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Progressive dilation of the ascending aorta in children with isolated bicuspid aortic valve

Kathryn W Holmes1, Christoph U Lehmann, Darshan Dalal

  • 1Department of Pediatrics, Johns Hopkins University, Baltimore, Maryland, USA. kwholmes@jhmi.edu

The American Journal of Cardiology
|April 3, 2007
PubMed

Insights

Children with bicuspid aortic valves (BAVs) show significant ascending aortic (AA) dilation, increasing yearly. Regular echocardiograms are crucial for monitoring aortic growth and detecting dilation in pediatric BAV patients.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Cardiovascular Imaging

Background:

  • Bicuspid aortic valves (BAVs) predispose individuals to ascending aortic (AA) dilation, stenosis, and dissection.
  • Aortic disease development in pediatric BAV patients is not well-documented.

Purpose of the Study:

  • To determine the rate of change in AA diameter in children with BAVs.
  • To identify risk factors associated with the development of aortic dilation.

Main Methods:

  • Reviewed echocardiograms of 276 children (<19 years) with isolated BAVs.
  • Normalized aortic measurements using z scores based on body surface area.
  • Calculated aortic growth rates in 112 patients with serial examinations.

Main Results:

  • At presentation, 12% had marked AA dilation (z>4) and 25% moderate dilation (z 2-4).
  • Mean AA diameter increased by 0.18 z score/year (p<0.0001).
  • Of 61 patients with normal initial z scores, 36% developed abnormal dilation (z>2) on follow-up.

Conclusions:

  • Ascending aortic diameter progresses in pediatric BAV patients, even those with normal initial z scores.
  • Serial echocardiography is essential for screening and monitoring aortic dilation in this population.

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