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Valvular heart disease
The bicuspid aortic valve: an integrated phenotypic classification of leaflet morphology and aortic root shape
  1. B M Schaefer1,
  2. M B Lewin2,
  3. K K Stout1,2,
  4. E Gill1,
  5. A Prueitt2,
  6. P H Byers1,
  7. C M Otto1
  1. 1
    Division of Cardiology, Department of Medicine, Seattle, WA, USA
  2. 2
    Division of Cardiology, Department of Pediatrics, Seattle, WA, USA
  1. Dr Benjamin M Schaefer, Northeast Cardiology Associates, One Northeast Drive, Bangor, ME 04401, USA; benschae{at}u.washington.edu

Abstract

Objective: To establish a classification of bicuspid aortic valve (BAV) that includes both leaflet morphology and aortic shape.

Setting: Two academic medical centres of the University of Washington, Seattle.

Patients: 191 adult patients with BAV.

Interventions: Review of clinical data and transthoracic echocardiograms.

Main outcome measures: Assessment of leaflet morphology; valve function; aortic shape and dimensions.

Results: We identified three morphologies: type 1, fusion of right and left coronary cusp (n = 152); type 2, right and non-coronary fusion (n = 39); and type 3, left and non-coronary fusion (n = 1). Comparing type 1 and 2 BAV, there were no significant differences in age, height, weight, blood pressure or aortic valve function. Type 1 was more common in men (69 vs 45%). The aortic sinuses were larger in type 1, while type 2 had larger arch dimensions. Myxomatous mitral valves were more common in type 2 BAV (13% vs 2.6%, p<0.05). Three aortic shapes were defined: normal (N), sinus effacement (E), and ascending dilatation (A). Comparing type 1 to type 2 BAV, shape N was more common in type 1 (60% vs 32%), and type A was more common in type 2 (35% vs 54%,); type E was rare (p<0.01 across all groups).

Conclusion: A comprehensive BAV phenotype includes aortic shape. Type 1 BAV is associated with male gender and normal aortic shape but a larger sinus diameter. Type 2 leaflet morphology is associated with ascending aorta dilatation , larger arch dimensions and higher prevalence of myxomatous mitral valve disease.

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Footnotes

  • Funding: Supported by a fellowship grant from the ACCF/Merck Fellowship (ACC, Bethesda, MD, USA) and by the Seattle Foundation (Seattle, WA, USA).

  • Competing interests: None.

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