Case reportDe Novo Obstruction After Supravalvular Aortic Stenosis Repair in Williams-Beuren Syndrome
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Comment
Supravalvular aortic stenosis seen in patients with WBS is a form of systemic elastin arteriopathy caused by deficient elastin deposition in the vessel wall [2]. In vitro and animal studies have linked elastin deficiency to increased cellular proliferation in the vessel wall with subsequent development of obstructive lesions 3, 4. The mechanism by which obstructive lesions develop is thought to be a two-step process, where injury-mediated remodelling occurs in the elastin-deficient thickened
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