title: De novo obstruction after supravalvular aortic stenosis repair in Williams-Beuren syndrome.
abstract: 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.
authors.affiliation: Department of Cardiology and Cardiac Surgery, Evelina Children's Hospital, London, United Kingdom; Department of Cardiothoracic Surgery, Alexandria University, Alexandria, Egypt.
authors.forename: Mohamed S
authors.initials: MS
authors.lastname: Nassar
authors.affiliation: Department of Cardiology and Cardiac Surgery, Evelina Children's Hospital, London, United Kingdom.
authors.forename: Kuberan
authors.initials: K
authors.lastname: Pushparajah
authors.affiliation: Department of Cardiology and Cardiac Surgery, Evelina Children's Hospital, London, United Kingdom.
authors.forename: Aaron
authors.initials: A
authors.lastname: Bell
authors.affiliation: Department of Cardiology and Cardiac Surgery, Evelina Children's Hospital, London, United Kingdom. Electronic address: conal.austin@gstt.nhs.uk.
authors.forename: Conal B
authors.initials: CB
authors.lastname: Austin
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D002675:Child, Preschool; D006801:Humans; D008297:Male; D012008:Recurrence; D018980:Williams Syndrome / Q000150:complications / Q000175:diagnosis / Q000601:surgery*