title: Association between bicuspid aortic valve phenotype and patterns of valvular dysfunction and bicuspid aortopathy: comprehensive evaluation using MDCT and echocardiography.
abstract: OBJECTIVE
  We sought to define the clinical importance of an integrated classification of bicuspid aortic valve (BAV) phenotypes and aortopathy using multidetector computed tomography (MDCT).
  
  
  BACKGROUND
  An association between BAV phenotypes and the pattern of valvular dysfunction or bicuspid aortopathy has yet to be definitely established.
  
  
  METHODS
  The study cohort included 167 subjects (116 men, age 54.6 ± 14.4 years) who underwent both MDCT and transthoracic echocardiography from 2003 to 2010. Two BAV phenotypes-fusion of the right and left coronary cusps (BAV-AP) and fusion of the right or left coronary cusp and noncoronary cusp (BAV-RL)-were identified. Forty-five patients showed normal aortic dimensions and were classified as type 0. In the remaining patients, hierarchic cluster analysis showed 3 different types of bicuspid aortopathy according to the pattern of aortic dilation: type 1 (aortic enlargement confined to the sinus of Valsalva [n = 34]), type 2 (aortic enlargement involving the tubular portion of the ascending aorta [n = 49]), and type 3 (aortic enlargement extending to the transverse aortic arch [n = 39]).
  
  
  RESULTS
  The prevalence of BAV-AP and BAV-RL was 55.7% and 44.3%, respectively. Comparing BAV-AP and BAV-RL, no differences in age or in the prevalence of male sex were determined. However, significant differences in the valvular dysfunction pattern were noted, with moderate-to-severe aortic stenosis predominating in patients with BAV-RL (66.2% vs. 46.2% in BAV-AP; p = 0.01), and moderate-to-severe aortic regurgitation in BAV-AP (32.3% vs. 6.8% in BAV-RL; p < 0.0001). A normal aorta was the most common phenotype in BAV-AP patients (33.3% vs. 18.9% in BAV-RL; p = 0.037), and type 3 aortopathy was the most common phenotype in BAV-RL patients (40.5% vs. 9.7% in BAV-AP; p < 0.0001).
  
  
  CONCLUSIONS
  The patterns of valvular dysfunction and bicuspid aortopathy differed significantly between the 2 BAV phenotypes, suggesting the possibility of etiologically different entities.
authors.affiliation: Cardiac Imaging Center, Asan Heart Institute, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
authors.forename: Joon-Won
authors.initials: JW
authors.lastname: Kang
authors.forename: Hae Geun
authors.initials: HG
authors.lastname: Song
authors.forename: Dong Hyun
authors.initials: DH
authors.lastname: Yang
authors.forename: Seunghee
authors.initials: S
authors.lastname: Baek
authors.forename: Dae-Hee
authors.initials: DH
authors.lastname: Kim
authors.forename: Jong-Min
authors.initials: JM
authors.lastname: Song
authors.forename: Duk-Hyun
authors.initials: DH
authors.lastname: Kang
authors.forename: Tae-Hwan
authors.initials: TH
authors.lastname: Lim
authors.forename: Jae-Kwan
authors.initials: JK
authors.lastname: Song
mesh_terms: D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D001011:Aorta / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000503:physiopathology; D001021:Aortic Valve / Q000002:abnormalities / Q000000981:diagnostic imaging* / Q000503:physiopathology; D001027:Aortography / Q000379:methods*; D000082882:Bicuspid Aortic Valve Disease; D016009:Chi-Square Distribution; D016000:Cluster Analysis; D004108:Dilatation, Pathologic; D015150:Echocardiography, Doppler; D005260:Female; D006349:Heart Valve Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D061330:Multidetector Computed Tomography; D010641:Phenotype; D011237:Predictive Value of Tests; D012189:Retrospective Studies