title: Supravalvar aortic stenosis in infancy.
abstract: Supravalvar aortic stenosis (SVAS) is a rare anomaly of the aortic root caused by a genetically based deficiency in elastin production. Concomitant primary and secondary cardiovascular lesions complicate surgical management and impact early and late outcomes. Because SVAS is a rare lesion, surgical series are relatively small and span lengthy time periods. Consequently, risk factors that influence early and late outcomes are not well defined. Patients who come to surgery during infancy are particularly challenging, but little attention has been directed as to whether or not young age influences outcomes. This review suggests that complicating associated features of elastin arteriopathy are more prevalent in patients who require relief of SVAS during infancy, and that concomitant lesions significantly increase the difficulty and risk of treating younger patients with SVAS.
authors: Department of Surgery, University of Colorado at Denver Health Sciences Center, Denver, CO, USA. mitchell.max@tchden.org
authors: Max B
authors: MB
authors: Mitchell
authors: Steven P
authors: SP
authors: Goldberg
mesh_terms: D000792:Angiography / Q000379:methods; D021921:Aortic Stenosis, Supravalvular / Q000151:congenital / Q000000981:diagnostic imaging / Q000601:surgery*; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000379:methods*; D023921:Coronary Stenosis / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D004549:Elastin; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis / Q000401:mortality; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D004549:Elastin