Diffuse Supravalvar Aortic Stenosis Associated with Congenital Anomaly of the Aortic Valve (Williams Syndrome): 1 case report.
- Author:
Soo Cheol KIM
;
Soon Ho CHON
;
Seog Ki LEE
;
Wook Sung KIM
;
Sam Se OH
;
Young Tak LEE
;
Woong Han KIM
;
Man Jong BAEK
;
Yang Bin JEON
;
Chang Ha LEE
;
Chan Young NA
;
Young Kwan PARK
;
Chong Whan KIM
- Publication Type:Case Report
- MeSH:
Adolescent;
Aorta;
Aorta, Thoracic;
Aortic Stenosis, Supravalvular*;
Aortic Valve*;
Arteries;
Brachiocephalic Trunk;
Circulatory Arrest, Deep Hypothermia Induced;
Coronary Vessels;
Humans;
Male;
Perfusion;
Williams Syndrome
- From:The Korean Journal of Thoracic and Cardiovascular Surgery
2000;33(9):748-751
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The diffuse form of supravalvar aortic stenosis represents a surgical challenge when the ascending aorta, aortic arch, proximal descending thoracic aorta and arch arteries are involved. It can be treated by a variety of surgical approaches. We report a case of severe diffuse supravalvar aortic stenosis combined with an aortic valve anomaly and occlusion of the right coronary artery ostium in a 14-year-old boy with Williams syndrome. We enlarged the aortic root (Nick's procedure), ascending aorta, aortic arch, proximal descending thoracic aorta, and innominate artery with patches and replaced aortic valve with 19 mm St. Jude valve. Deep hypothermic circulatory arrest and retrograde cerebral perfusion were used during repair of the arch and arch artery.
