Pathogenic role of mitochondrial DNA mutations in heart failure: clinical features, mechanisms, and therapeutic prospects

Heart failure (Heart failure, HF) is a complex clinical syndrome caused by any abnormality in the structure or function of the heart, resulting in impaired ventricular filling or ejection capacity, with mitochondrial dysfunction recognized as one of the key pathological foundations. In recent years, numerous studies have demonstrated that mitochondrial DNA (mtDNA) mutations play a significant role in cardiomyopathy and HF; however, systematic understanding of their modes of action in disease progression remains limited. Most studies have attributed the pathogenic effects of mtDNA mutations to impaired energy metabolism, emphasizing the consequences of defective oxidative phosphorylation and insufficient ATP production on myocardial function. Emerging evidence, however, indicates that mtDNA mutations also contribute to the development and progression of HF by inducing reactive oxygen species accumulation, disrupting mitochondrial structural and dynamic homeostasis, and activating innate immune inflammatory signaling pathways. Furthermore, variations in mtDNA mutation load and heteroplasmy levels constitute an important molecular basis for the diverse clinical phenotypes of HF, although the underlying mechanisms have yet to be systematically integrated. This review comprehensively summarizes the pathogenic mechanisms of cardiac mtDNA mutations and their heteroplasmy in HF, with particular emphasis on the intrinsic links among mitochondrial metabolic reprogramming, oxidative stress, immune activation, and myocardial remodeling, and outlines potential diagnostic and therapeutic strategies based on mitochondrial dysfunction and mtDNA stability.

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Publication Details

Journal
Frontiers in Cardiovascular Medicine
Published
2026-04-01
DOI
https://doi.org/10.3389/fcvm.2026.1781927
Primary Topic
Mitochondrial Function and Pathology
Type
article
Field-Weighted Citation Impact
0.00

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article

Pathogenic role of mitochondrial DNA mutations in heart failure: clinical features, mechanisms, and therapeutic prospects

M Ni, Chenguang Yao, Xueli Wang, Sini Kang et al.
Frontiers in Cardiovascular Medicine
Mitochondrial Function and Pathology
article

Pathogenic role of mitochondrial DNA mutations in heart failure: clinical features, mechanisms, and therapeutic prospects

M Ni, Chenguang Yao, Xueli Wang, Sini Kang, Aijia Zheng, Yuqing Peng, Yanhong Wei, Wenqing Jia, Hang Zheng
article en

Abstract

Heart failure (Heart failure, HF) is a complex clinical syndrome caused by any abnormality in the structure or function of the heart, resulting in impaired ventricular filling or ejection capacity, with mitochondrial dysfunction recognized as one of the key pathological foundations. In recent years, numerous studies have demonstrated that mitochondrial DNA (mtDNA) mutations play a significant role in cardiomyopathy and HF; however, systematic understanding of their modes of action in disease progression remains limited. Most studies have attributed the pathogenic effects of mtDNA mutations to impaired energy metabolism, emphasizing the consequences of defective oxidative phosphorylation and insufficient ATP production on myocardial function. Emerging evidence, however, indicates that mtDNA mutations also contribute to the development and progression of HF by inducing reactive oxygen species accumulation, disrupting mitochondrial structural and dynamic homeostasis, and activating innate immune inflammatory signaling pathways. Furthermore, variations in mtDNA mutation load and heteroplasmy levels constitute an important molecular basis for the diverse clinical phenotypes of HF, although the underlying mechanisms have yet to be systematically integrated. This review comprehensively summarizes the pathogenic mechanisms of cardiac mtDNA mutations and their heteroplasmy in HF, with particular emphasis on the intrinsic links among mitochondrial metabolic reprogramming, oxidative stress, immune activation, and myocardial remodeling, and outlines potential diagnostic and therapeutic strategies based on mitochondrial dysfunction and mtDNA stability.

Frontiers in Cardiovascular MedicineVol. 13
Hubei Water Resources Research Institute (CN), Hubei University of Technology (CN)
National Natural Science Foundation of China, Hubei University of Technology, Hubei University
Good health and well-being
Openalex Percentile: Top 36%
Mitochondrial Function and Pathology
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