Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is a rare disease, which leads to the progressive loss and remodeling of the pulmonary vessels, right heart failure, and death. Different clinical presentations can be responsible for such a bad prognosis disease and the underlying mechanisms still need to be fu...
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doaj-a2d80c9b95cb46a3ae8a0a0c31f49d512020-11-25T03:32:30ZengMDPI AGJournal of Clinical Medicine2077-03832020-02-019241010.3390/jcm9020410jcm9020410Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial HypertensionMarianne Riou0Mégane Pizzimenti1Irina Enache2Anne Charloux3Mathieu Canuet4Emmanuel Andres5Samy Talha6Alain Meyer7Bernard Geny8Unistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FranceUnistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FranceUnistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FranceUnistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FrancePulmonology Service, University Hospital of Strasbourg, 1 place de l’Hôpital, 67091 Strasbourg CEDEX, FranceInternal Medicine, Diabete and Metabolic Diseases Service, University Hospital of Strasbourg, 1 place de l’Hôpital, 67091 Strasbourg CEDEX, FranceUnistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FranceUnistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FranceUnistra, Translational Medicine Federation of Strasbourg (FMTS), Faculty of Medicine, Team 3072 “Mitochondria, Oxidative Stress and Muscle Protection”, 11 rue Humann, 67000 Strasbourg, FrancePulmonary arterial hypertension (PAH) is a rare disease, which leads to the progressive loss and remodeling of the pulmonary vessels, right heart failure, and death. Different clinical presentations can be responsible for such a bad prognosis disease and the underlying mechanisms still need to be further examined. Importantly, skeletal and respiratory muscle abnormalities largely contribute to the decreased quality of life and exercise intolerance observed in patients with PAH. At the systemic level, impaired oxygen supply through reduced cardiac output and respiratory muscle dysfunctions, which potentially result in hypoxemia, is associated with altered muscles vascularization, inflammation, enhanced catabolic pathways, and impaired oxygen use through mitochondrial dysfunctions that are likely participate in PAH-related myopathy. Sharing new insights into the pathological mechanisms of PAH might help stimulate specific research areas, improving the treatment and quality of life of PAH patients. Indeed, many of these muscular impairments are reversible, strongly supporting the development of effective preventive and/or therapeutic approaches, including mitochondrial protection and exercise training.https://www.mdpi.com/2077-0383/9/2/410pulmonary arterial hypertensionskeletal musclesrespiratory musclesoxygen supplycatabolismmitochondriaexercise |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Marianne Riou Mégane Pizzimenti Irina Enache Anne Charloux Mathieu Canuet Emmanuel Andres Samy Talha Alain Meyer Bernard Geny |
spellingShingle |
Marianne Riou Mégane Pizzimenti Irina Enache Anne Charloux Mathieu Canuet Emmanuel Andres Samy Talha Alain Meyer Bernard Geny Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension Journal of Clinical Medicine pulmonary arterial hypertension skeletal muscles respiratory muscles oxygen supply catabolism mitochondria exercise |
author_facet |
Marianne Riou Mégane Pizzimenti Irina Enache Anne Charloux Mathieu Canuet Emmanuel Andres Samy Talha Alain Meyer Bernard Geny |
author_sort |
Marianne Riou |
title |
Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension |
title_short |
Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension |
title_full |
Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension |
title_fullStr |
Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension |
title_full_unstemmed |
Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension |
title_sort |
skeletal and respiratory muscle dysfunctions in pulmonary arterial hypertension |
publisher |
MDPI AG |
series |
Journal of Clinical Medicine |
issn |
2077-0383 |
publishDate |
2020-02-01 |
description |
Pulmonary arterial hypertension (PAH) is a rare disease, which leads to the progressive loss and remodeling of the pulmonary vessels, right heart failure, and death. Different clinical presentations can be responsible for such a bad prognosis disease and the underlying mechanisms still need to be further examined. Importantly, skeletal and respiratory muscle abnormalities largely contribute to the decreased quality of life and exercise intolerance observed in patients with PAH. At the systemic level, impaired oxygen supply through reduced cardiac output and respiratory muscle dysfunctions, which potentially result in hypoxemia, is associated with altered muscles vascularization, inflammation, enhanced catabolic pathways, and impaired oxygen use through mitochondrial dysfunctions that are likely participate in PAH-related myopathy. Sharing new insights into the pathological mechanisms of PAH might help stimulate specific research areas, improving the treatment and quality of life of PAH patients. Indeed, many of these muscular impairments are reversible, strongly supporting the development of effective preventive and/or therapeutic approaches, including mitochondrial protection and exercise training. |
topic |
pulmonary arterial hypertension skeletal muscles respiratory muscles oxygen supply catabolism mitochondria exercise |
url |
https://www.mdpi.com/2077-0383/9/2/410 |
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