Zhuge Ruiqi, Zhou Rong, Ni Xinhai
Department of Cardiology,Fuwai Hospital,National Center for Cardiovascular Disease,CAMS and PUMC,Beijing 100037,China.
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2017 Apr 20;39(2):290-295. doi: 10.3881/j.issn.1000-503X.2017.02.021.
Mitochondrial cardiomyopathy (MCM) is a series of myocardial conditions characterized by abnormal heart-muscle structure,function,or both,secondary to genetic defects involving the mitochondrial respiratory chain,in the absence of concomitant coronary artery disease,hypertension,valvular disease,or congenital heart disease. MCM patients typically have hypertrophic or dilated cardiomyopathy. Arrhythmias and left ventricular myocardial noncompaction are less common,and heart failure may occur as the first symptom in some patients. Since MCM patients often have symptoms of multiple organ involvement,the symptoms are not specific and the diagnosis can be difficult. Thus,awareness of this disease must be increased in clinical settings. Treatments for MCM are mostly supportive and nonspecific. In this review,we summarize new advances in the diagnosis and management of MCM,with an improve the clinical management of this disease.
线粒体心肌病（MCM）是一系列心肌疾病，其特征是心肌结构、功能异常或两者皆有，继发于涉及线粒体呼吸链的基因缺陷，且无合并冠状动脉疾病、高血压、瓣膜病或先天性心脏病。MCM患者通常患有肥厚型或扩张型心肌病。心律失常和左心室心肌致密化不全较少见，心力衰竭可能是部分患者的首发症状。由于MCM患者常出现多器官受累症状，症状不具特异性，诊断可能困难。因此，临床环境中必须提高对该疾病的认识。MCM的治疗大多是支持性的且不具特异性。在本综述中，我们总结了MCM诊断和管理方面的新进展，以期改善该疾病的临床管理。
