PDF(Pubmed)
Abstract：
Hypertrophic cardiomyopathy (HCM) is a heart condition characterized by cellular and metabolic dysfunction, with mitochondrial dysfunction playing a crucial role. Although the direct relationship between genetic mutations and mitochondrial dysfunction remains unclear, targeting mitochondrial dysfunction presents promising opportunities for treatment, as there are currently no effective treatments available for HCM. This review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Extension for Scoping Reviews guidelines. Searches were conducted in databases such as PubMed, Embase, and Scopus up to September 2023 using \"MESH terms\". Bibliographic references from pertinent articles were also included. Hypertrophic cardiomyopathy (HCM) is influenced by ionic homeostasis, cardiac tissue remodeling, metabolic balance, genetic mutations, reactive oxygen species regulation, and mitochondrial dysfunction. The latter is a common factor regardless of the cause and is linked to intracellular calcium handling, energetic and oxidative stress, and HCM-induced hypertrophy. Hypertrophic cardiomyopathy treatments focus on symptom management and complication prevention. Targeted therapeutic approaches, such as improving mitochondrial bioenergetics, are being explored. This includes coenzyme Q and elamipretide therapies and metabolic strategies like therapeutic ketosis. Understanding the biomolecular, genetic, and mitochondrial mechanisms underlying HCM is crucial for developing new therapeutic modalities.
摘要：
肥厚型心肌病（HCM）是一种以细胞和代谢功能障碍为特征的心脏病，线粒体功能障碍起着至关重要的作用。尽管基因突变与线粒体功能障碍之间的直接关系尚不清楚，靶向线粒体功能障碍为治疗提供了有希望的机会，因为目前尚无有效的HCM治疗方法。本审查遵循了系统审查的首选报告项目和范围审查的荟萃分析扩展指南。在PubMed等数据库中进行了搜索，Embase,和截至2023年9月的Scopus使用“网格术语”。还包括相关文章的书目参考。肥厚型心肌病（HCM）受离子稳态的影响，心脏组织重塑，代谢平衡,基因突变,活性氧调节，和线粒体功能障碍。后者是一个共同的因素，不管是什么原因，并与细胞内钙处理有关，能量和氧化应激，和HCM诱导的肥大。肥厚型心肌病的治疗侧重于症状管理和并发症的预防。有针对性的治疗方法，比如改善线粒体生物能学，正在探索。这包括辅酶Q和埃拉米肽治疗和代谢策略，如治疗性酮症。了解生物分子，遗传,和线粒体机制对于开发新的治疗方式至关重要。
