Abstract: Background　Pediatric cardiomyopathy is a disease with poor prognosis，high mortality and disability. Over the years，researchers at home and abroad have actively explored and researched it，and published a large number of related literature. Objective　To analyze the current status and trends of domestic and overseas researches on pediatric cardiomyopathy，and provide a reference for related research. Methods　English literatures on pediatric cardiomyopathy were collected from the Web of Science Core Collection （SCI-Expanded，SSCI，ESCI） and PubMed database，and Chinese ones from CNKI and Wanfang Data Knowledge Service Platform. Literatures published on those databases before April 12，2018 were retrieved. The missing information is supplemented by Notepad++ software. The duplicate Chinese and English literatures were removed using EndNote X7 and CiteSpace 5.2.R1，respectively. Bibliometric analysis of journals containing those literatures was performed using Bibexcel. CiteSpace 5.2.R1 was also used to analyze the publication year，country，institution，journals，research hotspot and frontier of those literatures. Results　A total of 9 816 English literatures in the field of childhood cardiomyopathy were included，which showed an overall upward trend in number but a downward trend from 2014 to 2017. A total of 585 Chinese literatures were included，showing a downward trend in number from 2014 to 2017. And 362 English literatures were published in China，and the number was on the rise，with a large increase from 2015 to 2017.From 1997 to 2017，160 countries or regions published pediatric cardiomyopathy-related literatures，of which the United States ranked the first in number，and China the 9th. The top five research institutions with largest number of English literature were mainly located in the United States，among which Harvard University held the largest number with a more intensive and broader cooperation with other research institutions. Among Chinese research organizations which published Chinese or English literatures about pediatric cardiomyopathy，Shanghai Jiao Tong University published the most literatures with a sparse and relatively narrower cooperation with other research organizations. A total of 9 816 English literatures were published in 1 769 journals，and 585 Chinese literatures in 321 ones. The hotspots or cluster labels in English literatures involved extracorporeal membrane oxygenation，cardiovascular disease，genotype-phenotype correlation，valuable prognostic tool and clinical consequence，while Chinese literatures largely focused on anthracycline-induced cardiotoxicity，ATPase-deficient mitochondrial inner membrane protein ATAD3A，myosin binding protein C，functional mutation，TAZ mutation，childhood leukemia，gene product expression，chronic condition and familial atrial septal defect. The researches about scientific management，clinical outcome，cardiovascular magnetic resonance，ventricular assist devices，genetics and corresponding guidelines were frontier of pediatric cardiomyopathy. Conclusion　Researches about pediatric cardiomyopathy have achieved remarkable results，and the researches in China are gradually meeting the international standard. However，our research level still requires improvement. Inter-institution cooperation should be emphasized in the absence of a stable large-scale research direction.

Key words: Cardiomyopathies, Child, Bibliometrics, Visualization analysis

摘要： 背景　儿童心肌病是一种预后差、病死率及致残率较高的疾病。历年来，国内外研究者对其进行了积极的探索与研究，发表了大量相关文献。目的　分析国内外儿童心肌病研究的现状及趋势，为相关研究提供参考。方法　英文文献以Web of Science核心合集数据库（SCI-Expanded、SSCI、ESCI）、PubMed数据库为数据来源，中文文献以中国知网、万方数据知识服务平台为数据来源，检索儿童心肌病相关文献，检索时间为建库至2018-04-12。采用Notepad++软件补充缺失信息，中、英文文献分别采用EndNote X7软件、CiteSpace 5.2.R1软件去重处理。采用Bibexcel软件统计刊载文献的期刊。采用CiteSpace 5.2.R1软件分析文献的发表年代、国家/地区、研究机构、期刊、研究热点及前沿。结果　共纳入9 816篇儿童心肌病领域的英文文献，数量整体呈上升趋势，2014—2017年呈下降趋势；共纳入585篇中文文献，2014—2017年呈下降趋势；英文文献中有362篇为我国发表，文献数量呈上升趋势，2015—2017年增加幅度较大。1997—2017年共160个国家/地区发表文献，美国的文献数量最多，中国位居第9名。英文文献数量前5名的研究机构主要位于美国，文献数量最多的为Harvard University（美国），研究机构合作网络较为密集，与其他机构合作较多；中文文献及我国发表的英文文献的数量最多的研究机构为上海交通大学，研究机构合作网络稀疏，与其他机构合作较少。9 816篇英文文献共发表在1 769种期刊，585篇中文文献共发表在321种期刊。英文文献的研究热点为体外膜氧合、心血管疾病、基因型-表型相关性、有价值的预后工具和临床结局；国内中英文文献的聚类标签即研究热点为蒽环霉素诱导的心脏毒性、ATPase缺陷型线粒体内膜蛋白ATAD3A、肌球蛋白结合蛋白C、功能突变、TAZ突变、儿童白血病、基因产物表达、慢性病和家族性房间隔缺损。儿童心肌病的研究前沿为科学管理、临床结局、心血管磁共振、心室辅助装置、遗传学与相应指南。结论　儿童心肌病研究成果显著，我国该领域的研究逐渐面向国际，但研究水平仍待提升，未形成稳定的大规模研究方向，应注重机构间合作。

关键词: 心肌疾病, 儿童, 文献计量学, 可视化分析