Quan Junjun, Gerber Dustin, Li Ang, Huang Xupei, Tian Jie
Department of Anesthesiology Children's Hospital of Chongqing Medical University National Clinical Research Center for Child Health and Disorders Ministry of Education Key Laboratory of Child Development and Disorders Chongqing Key Laboratory of Pediatrics Chongqing China.
Department of Cardiology Children's Hospital of Chongqing Medical University Chongqing China.
Pediatr Discov. 2023 Jun 9;1(1):e7. doi: 10.1002/pdi3.7. eCollection 2023 Jun.
Cardiomyopathies comprise a group of disorders wherein the primary defect is in cardiac myocytes. The common forms of pediatric cardiomyopathies, classified according to their morphological and functional manifestations, include dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM), hypertrophic cardiomyopathy (HCM), and others. Cardiac gene mutations caused abnormal myofibril Ca sensitivity may be involved in the underlying molecular mechanisms of cardiomyopathies. Thus far, no effective treatment for cardiomyopathies has been developed, especially for HCM and RCM in which diastolic dysfunction occurs early and followed by diastolic heart failure. Our laboratory is among the first in the field to investigate the mechanisms underlying various cardiomyopathies and search for the treatment for these disorders. In the past, we and other researchers have found that (-)-epigallocatechin-3-gallate (EGCg), the major biomedical polyphenol extracted from green tea, possess multiple therapeutic effects on protecting cardiac function and correcting impaired relaxation. Given its therapeutic effects, EGCg might be a potential drug candidate for administration to patients with cardiomyopathy and heart failure. In this review, we will discuss the molecular mechanisms associated with the pathogenesis of diastolic dysfunction and summarize the pharmacological effects of EGCg on experimental animals and pediatric patients with cardiomyopathies and diastolic dysfunction.
心肌病是一组主要病变在于心肌细胞的疾病。根据形态学和功能表现分类,小儿心肌病的常见类型包括扩张型心肌病(DCM)、限制型心肌病(RCM)、肥厚型心肌病(HCM)等。心脏基因突变导致肌原纤维钙敏感性异常可能参与了心肌病的潜在分子机制。迄今为止,尚未开发出针对心肌病的有效治疗方法,尤其是对于早期出现舒张功能障碍并随后发展为舒张性心力衰竭的HCM和RCM。我们实验室是该领域最早研究各种心肌病潜在机制并寻找这些疾病治疗方法的实验室之一。过去,我们和其他研究人员发现,从绿茶中提取的主要生物活性多酚(-)-表没食子儿茶素-3-没食子酸酯(EGCg)对保护心脏功能和纠正舒张功能受损具有多种治疗作用。鉴于其治疗效果,EGCg可能是一种潜在的药物候选物,可用于心肌病和心力衰竭患者。在这篇综述中,我们将讨论与舒张功能障碍发病机制相关的分子机制,并总结EGCg对实验动物以及患有心肌病和舒张功能障碍的小儿患者的药理作用。