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Open AccessReview

Advanced Evolution of Pathogenesis Concepts in Cardiomyopathies

1
Department of Obstetrics and Gynecology, Kaohsiung Veterans General Hospital, Kaohsiung 813, Taiwan
2
Department of Emergency Medicine, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei 231, Taiwan
3
Department of Emergency Medicine, School of Medicine, Tzu Chi University, Hualien 970, Taiwan
4
Department of Medical Research, Buddhist Tzu Chi General Hospital, Hualien 970, Taiwan
5
Institute of Medicine, Chung Shan Medical University, Taichung 402, Taiwan
6
Chinese Medicine Department, Show Chwan Memorial Hospital, Changhua 500, Taiwan
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
J. Clin. Med. 2019, 8(4), 520; https://doi.org/10.3390/jcm8040520
Received: 7 March 2019 / Revised: 12 April 2019 / Accepted: 12 April 2019 / Published: 16 April 2019
(This article belongs to the Special Issue Cardiovascular and Neurological Emergency)
Cardiomyopathy is a group of heterogeneous cardiac diseases that impair systolic and diastolic function, and can induce chronic heart failure and sudden cardiac death. Cardiomyopathy is prevalent in the general population, with high morbidity and mortality rates, and contributes to nearly 20% of sudden cardiac deaths in younger individuals. Genetic mutations associated with cardiomyopathy play a key role in disease formation, especially the mutation of sarcomere encoding genes and ATP kinase genes, such as titin, lamin A/C, myosin heavy chain 7, and troponin T1. Pathogenesis of cardiomyopathy occurs by multiple complex steps involving several pathways, including the Ras-Raf-mitogen-activated protein kinase-extracellular signal-activated kinase pathway, G-protein signaling, mechanotransduction pathway, and protein kinase B/phosphoinositide 3-kinase signaling. Excess biomechanical stress induces apoptosis signaling in cardiomyocytes, leading to cell loss, which can induce myocardial fibrosis and remodeling. The clinical features and pathophysiology of cardiomyopathy are discussed. Although several basic and clinical studies have investigated the mechanism of cardiomyopathy, the detailed pathophysiology remains unclear. This review summarizes current concepts and focuses on the molecular mechanisms of cardiomyopathy, especially in the signaling from mutation to clinical phenotype, with the aim of informing the development of therapeutic interventions. View Full-Text
Keywords: cardiomyopathy; genetic mutations; cardiac remodeling; apoptosis; fibrosis cardiomyopathy; genetic mutations; cardiac remodeling; apoptosis; fibrosis
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Li, C.-J.; Chen, C.-S.; Yiang, G.-T.; Tsai, A. .-Y.; Liao, W.-T.; Wu, M.-Y. Advanced Evolution of Pathogenesis Concepts in Cardiomyopathies. J. Clin. Med. 2019, 8, 520.

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