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[马凡氏综合征的心血管表现。病例报告及治疗综述]

[The cardiovascular manifestations of Marfan's syndrome. A case report and review of the management].

作者信息

Bértoli F, Remón J A, González R E, Poppe N

机构信息

Servicio de Patología, Complejo Hospitalario Arnulfo Arias Madrid, Caja de Seguro Social.

出版信息

Rev Med Panama. 1993 Sep;18(3):196-204.

PMID:8146346
Abstract

The authors present the clinical history of a patient with Marfan's syndrome (MS) and aortic insufficiency, who expired at 22 years of age, in order to illustrate the clinical evolution and review the management of this problem. It is known that patients with MS and heart failure due to dilatation of the aortic annulus have an early mortality of almost 100%. The authors conclude that echocardiographic follow up of these patients is vital in their medical and surgical management and that the use of beta blockers is the best medical therapy, and that prophylactic surgery with composite graft (aortic valve and ascending aorta) gives the best survival rate.

摘要

作者介绍了一名患有马凡氏综合征(MS)和主动脉瓣关闭不全的患者的临床病史,该患者22岁时死亡,目的是说明临床病程并回顾该问题的处理方法。已知患有MS且因主动脉瓣环扩张导致心力衰竭的患者早期死亡率几乎为100%。作者得出结论,对这些患者进行超声心动图随访对其药物和手术治疗至关重要,使用β受体阻滞剂是最佳药物治疗方法,采用复合移植物(主动脉瓣和升主动脉)进行预防性手术可获得最佳生存率。

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