主动脉病包括一系列诱发扩张的条件,动脉瘤,解剖,或主动脉和其他血管破裂。在儿童中通常被诊断为主动脉病,从婴儿期到青春期,主要影响胸主动脉,与外周血管系统的可变参与。发病机制包括结缔组织疾病,平滑肌收缩障碍,先天性心脏病,包括二叶主动脉瓣,在其他人中。美国心脏协会已经发布了胸主动脉疾病的诊断和管理指南。然而,这些指南主要针对成年人,不能巧妙地应用于具有新兴特征的成长中的儿童,生长和发育变化,包括青春期,与成年人相比,风险状况不同。降低儿童进行性主动脉扩张和夹层或破裂风险的管理是复杂的,涉及基因检测。心血管成像,医学治疗,改变生活方式,和手术指导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具有异质性,包括遗传和非遗传条件,并且有有限的公开证据来指导儿童护理。为了优化护理并减少管理差异,小儿主动脉病的专家召集会议,以产生有关主动脉病患儿的心血管护理的科学声明。现有证据和专家共识相结合,形成了这个科学声明。回顾了小儿主动脉病的最常见原因。本文件为儿童主动脉病的心血管管理提供了一般框架,同时允许根据每个孩子和家庭的个人和家庭特征进行修改。
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.