title: Aortic valve dysfunction and aortic dilation in adults with coarctation of the aorta.
abstract: OBJECTIVE
  To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA).
  
  
  BACKGROUND
  Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease.
  
  
  METHODS
  We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010.
  
  
  RESULTS
  Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement.
  
  
  CONCLUSIONS
  In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV.
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Mass, USA.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Clair
authors.forename: Susan M
authors.initials: SM
authors.lastname: Fernandes
authors.forename: Paul
authors.initials: P
authors.lastname: Khairy
authors.forename: Dionne A
authors.initials: DA
authors.lastname: Graham
authors.forename: Eric V
authors.initials: EV
authors.lastname: Krieger
authors.forename: Alexander R
authors.initials: AR
authors.lastname: Opotowsky
authors.forename: Michael N
authors.initials: MN
authors.lastname: Singh
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Erik J
authors.initials: EJ
authors.lastname: Meijboom
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Landzberg
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D001011:Aorta / Q000473:pathology*; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D001017:Aortic Coarctation / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D001021:Aortic Valve / Q000002:abnormalities / Q000503:physiopathology*; D001022:Aortic Valve Insufficiency / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D001024:Aortic Valve Stenosis / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D000082882:Bicuspid Aortic Valve Disease; D001900:Boston / Q000453:epidemiology; D004108:Dilatation, Pathologic; D005260:Female; D006349:Heart Valve Diseases / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D055815:Young Adult
keywords: Aortic Dilation; Aortic Valve Dysfunction; Bicuspid Aortic Valve; Coarctation of the Aorta