患有和未患有马凡综合征的患者的主动脉夹层比较（来自国际主动脉夹层注册研究的结果）

Comparison of aortic dissection in patients with and without Marfan's syndrome (results from the International Registry of Aortic Dissection).

作者信息

Januzzi James L, Marayati Firas, Mehta Rajendra H, Cooper Jeanna V, O'Gara Patrick T, Sechtem Udo, Bossone Eduardo, Evangelista Arturo, Oh Jae K, Nienaber Christoph A, Eagle Kim A, Isselbacher Eric M

机构信息

Thoracic Aorta Center and Cardiology Division, Massachusetts General Hospital, Boston, 02114, USA.

出版信息

Am J Cardiol. 2004 Aug 1;94(3):400-2. doi: 10.1016/j.amjcard.2004.04.049.

DOI:10.1016/j.amjcard.2004.04.049

PMID:15276119

Abstract

Among 1,049 patients diagnosed with aortic dissection in a multinational registry, patients with Marfan's syndrome were typically younger and had unique presentations. Despite their younger age at presentation, patients with Marfan's syndrome and aortic dissection had a high mortality rate, similar to patients without Marfan's syndrome, an older patient cohort. Our data support the importance of aneurysm surveillance and prophylactic surgical intervention for patients with Marfan's syndrome to potentially reduce the risk of mortality.

摘要

在一个跨国登记处确诊的1049例主动脉夹层患者中，患有马凡综合征的患者通常更年轻，且有独特的临床表现。尽管马凡综合征合并主动脉夹层的患者发病时年龄较轻，但他们的死亡率很高，与没有马凡综合征的老年患者群体相似。我们的数据支持对马凡综合征患者进行动脉瘤监测和预防性手术干预的重要性，这可能会降低死亡风险。