Disease progression and variation in clinical practice for isolated bicuspid aortic valve in children

Disease progression of an isolated bicuspid aortic valve (BAV) in children is poorly understood and adult management guidelines may not be applicable. Thus, we sought to evaluate disease progression of pediatric isolated BAV and its relationship to current management practices.

Children with a BAV and ≤mild aortic stenosis (AS) and/or aortic regurgitation (AR) at the time of initial evaluation were included in this retrospective cohort study (1/2005-12/2014). Outcomes included change in z-scores for aortic root and ascending aorta diameters, cardiac interventions, adverse outcomes, recommended follow-up interval, and frequency of cardiac imaging studies at each follow up evaluation, as well as AS/AR severity at final evaluation. Outcomes were analyzed using generalized mixed-effect models with subject and provider clustering.

BAV disease progression was evaluated in 294 subjects over 4.1 ± 2.4 (range 0.2-9.5) years. Ascending aorta z-scores increased by 0.1/year (P < .001) but aortic root diameter z-scores were unchanged. AS and/or AR progressed to >mild in 9 (3%), 1 subject underwent cardiac intervention, and none had a major complication. Management was evaluated in 454 subjects (1343 encounters) with 27 different cardiologists. The average recommended follow-up interval was 1.5 ± 0.9 years. Younger age at diagnosis, greater aortic root or ascending aorta z-score at diagnosis, ≥mild AS/AR at follow-up, and earlier diagnosis era were associated with shorter recommended follow-up interval (P < .001 for all). Imaging was obtained at 87% of follow-up encounters and was associated with age at encounter with children ≥12 years most frequently imaged (P < .001). Provider accounted for 14% of variability in recommended follow-up interval and 24% of imaging variability (P < .001 for both).

We found little to no evidence of disease progression in children with an isolated BAV. Given the low risk, close follow-up and frequent cardiac imaging for BAV surveillance may not be warranted for children.