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成人马凡综合征和其他胸主动脉扩张的医学治疗:当代综述。

Medical Therapies for Marfan Syndrome and Other Thoracic Aortic Dilatation in Adults: A Contemporary Review.

机构信息

Section of Cardiovascular Imaging, Robert and Suzanne Tomsich Department of Cardiovascular Medicine, Sydell and Arnold Miller Family Heart, Vascular and Thoracic Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk J1-5, Cleveland, OH, 44195, USA.

出版信息

Am J Cardiovasc Drugs. 2021 Nov;21(6):609-617. doi: 10.1007/s40256-021-00472-y. Epub 2021 Mar 22.

Abstract

Thoracic aortic dilatation often has an asymptomatic course but may present with lethal complications such as aortic rupture or dissection, particularly when the thoracic aorta is aneurysmally enlarged; therefore, appropriate diagnosis, surveillance, and management are vital. Recommendations regarding imaging and surgical management are outlined in contemporary clinical practice guidelines. Dedicated guidelines regarding medical therapies for the management of thoracic aortic dilatation are lacking. Most of the medical treatment strategies, especially recommendations regarding pharmacological medical therapies related to β-blockade and angiotensin receptor blockers, are derived from data on patients with Marfan syndrome. The main aims of medical therapies for the management of thoracic aortic dilatation are (1) to control the progression of the disease, and (2) to prevent complications related to the disease (such as aortic dissection and mortality). This paper reviews the contemporary evidence and highlights the gaps in evidence to be investigated in further studies.

摘要

胸主动脉扩张通常呈无症状过程,但可能出现致命并发症,如主动脉破裂或夹层,特别是当胸主动脉呈瘤样扩张时;因此,适当的诊断、监测和管理至关重要。目前的临床实践指南中概述了有关影像学和手术管理的建议。缺乏针对胸主动脉扩张管理的医学治疗的专门指南。大多数药物治疗策略,特别是与β受体阻滞剂和血管紧张素受体阻滞剂相关的药物治疗建议,均源自马凡综合征患者的数据。药物治疗胸主动脉扩张的主要目的是(1)控制疾病的进展,(2)预防与疾病相关的并发症(如主动脉夹层和死亡率)。本文综述了目前的证据,并强调了进一步研究中需要调查的证据空白。

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