当前位置: X-MOL 学术Circ. Res. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Classification, Epidemiology, and Global Burden of Cardiomyopathies
Circulation Research ( IF 20.1 ) Pub Date : 2017-09-15 , DOI: 10.1161/circresaha.117.309711
William J. McKenna 1 , Barry J. Maron 1 , Gaetano Thiene 1
Affiliation  

In the past 25 years, major advances were achieved in the nosography of cardiomyopathies, influencing the definition and taxonomy of this important chapter of cardiovascular disease. Nearly, 50% of patients dying suddenly in childhood or adolescence or undergoing cardiac transplantation are affected by cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic, restrictive, and noncompacted) and added to update the World Health Organization classification. Myocarditis has also been named inflammatory cardiomyopathy. Extraordinary progress accomplished in molecular genetics of inherited cardiomyopathies allowed establishment of dilated cardiomyopathy as mostly cytoskeleton, force transmission disease; hypertrophic–restrictive cardiomyopathies as sarcomeric, force generation disease; and arrhythmogenic cardiomyopathy as desmosome, cell junction disease. Channelopathies (short and long QT, Brugada, and catecholaminergic polymorphic ventricular tachycardia syndromes) should also be considered cardiomyopathies because of electric myocyte dysfunction. Cardiomyopathies are easily diagnosed but treated only with palliative pharmacological or invasive therapy. Curative therapy, thanks to insights into the molecular pathogenesis, has to target the fundamental mechanisms involved in the onset and progression of these conditions.


中文翻译:

心肌病的分类,流行病学和全球负担

在过去的25年中,心肌病的影像学取得了重大进展,影响了这一重要的心血管疾病章节的定义和分类。几乎有50%的儿童或青少年突然死亡或接受心脏移植手术时会受到心肌病的影响。已发现新的心肌病(致心律失常,限制性和非致密性),并已添加这些新的心肌病以更新世界卫生组织的分类。心肌炎也被称为炎症性心肌病。遗传性心肌病的分子遗传学方面取得了非凡的进展,使扩张型心肌病主要是细胞骨架,力传递疾病的建立。肥大性-限制性心肌病,为肌节,发力疾病;和心律失常性心肌病,如桥粒,细胞连接疾病。通道病变(短时和长时QT,Brugada和儿茶酚胺能性多形性室性心动过速综合征)也应被视为心肌病,原因是肌细胞功能异常。心肌病很容易诊断,但仅可采用姑息性药理或侵入性疗法进行治疗。由于对分子发病机理的深入了解,治疗性疗法必须针对这些疾病的发作和发展所涉及的基本机制。
更新日期:2017-09-15
down
wechat
bug