心肌病新的定义及分类.pdfVIP

· 7l2· 国际内科学杂志2007年12月第34卷第12期 心肌病新的定义及分类 王立军 综述； 马 虹 审校 摘要：美国心脏病协会(AHA)提出了新的心肌病的定义和分类。新定义的心肌病不仅包括心肌的机 械功能障碍，还包括心肌离子通道病等。根据器官受累情况，AHA将心肌病分为原发性和继发性两大 类。前者病变局限于心肌，为数较少；后者为全身性疾病的一部分。原发性心肌病又分为遗传性、混合 性(遗传和非遗传)和获得性3个亚类。混合性包括扩张型心肌病和原发性限制型心肌病；获得性者包 括心肌炎、应激性心肌病( Fako一 rsubo综合征)等。 关键词：心肌病； 定义；分类 中图分类号：11541．4 文献标识码：A 文章编号：1004—2369(2007)12-0712-04 New definition and classification of the cardiomyopathies WANG Li-jun‘，MA Hong 、1 Department of Cardiology The Affiliated Baoan Hospital of Nanfang Medical University，Shenzhen 518101，China；2 Depart— ． ， ment of Cardiology，The First Affiliated Hospital of Sun Yat—sen University，Guangzhou 5 10080，China Abstract：The American Heart Association(AHA)proposed a new definition and classification of cardiomyop— athies The new definition involved not only myocardial mechanical dysfunction，but also cardiac primary elec— trical disease(ion channelopalhies)、Car(1iomy。pathies were divided into two nlajor groups based on the situa— tion of predominant organ involved．Primary car(1i()my。paIhies were those solely or predominantly confined to myocardium and relatively few in num1)er．Seconda~,cardiomyopathies showed pathological myocardial involve— ment as part of a large number and variety of generalized systemic(muhi—organ)disorders．Primary cardio— myopathies were further divided into three groups：genetic，mixed(genetic and nongenetie)and acquired．The mixed cardiomyopathies included dilated cardiomyopathy and primaDrestrictive cardiomyopathy．The acquired eardiomyopathies included myocarditis and stress cardiomyopathy(Tako—Tsubo syndrome)and SO on． Key words：Cardiomyopathy；Definition；Classification (Int J Intern Med，2007，34：712—715) 2006年4月美国心脏病协会(AHA)组织有关 至分子水平。心肌离子通道病的定义为编码心肌离 专家对心肌病进行重新定义和分类¨ 。本文对其 子通道亚单位的基因发生突变／表达及膜定位异常 作一简要介绍。