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Medical Therapies for Marfan Syndrome and Other Thoracic Aortic Dilatation in Adults: A Contemporary Review
American Journal of Cardiovascular Drugs ( IF 3 ) Pub Date : 2021-03-22 , DOI: 10.1007/s40256-021-00472-y
Duygu Kocyigit 1 , Brian P Griffin 1 , Bo Xu 1
Affiliation  

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.



中文翻译:

成人马凡综合征和其他胸主动脉扩张的医学治疗:当代评论

胸主动脉扩张通常没有症状,但可能会出现致命的并发症,如主动脉破裂或夹层,尤其是当胸主动脉动脉瘤变小时;因此,适当的诊断、监测和管理至关重要。当代临床实践指南中概述了有关成像和手术管理的建议。缺乏关于治疗胸主动脉扩张的药物治疗的专门指南。大多数医学治疗策略,尤其是与 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,均来自马凡综合征患者的数据。治疗胸主动脉扩张的药物治疗的主要目的是 (1) 控制疾病的进展,和 (2) 预防与疾病相关的并发症(如主动脉夹层和死亡率)。本文回顾了当代证据,并强调了在进一步研究中需要调查的证据差距。

更新日期:2021-03-22
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