Population-based treated prevalence, risk factors, and outcomes of bicuspid aortic valve in a pediatric Medicaid cohort

Abstract

Background:We investigated the treated prevalence of bicuspid aortic valve in a pediatric population with congenital heart disease and its incident complications.Materials and Methods:A 15-year retrospective data set was analyzed. Selection criteria included age ≤17 years, enrollees in the South Carolina State Medicaid program and diagnosed as having bicuspid aortic valve on one or more service visits.Results:The 15-year-treated prevalence of predominantly isolated bicuspid aortic valve was 2% (20/1000) of pediatric congenital heart disease cases, with a non-African American: African-American ratio of 3.5:1, and a male:female ratio of 1.6:1. Aortic stenosis (28.0%), ventricular septal defect (20.6%), and coarctation of the aorta (20.6%) were the most prevalent coexisting congenital heart lesions. Of the 378 bicuspid aortic valve cases examined, 10.3% received aortic valve repair/replacement, which was significantly more likely to be performed in children with diagnosed aortic stenosis (adjusted odds ratio = 12.90; 95% confidence interval = 5.66–29.44). Cohort outcomes over the study period indicated that 9.5% had diagnosed heart failure, but <1% had diagnosed supraventricular tachycardia, infective endocarditis, aneurysm, dissection, or death.Conclusions:The majority of isolated bicuspid aortic valve cases without aortic stenosis did not require surgical intervention. Outcomes for cases requiring repair/replacement were relatively benign.

Whole Exome Sequencing Uncovers the Genetic Complexity of Bicuspid Aortic Valve in Families with Early Onset Complications

“…The robust heritability of BAV was demonstrated in studies showing that primary relatives are ten-fold more likely to have a BAV than matched controls (3). While more than 50% of individuals with BAV undergo aortic valve surgery during their lifetimes, phenotypic expression of BAV varies widely, with only approximately 10% of patients with BAV requiring aortic valve surgery in the first two decades of life (4,5).…”

Prevalence of Bicuspid Aortic Valve: Systematic Review and Meta-Analysis of Prevalence Studies

“…Summary of Echocardiography Sources of Prevalence of Bicuspid Aortic Valve with total calculated 163 weighted(9,14,17,35,38,39,41,42). 164…”