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神经肌肉疾病与临床电生理学家的作用。

Neuromuscular Disorders and the Role of the Clinical Electrophysiologist.

机构信息

Beth Israel Deaconess Medical Center, Boston, Massachusetts.

Beth Israel Deaconess Medical Center, Boston, Massachusetts.

出版信息

JACC Clin Electrophysiol. 2017 Oct;3(10):1069-1079. doi: 10.1016/j.jacep.2017.04.023. Epub 2017 Sep 13.

DOI:10.1016/j.jacep.2017.04.023
PMID:29759488
Abstract

Cardiac involvement is common and may be the presenting or predominant manifestation in a variety of neuromuscular disorders, most notably the inherited muscle disorders, or muscular dystrophies. Cardiac manifestations of the neuromuscular disorders result from pathological involvement of the myocardium and the cardiac conduction system, with resulting cardiomyopathy or rhythm disturbances including supraventricular arrhythmias, life-threatening ventricular arrhythmias, and sudden cardiac death. Many of these neuromuscular disorders are rare and may be unrecognized by even experienced specialists in internal and cardiovascular medicine. Furthermore, the initial cardiac manifestations in these patients are often asymptomatic. The goal of this investigation is to review the scope of cardiac conduction defects and rhythm disturbances in these disorders and to propose some practical recommendations for arrhythmia monitoring and management of these patients.

摘要

心脏受累很常见,可能是多种神经肌肉疾病的表现或主要表现,尤其是遗传性肌肉疾病或肌营养不良症。神经肌肉疾病的心脏表现源于心肌和心脏传导系统的病理性受累,导致心肌病或节律紊乱,包括室上性心律失常、危及生命的室性心律失常和心脏性猝死。这些神经肌肉疾病中有许多是罕见的,即使是内科和心血管医学方面经验丰富的专家也可能不认识。此外,这些患者的初始心脏表现通常无症状。本研究旨在综述这些疾病中心脏传导缺陷和节律紊乱的范围,并为这些患者的心律失常监测和管理提出一些实用建议。

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