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Bicuspid aortic valve and its ascending aortopathy
Emma C Dunne1, Ronald V Lacro2,3, Jonathan N Flyer1,4
1The Robert Larner, M.D. College of Medicine at The University of Vermont, Burlington, Vermont.
Insights
Pediatric bicuspid aortic valve (BAV) research highlights familial screening, safe exercise, and medical management to prevent aortic dilation and improve long-term outcomes for children with BAV.
Area of Science:
- Cardiovascular Medicine
- Pediatric Cardiology
- Genetics
Background:
- Bicuspid aortic valve (BAV) is a common congenital heart defect.
- BAV is associated with progressive aortic dilation, termed bicuspid aortopathy.
- Bicuspid aortopathy increases risks for aortic aneurysm, dissection, and adult surgery.
Conclusions:
- Pediatric bicuspid aortopathy requires further investigation and preventive strategies.
- Recent literature supports familial screening, exercise, medical prophylaxis, and registry-based evaluation.
- Continued scientific inquiry is crucial for improving long-term cardiovascular outcomes in pediatric BAV patients.
Purpose Of Review:
To synthesize and critically assess recent clinical and research advancements in pediatric bicuspid aortic valve (BAV) and its associated aortopathy.
Recent Findings:
In pediatric patients with BAV, progressive aortic dilation (i.e. bicuspid aortopathy) is commonly present and associated with increased risk for aortic aneurysm, dissection, and surgery in adulthood. Ongoing research explores the cause, incidence, and progression of bicuspid aortopathy to promote earlier diagnosis and improve preventive management. Recent findings include: high familial incidence and need for improved familial screening; safety of recreational physical activity in most affected children; potential for medical management to slow aortic growth; feasibility of pediatric registries to evaluate longitudinal outcomes; and potential genetic and hemodynamic biomarkers for disease risk stratification.
Summary:
Pediatric bicuspid aortopathy is an important area for investigation and preventive management to improve long-term cardiovascular outcomes. Recent literature promotes familial screening, recreational exercise, medical prophylaxis, registry-based longitudinal evaluation, and continued scientific inquiry.
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