Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, United States of America.
Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Palo Alto, CA, United States of America; Division of Cardiovascular Medicine, Department of Medicine, Stanford University, Palo Alto, CA, United States of America.
Prog Cardiovasc Dis. 2020 Jul-Aug;63(4):425-433. doi: 10.1016/j.pcad.2020.05.012. Epub 2020 Jun 1.
Bicuspid aortic valve (BAV) is one of the most common congenital heart defects in children, adolescents and adults. BAV can occur as an isolated lesion or in association with other congenital heart defects, such as coarctation of the aorta or genetic syndromes like Turner syndrome. Although the majority of long term complications associated with BAV manifest later in life, children and adolescents may present with early onset valvular dysfunction or dilation of the aorta. BAV is a heterogeneous disease with a wide array of presentations at various ages, depending on the degree of aortic valve dysfunction, aortic dilation and presence of associated lesions. Aortic valve stenosis and/or regurgitation are the primary indications for intervention in children and adolescents with BAV. Although a majority of young patients with BAV also have some aortic dilation, interventions on the aorta are very rare during this time frame. Children and adolescents with BAV benefit from comprehensive assessment of their risk profile to determine follow-up surveillance intervals, sports recommendations, and timing of surgical intervention. The morphologic phenotype of BAV is important to identify, as it may predict future complications and prognosis.
二叶式主动脉瓣（BAV）是儿童、青少年和成年人中最常见的先天性心脏缺陷之一。BAV 可单独发生，也可与其他先天性心脏缺陷相关，如主动脉缩窄或特纳综合征等遗传综合征。尽管与 BAV 相关的大多数长期并发症在以后的生活中出现，但儿童和青少年可能会出现早期瓣膜功能障碍或主动脉扩张。BAV 是一种异质性疾病，在不同年龄表现出广泛的表现，取决于主动脉瓣功能障碍、主动脉扩张和相关病变的存在程度。主动脉瓣狭窄和/或反流是儿童和青少年 BAV 干预的主要指征。尽管大多数患有 BAV 的年轻患者也有一些主动脉扩张，但在此期间很少对主动脉进行干预。患有 BAV 的儿童和青少年受益于全面评估其风险状况，以确定随访监测间隔、运动建议和手术干预时机。BAV 的形态表型很重要，因为它可能预测未来的并发症和预后。
