmonary Artery*
Tunica Media
Williams Syndrome*