Bicuspid Aortic Valve and Associated Aortic Dilatation in the Young

Study Questions:

What is the rate of growth of the aorta in young patients with bicuspid aortic valve (BAV)?

Methods:

A retrospective review was performed at a single center. Of an inception cohort of 1,192 patients with BAV identified between 1986 and 1999, 333 patients were randomly selected for this study. Mild aortic dilatation was defined as a Z-score >2 but ≤4, moderate dilatation as a Z-score >4 but ≤6, and severe dilatation as a Z-score >6.

Results:

Of the 333 patients studied, 74% were male, and the median age at the most recent study was 13.5 (range 0-30) years. Moderate or severe aortic root dilatation was present in 14 (5%) patients, whereas moderate or severe ascending aortic dilatation was present in 53 (16%) patients. Over the follow-up period, minimal change in aortic Z-scores were noted. Predictors of moderate or greater aortic root dilatation included moderate or greater aortic dilatation, absence of moderate or greater aortic stenosis, and fusion of the right and left coronary leaflets. Predictors of moderate or greater ascending aortic dilatation included moderate or greater aortic regurgitation and absence of aortic coarctation.

Conclusions:

Significant dilatation of the ascending aorta is more common than significant dilatation of the aortic root in young patients with BAV. Aortic Z-scores remain relatively stable during childhood.

Perspective:

A practical consideration in the care of young patients with BAV is determining the frequency of surveillance echocardiograms to assess for aortic dilatation. This study shows that in children, there is little progression of aortic size. For patients with only mild or moderate aortic dilatation and no evidence of increasing dimensions, annual or biennial echocardiograms do not appear to be indicated.

Keywords: Heart Diseases, Follow-Up Studies, Heart Valve Diseases, Dilatation, Echocardiography


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