心肌病是儿科严重心脏病之一,预后差.近年来儿科心肌病流行病学研究取得许多进展,揭示了儿科心肌病的病因、病程及临床特点,对临床处理产生积极的影响.同时随着分子遗传学的迅速发展及心肌病基础及临床研究的进展,对心肌病的认识更深入,原有的心肌病定义及分类已有修改的必要.目前儿童心肌病病死率及病残率仍较高,关注儿童心肌病的病因诊断,加强多中心、多学科心肌病临床及基础研究对改善心肌病患儿预后具有重要意义.%Cardiomyopathy is one of the most serious pediatric heart diseases with poor prognosis. In recent years, great progress has been achieved in epidemiologic investigations, which have revealed the etiology, disease course and clinical characteristics of pediatric cardiomyopathy, and played an active role in guiding clinical practice. With the fast development of molecular genetics and advances in basic and clinical researches on cardiomyopathy, deeper insight has been gained into cardiomyopathy, which gives rise to a necessary revision on the definition and classification of cardiomyopathy. Since the mortality and morbidity of pediatric cardiomyopathy remain high at present, more attention must be paid to the etiological diagnosis, multi-disciplinary and multi-center cooperation on basic and clinical studies on pediatric cardiomyopathy, in order to improve the prognosis of children with cardiomyopathy.
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