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心内膜心肌纤维化所致限制性心肌病

[Restrictive myocardiopathy caused by endomyocardial fibrosis].

作者信息

Mayor Molina G, Villarroel Salcedo M T, Armendáriz Rada J J, Mur Franco J, Peleato Peleato A, Tornos Mas M P, Murtra Ferré M

机构信息

Sección de Cardiología, Hospital San Jorge, Huesca.

出版信息

Rev Esp Cardiol. 1990 Feb;43(2):119-22.

PMID:2326531
Abstract

Endomyocardial fibrosis, an uncommon condition, is the most important cause of restrictive cardiomyopathy. Etiology of endomyocardial fibrosis is unknown, but evidence continues to accumulate that a close connection exists between eosinophilia and endomyocardial disease. Patients with this condition respond poorly to medical treatment, and survival is only for a few months or years when late stage heart disease is present. Surgery is nowadays recommended in these cases. A marked eosinophilia was found in a 58 years old woman that was admitted to the hospital because of a severe congestive cardiac insufficiency. Two-dimensional echocardiographic study and typical angiographic appearance suggested the presence of endomyocardial fibrosis. Endocardectomy and mitral valvular replacement were performed.

摘要

心内膜心肌纤维化是一种罕见疾病,是限制性心肌病的最重要病因。心内膜心肌纤维化的病因尚不清楚,但越来越多的证据表明嗜酸性粒细胞增多症与心内膜疾病之间存在密切联系。患有这种疾病的患者对药物治疗反应不佳,出现晚期心脏病时生存期仅为几个月或几年。如今这些病例建议进行手术治疗。一名58岁女性因严重充血性心力衰竭入院,发现其有明显的嗜酸性粒细胞增多。二维超声心动图检查和典型的血管造影表现提示存在心内膜心肌纤维化。遂进行了心内膜切除术和二尖瓣置换术。

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