De novo obstruction after supravalvular aortic stenosis repair in Williams-Beuren syndrome.

Williams-Beuren syndrome is characterized by diffuse arteriopathy due to elastin gene deficiency. We present a patient with de novo supravalvular stenosis due to excessive intimal hyperplasia after a previous repair. This case report supports in vitro and animal studies that have linked elastin deficiency to increased cellular proliferation in the vessel wall with the subsequent development of obstructive lesions.
(Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)