Although patients with bicuspid aortic valves (BAVs) are predisposed to ascending aortic (AA) dilation, stenosis, and dissection, the development of aortic disease in children with BAVs is poorly described. The purposes of this study were to determine the rate of change of AA diameter in children with BAVs and to identify risk factors for the development of aortic dilation. The echocardiograms of 276 children aged<19 years (mean 8.5+/-5.3) with isolated BAVs were reviewed. Aortic measurements were normalized to z scores on the basis of body surface area. In a subset of 112 patients with serial examinations, aortic growth rates were calculated and risk factors for more rapid aortic growth determined. At presentation, 33 patients (12%) demonstrated marked AA dilation (z>4), and 70 (25%) were moderately abnormal (z between 2 and 4). The mean+/-SD AA diameter increased more than expected, at a rate of 0.18+/-0.30 z score per year (p<0.0001). In 61 patients with normal AA diameters on initial study, 22 (36%) had abnormal diameters, with z scores>2, at follow-up. Univariate analysis demonstrated right-noncoronary commissural fusion (p<0.02) and aortic valve gradient on initial examination (p<0.02) as significant predictors of AA growth. In multivariate analysis, however, the significance of gradient and valve morphology was diminished (p = 0.06 for both). In conclusion, the progression of AA diameter in patients with normal z scores on initial examination suggests that serial echocardiograms are required to screen for the development of significant aortic dilation.