致心律失常性左室心肌病的研究进展  

Arrhythmogenic Left Ventricular Cardiomyopathy

作　　者：韩雅洁 范小倩 巩彩霞 赵子龙 乔成栋[2] HAN Yajie;FAN Xiaoqian;GONG Caixia;ZHAO Zilong;QIAO Chengdong(The First Clinical Medical College of Lanzhou University,Lanzhou 730000,Gansu,China;Department of Geriatrics,The First Hospital of Lanzhou University,Lanzhou 730000,Gansu,China)

机构地区：[1]兰州大学第一临床医学院,甘肃兰州730000 [2]兰州大学第一医院老年病科,甘肃兰州730000

出　　处：《心血管病学进展》2021年第4期342-346,共5页Advances in Cardiovascular Diseases

摘　　要：致心律失常性左室心肌病是以左室心肌逐渐被脂肪和纤维组织替代的罕见遗传性心肌病,易发生危及生命的心律失常和心源性猝死。临床上主要依靠家族遗传病史、心电图、影像学和心内膜活检等作出诊断。有时起病隐匿,部分患者以猝死为首发症状,尸检后才作出诊断。治疗包括抗心律失常药物、治疗心力衰竭药物、射频导管消融、植入植入型心律转复除颤器以及心脏移植等。致心律失常性左室心肌病在病因、发病机制、诊断和治疗方面仍存在许多有待解决的问题,引起全社会的广泛关注。Arrhythmogenic left ventricular cardiomyopathy(ALVC)is a rare hereditary cardiomyopathy in which the left ventricular myocardium is gradually replaced by fat and fibrous tissue.It is prone to life-threatening arrhythmias and sudden cardiac death.Clinical diagnosis mainly depends on family genetic history,electrocardiogram,imaging,endocardial biopsy and so on.Sometimes the onset is insidious,some patients have sudden death as the first symptom and the diagnosis is made after autopsy.Treatment includes antiarrhythmic drugs,drugs for heart failure,radiofrequency catheter ablation,implantable cardioverter defibrillator,heart transplantation and so on.There are still many problems to be solved in the etiology,pathogenesis,diagnosis and treatment of ALVC,which has aroused widespread concern of the whole society.

关 键 词：致心律失常性左室心肌病 发病机制 流行病学 诊断 治疗 

分 类 号：R54[医药卫生—心血管疾病]