title: Aortic dilation rates in patients with biscuspid aortic valve: correlations with cusp fusion phenotype.
abstract: OBJECTIVE
  The study aim was to compare the pattern and rate of aortic dilation in patients with isolated non-surgical bicuspid aortic valve (BAV) with left-right fusion (L-R) and right-non coronary fusion (R-N). Although the etiology and optimal management of aortic dilation associated with BAV remain the subject of debate, recent data have suggested that L-R and R-N cusp fusion phenotypes represent distinct pathological entities.
  
  
  METHODS
  Consecutive patients with BAV and at least two echocardiographic assessments made between 2006 and 2012 were reviewed. Patients with hemodynamically significant valvular disease, aortic aneurysm (> 50 mm) or unrepaired aortic coarctation were excluded. Longitudinal analyses of the aortic annulus, sinuses of Valsalva, sinotubular junction and ascending aortic diameters were performed using mixed-effect models.
  
  
  RESULTS
  A total of 590 echocardiographic studies was analyzed in 212 patients (mean age 33 ± 14 years), of which 147 had L-R phenotype and 65 had R-N phenotype. The median follow up was 3.6 years. Baseline aortic diameters at the sinuses of Valsalva were larger in patients with L-R compared to R-N fusion (33.8 ± 5.3 mm versus 30.8 ± 4.8 mm; p < 0.001). At this level, the rate of aortic dilation was higher with L-R versus R-N fusion (0.41 ± 0.11 mm/year versus 0.01 ± 0.08 mm/year; p < 0.001). The rate of proximal ascending aortic dilation was also higher with L-R versus R-N fusion (0.58 ± 0.08 mm/year versus 0.18 ± 0.09 mm/year; p < 0.001).
  
  
  CONCLUSIONS
  Aortic dilation rates vary according to the pattern of BAV cusp fusion, with faster rates of aortic sinus and ascending aortic dilation associated with the L-R compared to R-N phenotype.
authors: Maude
authors: M
authors: Pagé
authors: François-Pierre
authors: FP
authors: Mongeon
authors: Louis-Mathieu
authors: LM
authors: Stevens
authors: Vicky
authors: V
authors: Soulière
authors: Paul
authors: P
authors: Khairy
authors: Ismail
authors: I
authors: El-Hamamsy
mesh_terms: D000328:Adult; D001011:Aorta / Q000000981:diagnostic imaging / Q000473:pathology*; D001021:Aortic Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D000082882:Bicuspid Aortic Valve Disease; D004108:Dilatation, Pathologic / Q000150:complications / Q000000981:diagnostic imaging; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012189:Retrospective Studies; D014463:Ultrasonography; D055815:Young Adult