右室心肌病以发作性晕厥、右室源性室速、右室扩张和右心功能减退或衰竭为特征。由于超声心动图、心脏核素、临床电生理及心内膜心肌活检等检查的广泛应用,近年来本病报告增多,且为年轻人猝死的又一重要原因。本文收集截止1988年的文献,介绍右室心肌病病因、病理基础、临床表现、心电图特征、超声心动图、心脏核素、电生理学、心内膜心肌活检等方面研究进展,并综合诊断与治疗方法。 Right ventricular cardiomyopathy is characterized by episodic syncope, right ventricular-derived ventricular tachycardia, right ventricular dilatation, and impaired or obscured right heart function. Due to the wide range of applications of echocardiography, cardiac nuclide, clinical electrophysiology and endomyocardial biopsy, the report of this disease has increased in recent years and is another important cause of sudden death among young people. This article collected until 1988, the literature introduces the etiology, pathology, right ventricular cardiomyopathy, clinical manifestations, electrocardiogram characteristics, echocardiography, cardiac nuclide, electrophysiology, endomyocardial biopsy and other aspects of research, and comprehensive diagnosis and treatment method.