Aortopathy encompasses a spectrum of conditions predisposing to dilation, aneurysm, dissection, or rupture of the aorta and other blood vessels. Aortopathy is diagnosed commonly in children, from infancy through adolescence, primarily affecting the thoracic aorta, with variable involvement of the peripheral vasculature. Pathogeneses include connective tissue disorders, smooth muscle contraction disorders, and congenital heart disease, including bicuspid aortic valve, among others. The American Heart Association has published guidelines for diagnosis and management of thoracic aortic disease. However, these guidelines are predominantly focused on adults and cannot be applied adeptly to growing children with emerging features, growth and developmental changes, including puberty, and different risk profiles compared with adults. Management to reduce risk of progressive aortic dilation and dissection or rupture in children is complex and involves genetic testing, cardiovascular imaging, medical therapy, lifestyle modifications, and surgical guidance that differ in many ways from adult management. Pediatric practice varies widely, likely because aortopathy is pathogenically heterogeneous, including genetic and nongenetic conditions, and there is limited published evidence to guide care in children. To optimize care and reduce variation in management, experts in pediatric aortopathy convened to generate this scientific statement regarding the cardiovascular care of children with aortopathy. Available evidence and expert consensus were combined to create this scientific statement. The most common causes of pediatric aortopathy are reviewed. This document provides a general framework for cardiovascular management of aortopathy in children, while allowing for modification based on the personal and familial characteristics of each child and family.

中文翻译：

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儿童主动脉病的心血管治疗：美国心脏协会的科学声明。


主动脉病包括一系列容易导致主动脉和其他血管扩张、动脉瘤、夹层或破裂的病症。主动脉病通常在从婴儿期到青春期的儿童中被诊断出来，主要影响胸主动脉，并不同程度地累及周围血管系统。发病机制包括结缔组织疾病、平滑肌收缩疾病和先天性心脏病，包括二叶式主动脉瓣等。美国心脏协会发布了胸主动脉疾病的诊断和治疗指南。然而，这些指南主要针对成年人，不能熟练地应用于具有新特征、生长和发育变化（包括青春期）以及与成人相比不同风险状况的成长中儿童。降低儿童进行性主动脉扩张、夹层或破裂风险的管理非常复杂，涉及基因检测、心血管成像、药物治疗、生活方式改变和手术指导，这些在许多方面与成人管理不同。儿科实践差异很大，可能是因为主动脉病的致病异质性，包括遗传和非遗传性疾病，并且已发表的指导儿童护理的证据有限。为了优化护理并减少管理差异，小儿主动脉病专家聚集在一起，就主动脉病儿童的心血管护理提出了这一科学声明。现有证据和专家共识相结合，形成了这一科学声明。回顾了小儿主动脉病的最常见原因。 本文件提供了儿童主动脉病心血管治疗的总体框架，同时允许根据每个儿童和家庭的个人和家庭特征进行修改。