title: Cardiac outcomes in adults with supravalvar aortic stenosis.
abstract: OBJECTIVE
  Supravalvar aortic stenosis is a rare form of left ventricular outflow tract obstruction that is often progressive in childhood. Little data are available on outcomes in the adult population. Our aim was to define cardiac outcomes in adults with supravalvar aortic stenosis.
  
  
  RESULTS
  This is a multicentre retrospective study of cardiac outcomes in adults (≥18 years) with supravalvar aortic stenosis. We examined: (i) adverse cardiac events (cardiovascular death, myocardial infarction, stroke, heart failure, sustained arrhythmias, and infective endocarditis) and (ii) the need for cardiac surgery in adulthood. One hundred and thirteen adults (median age at first visit 19 years; 55% with Williams-Beuren syndrome; 67% with surgical repair in childhood) were identified. Adults without Williams-Beuren syndrome had more severe supravalvar aortic stenosis and more often associated left ventricular outflow tract obstructions (P < 0.001). In contrast, mitral valve regurgitation was more common in patients with Williams-Beuren syndrome. Eighty-five per cent of adults (96/113) had serial follow-up information (median follow-up 6.0 years). Of these patients, 13% (12/96) had an adverse cardiac event and 13% (12/96) had cardiac operations (7 valve repair or replacements, 4 supravalvar aortic stenosis repairs, 1 other). Cardiac surgery was more common in adults without Williams-Beuren syndrome (P = 0.007). Progression of supravalvar aortic stenosis during adulthood was rare.
  
  
  CONCLUSIONS
  Adults with supravalvar aortic stenosis remain at risk for cardiac complications and reoperations, while progression of supravalvar aortic stenosis in adulthood is rare. Valve surgery is the most common indication for cardiac surgery in adulthood.
authors: Adult Congenital Heart Disease Program, University Hospital Zurich, Zurich, Switzerland. matthias.greutmann@usz.ch
authors: Matthias
authors: M
authors: Greutmann
authors: Daniel
authors: D
authors: Tobler
authors: Nakul Chander
authors: NC
authors: Sharma
authors: Isabelle
authors: I
authors: Vonder Muhll
authors: Siegrun
authors: S
authors: Mebus
authors: Harald
authors: H
authors: Kaemmerer
authors: Pia K
authors: PK
authors: Schuler
authors: John E
authors: JE
authors: Deanfield
authors: Luc
authors: L
authors: Beauchesne
authors: Omid
authors: O
authors: Salehian
authors: Andreas
authors: A
authors: Hoffmann
authors: Vasilisa
authors: V
authors: Golovatyuk
authors: Erwin N
authors: EN
authors: Oechslin
authors: Candice K
authors: CK
authors: Silversides
mesh_terms: D000293:Adolescent; D000328:Adult; D021921:Aortic Stenosis, Supravalvular / Q000628:therapy*; D002318:Cardiovascular Diseases / Q000209:etiology* / Q000601:surgery; D018572:Disease-Free Survival; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D019616:Thoracic Surgical Procedures / Q000706:statistics & numerical data; D016896:Treatment Outcome; D055815:Young Adult