期刊
CURRENT OPINION IN PEDIATRICS
卷 35, 期 5, 页码 538-545出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MOP.0000000000001276
关键词
aortic aneurysm; aortic dissection; bicuspid aortic valve; bicuspid aortopathy; congenital heart disease
类别
The purpose of this review is to summarize and critically evaluate recent clinical and research advancements in pediatric BAV and its associated aortopathy. Recent findings show that progressive aortic dilation is commonly present in pediatric patients with BAV and is associated with increased risk for aortic aneurysm, dissection, and surgery in adulthood. Ongoing research aims to explore the cause, incidence, and progression of bicuspid aortopathy to improve early diagnosis and preventive management.
Purpose of reviewTo synthesize and critically assess recent clinical and research advancements in pediatric bicuspid aortic valve (BAV) and its associated aortopathy.Recent findingsIn 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.SummaryPediatric 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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