Early Manifestation of Supravalvular Aortic and Pulmonary Artery Stenosis in a Patient with Williams Syndrome.
10.5090/kjtcs.2016.49.1.115
- Author:
Jong Uk LEE
1
;
Woo Sung JANG
;
Young Ok LEE
;
Joon Yong CHO
Author Information
1. Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital, Kyungpook National University School of Medicine, Korea. whiteuri@hanmail.net
- Publication Type:Case Report
- Keywords:
Congenital heart disease;
Stenosis;
Aorta;
Pulmonary artery;
Williams syndrome
- MeSH:
Aorta;
Aortic Stenosis, Supravalvular;
Arteries;
Constriction, Pathologic*;
Heart Defects, Congenital;
Humans;
Parturition;
Pulmonary Artery*;
Tunica Media;
Williams Syndrome*
- From:The Korean Journal of Thoracic and Cardiovascular Surgery
2016;49(2):115-118
- CountryRepublic of Korea
- Language:English
-
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.
