2006-2016年国内多中心1823例住院儿童心肌病的调查分析

A multicenter study of 1823 cases of hospitalized childhood cardiomyopathy from 2006 to 2016

目的分析国内16家医院近10年心肌病患儿的临床资料,为心肌病患儿的精准诊治提供参考。方法回顾性分析2006年7月至2016年6月国内16家医院儿科住院治疗的1823例心肌病患儿的性别、年龄、入院时心功能状态、入院时伴发疾病、心电图、超声心动图、实验室检查、基因检测等资料,电话随访了解心肌病患儿出院后情况。结果 1823例患儿占同期儿科住院患儿的0.1%,其中扩张型心肌病最常见(33.57%),其次为心内膜弹力纤维增生症(27.81%)和左室心肌致密化不全(11.03%);发病年龄以0～6月龄最多(27.3%);其次为1～3岁(22.7%)。入院时NYHA分级Ⅲ级及以上、Ross分级7分及以上者占43.2%,心源性休克的发生率为4.3%;住院期间栓塞事件的发生率为0.6%,以扩张型心肌病和左室心肌致密化不全为主。36.3%的心肌病患儿存在心律失常,以期前收缩、心动过速、传导阻滞及预激综合征最常见。18.3%的心肌病患儿超声心动图检查中有心包积液,瓣膜反流的发生率为77.6%,以二尖瓣反流最多。总体病死率为2.80%。结论国内儿童心肌病发病人数逐年增多,心肌病的类型以扩张型心肌病、心内膜弹力纤维增生症和左室心肌致密化不全为主;发病年龄3岁内为多,常伴有心律失常;仅有3.24%的心肌病患儿完善基因检测;需要进一步提高儿童心肌病患儿的诊治水平。

Objective To analyze the clinical data of hospitalized children with cardiomyopathy in 16 hospitals in China in nearly 10 years,and to provide evidence for its precision diagnosis and treatment. Methods The clinical data of hospitalized children treated in 16 pediatric departments in China from July 2006 to June 2016 were analyzed retrospectively,which included the gender,age,cardiac function on admission,concomitant disease on admission,electrocardiogram,echocardiograph,laboratory examinations and genetic tests;Their conditions after discharge were followed up by making telephone calls. Results A total of 1823 hospitalized children with cardiomyopathy were admitted,accounting for 0.1% of pediatric hospitalized children during the same period. Dilated cardiomyopathy(DCM)was the most common kind of cardiomyopathy(33.57%),followed by endocardial fibroelastosis(27.81%)and left ventricular noncompaction(LVNC)(11.03%). The age at onset was diverse. The largest percentage was 0-6 month-old children(27.3%),followed by 1-3 year-olds(22.7%). A total of 43.2% of the patients’ cardiac function was NYHA class Ⅲ or above and Ross grade7 or above,and 4.3% had cardiogenic shock;the incidence of thromboembolism during hospitalization was 0.6%,mainly in patients with DCM and LVNC. Arrhythmia was presented in 36.3% of patients,and the most common types included premature beats,tachycardia,conduction block,and pre-excitation syndrome. Echocardiography revealed 18.3% of the patients had pericardial effusion. The incidence of valvular regurgitation was 77.6%,and mitral regurgitation was more common. The overall mortality rate was 2.80%. Conclusion The hospitalized children with cardiomyopathy have increased year by year. The common types of cardiomyopathy are DCM,EFE,and LVNC. The age of onset is under age 3 in most children with cardiomyopathy,and arrhythmia is the most common complication. Only 3.24% of the patients completed genetic test. It is necessary to further improve the diagnosis and treatment of childhood cardiomyopathy.