Clift Paul F, Cervi Elena
Department of Cardiology, University Hospital Birmingham, Birmingham, UK.
Department of Cardiology, Great Ormond St Hospital, London, UK.
Echo Res Pract. 2019 Dec 5;7(1):R1-R10. doi: 10.1530/ERP-19-0049. eCollection 2020 Mar.
Aortic diseases may be diagnosed after a long period of subclinical development or they may have an acute presentation. Acute aortic syndrome is often the first sign of the disease, which needs rapid diagnosis and decision making to reduce the extremely poor prognosis. Aortic dilatation is a well-recognised risk factor for acute events and can occur as a result of trauma, infection, or, most commonly, from an intrinsic abnormality in the elastin and collagen components of the aortic wall. Over the years it has become clear that a few monogenic syndromes are strongly associated with aneurysms and often dictate a severe presentation in younger patients while the vast majority have a multifactorial pathogenesis. Conventional cardiovascular risk factors and ageing play an important role. Management strategy is based on prevention consisting of regular follow-up with cross-sectional imaging, chemoprophylaxis of further dilatation with drugs proved to slow down the disease progression and preventative surgery when dimension exceeds internationally recognised cut-off values for aortic diameters and the risk of rupture/dissection is therefore deemed very high.
主动脉疾病可能在经过长时间的亚临床发展后被诊断出来，也可能有急性发作的表现。急性主动脉综合征往往是该病的首发症状，需要快速诊断并做出决策，以降低极差的预后。主动脉扩张是急性事件的一个公认危险因素，可能由创伤、感染引起，或者最常见的是由于主动脉壁弹性蛋白和胶原蛋白成分的内在异常导致。多年来已经明确，一些单基因综合征与动脉瘤密切相关，并且常常在年轻患者中表现严重，而绝大多数患者具有多因素发病机制。传统的心血管危险因素和衰老起着重要作用。管理策略基于预防，包括定期进行横断面成像随访、使用已证明能减缓疾病进展的药物进行化学预防以防止进一步扩张，以及当主动脉直径超过国际公认的临界值且破裂/夹层风险因此被认为非常高时进行预防性手术。
