心肌病定义及分型标准修订和争议

Controversial and New Suggestions in Definition and Classification of Cardiomyopathy

心肌病是一大类病因繁杂,表现多样的心肌疾病。一直以来,对于心肌病的病因和发病机制认识不足,带来了对于心肌病定义和分类的模糊不清。近年来,随着分子生物学技术在心肌病研究中的应用,使我们对于心肌病的认识大大提高。基于分子心脏病学的进展,美国心脏协会和欧洲心脏学会相继发布了心肌病新的定义和分类修订建议,但二者存在明显的差异。这造成了在心肌病定义和分类上的分歧。了解心肌病定义和分类的发展过程将有助于我们提高对于心肌病病因和发病机制的认识和正确地在临床中对于心肌病进行科学的分类诊断。

Cardiomyopathy is a complex group of heart muscle diseases, with multiple etiologies and heterogeneous phenotypic expressions. Inadequate understanding of the causes and the pathogenesis of cardiomyopathy, has caused confusion in the definitions of and classifications of cardiomyopathy. In recent years the application of molecular biology in cardiomyopathy research has greatly enhanced the understanding of cardiomyopathy. Based on the progress of molecular cardiology, both the American Heart Association and the European Society of Cardiology have issued a new definition and classification proposal for cardiomyopathy. There is, however, an obvious difference between the two different societies. This has resulted in worldwide controversy over the new definition and classification of cardiomyopathy. Therefore, understanding the development process of the definition and classification used in cardiomyopathy will raise our awareness about the etiologies and pathogenesis in cardiomyopathy and apply scientific classification in the diagnosis of cardiomyopathy.