New England Congenital Cardiology Association Bicuspid Aortopathy Registry (NECCA BAR): A regional preventive cardiovascular care collaboration

Background: Bicuspid aortic valve is the most common congenital heart defect, and its association with proximal aortic dilation (aortopathy) increases risk for aortic aneurysm and dissection in adults. Maximal aortic root and ascending aortic dimensions are predictors of adverse cardiovascular outcomes. Recent single center data demonstrate medical therapy with beta or angiotensin II receptor blockers tempers aortic growth rates. Although randomized clinical trials may not be feasible, larger population studies would better inform bicuspid aortic valve preventive cardiovascular care.Objective: Improve regional pediatric bicuspid aortopathy collaboration with standardized medical management guidelines and patient care registry.Methods: The New England Congenital Cardiology Association (NECCA) developed consensus-based prophylactic treatment guidelines for bicuspid aortopathy and designed a regional clinical registry (NECCA BAR) to study cardiovascular practice variation and short-term outcomes in subjects with moderate to severe bicuspid aortopathy. Individuals 6 months to 25 years of age with bicuspid aortic valve (all variants) and body surface area adjusted aortic root or ascending aorta z score > 4.0 SD, and/or absolute dimension >4.0 cm, are eligible for inclusion. Standardized bicuspid aortopathy medical management guidelines were established among New England congenital cardiology practices. The primary aims are to compare cardiovascular practice variation by guideline adherence (medical therapy and athletic participation), and evaluate medication effectiveness by comparing rates of annual change in aortic root and ascending aorta dimensions/z scores over 3 years. Additional endpoints include aortic valve disease progression (stenosis/regurgitation), incidence of aortic dissection, surgery, death, and incidence of adverse drug events.Results: Recruitment in progress; data analysis pending.

Automated summary

This study aims to improve regional collaboration for pediatric bicuspid aortopathy and investigate cardiovascular practice variation and short-term outcomes through the development of standardized medical management guidelines and patient care registry. Recruitment and data analysis are currently in progress.