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法布瑞病导致的肥厚型心肌病扩张期伴心房颤动和室性心动过速。

Dilated phase of hypertrophic cardiomyopathy caused by Fabry disease with atrial flutter and ventricular tachycardia.

机构信息

Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-Cho, Chuoh-Ku, Kobe 650-0017, Hyogo, Japan.

出版信息

J Cardiol. 2009 Aug;54(1):139-43. doi: 10.1016/j.jjcc.2008.10.004. Epub 2008 Dec 4.

Abstract

We describe a case of a 60-year-old male with dilated phase of hypertrophic cardiomyopathy caused by Fabry disease. He was diagnosed to have a cardiac variant of Fabry disease by an enzyme assay and a right ventricular endomyocardial biopsy which revealed specific features of this disease and cardiac involvement was the sole manifestation. He has developed dilated cardiomyopathy with sustained atrial flutter and frequent non-sustained ventricular tachycardia requiring isthmus ablation and cardiac resynchronization therapy with defibrillator.

摘要

我们描述了一例由法布病引起的扩张型肥厚型心肌病的 60 岁男性患者。通过酶测定和右心室心内膜心肌活检诊断为心脏型法布病,该活检显示出该病的特定特征,且心脏受累是唯一表现。他已发展为扩张型心肌病伴持续性心房颤动和频繁的非持续性室性心动过速,需要进行峡部消融和带除颤器的心脏再同步治疗。

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