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Bicuspid aortic valve morphology and interventions in the young.

Susan M Fernandes1, Paul Khairy, Stephen P Sanders

  • 1Department of Cardiology, Children's Hospital, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA.

Journal of the American College of Cardiology
|June 5, 2007
PubMed
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The morphology of bicuspid aortic valve (BAV) impacts outcomes. Right- and noncoronary leaflet fusion (R-N) predicts earlier valve intervention and faster disease progression compared to right- and left-coronary leaflet fusion (R-L).

Area of Science:

  • Cardiology
  • Cardiac Surgery
  • Medical Genetics

Background:

  • Bicuspid aortic valve (BAV) morphology has prognostic relevance.
  • Right- and noncoronary leaflet (R-N) fusion is linked to more severe valve dysfunction than other BAV subtypes.
  • The clinical impact of BAV subtypes on valve intervention remains unclear.

Purpose of the Study:

  • To investigate the association between bicuspid aortic valve (BAV) morphologic subtypes and the need for valve intervention in young patients.
  • To determine if specific BAV morphologies predict clinical outcomes.

Main Methods:

  • A nested cohort study included 310 patients with R-N or right- and left-coronary leaflet (R-L) fusion BAV.
  • Patients were randomly selected from an inception cohort identified between 1986 and 1999.

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  • Retrospective data collection from medical charts and databases supplemented the analysis.

    • Main Results:

    • Right- and noncoronary leaflet (R-N) fusion (n=108) was a strong predictor of valve intervention compared to right- and left-coronary leaflet (R-L) fusion (n=202) (HR 4.5, p<0.0001).
    • R-N fusion correlated with increased progression of aortic valve gradient (GEE RR 27.2, p=0.0386) and aortic regurgitation (GEE RR 2.4, p=0.0029) over a median follow-up of 16.1 years.
    • Longitudinal analysis of 799 echocardiograms supported these findings.

    Conclusions:

    • Bicuspid aortic valve (BAV) morphology significantly predicts clinically important outcomes.
    • Right- and noncoronary leaflet (R-N) fusion is associated with accelerated aortic stenosis and regurgitation progression.
    • Patients with R-N fusion BAV experience a shorter time to valve intervention.