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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. Improve regional pediatric bicuspid aortopathy collaboration with standardized medical management guidelines and patient care registry. 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. Recruitment in progress; data analysis pending. We describe the development of regional pediatric clinical care guidelines and a patient care registry designed to refine medical management of bicuspid aortopathy. • Bicuspid aortopathy carries risk for aortic aneurysm, surgery, dissection and death. • Losartan or atenolol reduced aortic growth rates in a small group of young patients. • Standard clinical recommendations can improve regional management and collaboration. • Larger registry data are needed to track bicuspid aortopathy patients and outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures., Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs., Results: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice., Conclusion: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients., (© 2019 Wiley Periodicals, Inc.)