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[马凡综合征的心血管异常]

[Cardiovascular abnormalities in Marfan syndrome].

作者信息

Fast J H, Boers G H, Meijers-Jacobs S, Cruysberg J R, Hamel B C

机构信息

Afd. Cardiologie, De Wever-Ziekenhuis, Heerlen.

出版信息

Ned Tijdschr Geneeskd. 1992 Jun 27;136(26):1259-63.

PMID:1620255
Abstract

During the period February to December 1990, 52 adult patients were referred to our clinic for evaluation of the presence of the Marfan syndrome. In 24 out of 52 patients the Marfan syndrome was diagnosed. Cardiac abnormalities were found in all patients: mitral insufficiency because of mitral valve prolapse (83%), aortic dilatation (67%), aortic insufficiency (38%), tricuspid valve insufficiency with or without tricuspid valve prolapse (17%) and atrial septal defect (4%). In 3 patients an aneurysm of the ascending aorta was found. Early recognition of the Marfan syndrome is relevant for prevention of the life threatening complication of aortic dissection. In patients with valve abnormalities endocarditis prophylaxis is advised. A Marfan outpatient clinic offers optimal diagnostic possibilities.

摘要

在1990年2月至12月期间,52名成年患者被转诊至我们的诊所,以评估是否患有马凡综合征。52名患者中有24名被诊断为马凡综合征。所有患者均发现心脏异常:二尖瓣脱垂导致二尖瓣关闭不全(83%)、主动脉扩张(67%)、主动脉瓣关闭不全(38%)、伴有或不伴有三尖瓣脱垂的三尖瓣关闭不全(17%)以及房间隔缺损(4%)。3名患者发现升主动脉瘤。早期识别马凡综合征对于预防危及生命的主动脉夹层并发症至关重要。对于有瓣膜异常的患者,建议进行心内膜炎预防。马凡门诊诊所提供了最佳的诊断可能性。

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