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X 连锁先天性肌营养不良症的心肌病早期发病和猝死的一级预防。

Early onset of cardiomyopathy and primary prevention of sudden death in X-linked Emery-Dreifuss muscular dystrophy.

机构信息

Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, Monaldi Hospital, Naples, Italy.

出版信息

Neuromuscul Disord. 2010 Mar;20(3):174-7. doi: 10.1016/j.nmd.2009.12.004. Epub 2010 Feb 10.

DOI:10.1016/j.nmd.2009.12.004
PMID:20149661
Abstract

We report the case of 14-year-old boy with X-linked Emery-Dreifuss muscular dystrophy who developed sick sinus syndrome and required placement of an implantable intracardiac cardioverter-defibrillator (ICD) to prevent sudden death. He demonstrated no significant risk factors for sudden death such as depressed left ventricular ejection fraction, or spontaneous or inducible ventricular tachycardia. One month after implantation, the patient experienced one appropriate ICD discharge.

摘要

我们报告了一例 14 岁男孩,患有 X 连锁肌萎缩侧索硬化症,发展为病态窦房结综合征,需要植入植入式心脏复律除颤器(ICD)以预防猝死。他没有明显的猝死风险因素,如左心室射血分数降低、自发性或诱发性室性心动过速。植入后 1 个月,患者经历了一次适当的 ICD 放电。

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