小儿扩张型心肌病病因及临床分析

摘要

目的 探讨小儿扩张型心肌病(dilated cardiomyopathy,DCM)的发病年龄、病因、心功能状况及心律失常的特点.方法 对我院2002年1月至2011年12月收治的112例DCM患儿的发病年龄、病因、临床特点进行分析,并按心功能Ⅰ、Ⅱ、Ⅲ、Ⅳ级分为4组,比较各组心律失常的发生率及类型.结果 112例DCM患儿中,＜1岁确诊的52例(46.42％)；诊断时有心力衰竭107例(95.53％)；能明确病因的25例(22.32％),其中心肌炎16例(14.28％),家族性单纯DCM 5例(4.46％),原发性肉碱缺乏症2例(1.78％),Duchenne肌营养不良1例(0.89％),药物毒性反应(阿霉素)所致DCM 1例(0.89％)；96例(85.71％)患儿出现各种心律失常.按心功能Ⅰ、Ⅱ、Ⅲ、Ⅳ级分级的4组中,各组出现1种心律失常的发生率分别为20.00％、31.25％、36.17％和42.85％,出现2种以上心律失常者的发生率分别为0、18.75％、38.29％和50.00％,出现恶性心律失常的发生率分别为0、12.50％、40.42％和67.85％.心功能Ⅱ、Ⅲ、Ⅳ级组间比较差异有统计学意义(P＜0.05).结论 小儿DCM在1岁以内发病率最高；其主要病因有心肌炎、家族性、代谢缺陷及神经肌肉疾病等；心力衰竭常为患儿的首发症状及主要临床表现；DCM的心律失常发生率高且多样,常为多种心律失常并存,且心律失常的发生与患儿心功能密切相关；小儿DCM各系统栓塞罕见.%Objective To determine the etiology and clinical characteristics of children with dilated cardiomyopathy.Methods 112 children with DCM from January 2002 to December 2011 were studied.The age,cause and the clinical manifestations were analyzed.According to the children with heart function,they were divided into four groups,the incidence and type of arrhythmia were compared.Results In 112 cases of DCM in children,＜ 1 year old diagnosed 52 cases (46.42％),107 cases (95.53％) with heart failure,25 cases (22.32％) with chear causes,96 cases(85.71％) children with a variety of arrhythmias.According to cardiac function in Ⅰ,Ⅱ and Ⅲ and Ⅳ level,they were divided into four groups.The incidence of one kind of arrhythrnia were 20.00％,31.25％,36.17％ and 42.85％.The incidence with two or more kinds of arrhythmia was 0,18.75％,38.29％ and 50.00％ respectively.The incidence of malignant arrhythmia was 0,12.50％,40.42％and 67.85％ in different groups.There was significant differences among the groups of Ⅱ and Ⅲ and Ⅳ level (P ＜ 0 05).Conclusion Children with dilated cardiomyopathy disease younger than I year old had the highest incidence.The major causes were myocarditis,hereditary and congenital metabolic diseases.The incidence of arrhythmia in DCM were high and diverse,often exist for a variety of arrhythmia,and arrhythmia have closely relation with heart function.The embolism in children with DCM was very rare.