线粒体是人体所有细胞的能量工厂，是人体有氧代谢的中心，为细胞新陈代谢所必须。线粒体心肌病是指由于线粒体功能障碍而引起的心肌损害，其特点是心肌结构或/和功能异常，典型的临床表现是肥厚型心肌病、扩张型心肌病、心律失常、左室心肌致密化不全和心功能衰竭。文章综述线粒体心肌病的病理生理、临床表现及可能的治疗方法。“,”Mitochondria are the energy factory of all the cells, the center of aerobic metabolism, and essential for the me-tabolism of cells. Mitochondrial cardiomyopathy refers to myocardial damage caused by mitochondrial dysfunction and is char-acterized by cardiac structural and (or) functional abnormalities. The typical clinical feature of mitochondrial cardiomyopathy is hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmias, noncompaction of left ventricular and heart failure. This article focuses on the pathophysiology, clinical manifestations and possible treatments of mitochondrial cardiomyopathy.