title: Rates and determinants of progressive aortic valve dysfunction in aortic coarctation.
abstract: OBJECTIVE
  Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA.
  
  
  METHODS
  96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥ 20 mmHg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥ 37 mm.
  
  
  RESULTS
  All patients (median age 28.0 years, range 17-61 years; male 57%) were followed with a median follow-up of 7.0 years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV). Median AS progression was 1.1 mmHg/5 years (range - 13-28). Determinants for AS at follow-up were age (ß=0.20, P=0.01), aortic dilatation (ß=4.6, P=0.03), and baseline aortic valve gradient (ß=0.93, P<0.001). BAV was predictive for AR. (ß=0.91, P=0.049).
  
  
  CONCLUSIONS
  Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA.
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: Paul
authors.initials: P
authors.lastname: Luijendijk
authors.forename: Arnaud W J M
authors.initials: AW
authors.lastname: Stevens
authors.forename: Rianne H A C M
authors.initials: RH
authors.lastname: de Bruin-Bon
authors.forename: S Matthijs
authors.initials: SM
authors.lastname: Boekholdt
authors.forename: Joris W J
authors.initials: JW
authors.lastname: Vriend
authors.forename: Hubert W
authors.initials: HW
authors.lastname: Vliegen
authors.forename: Berto J
authors.initials: BJ
authors.lastname: Bouma
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
mesh_terms: D000293:Adolescent; D000328:Adult; D001017:Aortic Coarctation / Q000175:diagnosis* / Q000453:epidemiology*; D001021:Aortic Valve; D000082882:Bicuspid Aortic Valve Disease; D016208:Databases, Factual / Q000639:trends; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000453:epidemiology*; D006349:Heart Valve Diseases / Q000175:diagnosis* / Q000453:epidemiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D055815:Young Adult