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Review
. 2020 Feb 3;9(2):410.
doi: 10.3390/jcm9020410.

Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension

Marianne Riou  1   2   3 , Mégane Pizzimenti  1   2 , Irina Enache  1   2 , Anne Charloux  1   2 , Mathieu Canuet  3 , Emmanuel Andres  4 , Samy Talha  1   2 , Alain Meyer  1   2 , Bernard Geny  1   2
Affiliations
Review

Skeletal and Respiratory Muscle Dysfunctions in Pulmonary Arterial Hypertension

Marianne Riou et al. J Clin Med. .

Abstract

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.

Keywords: Pulmonary arterial hypertension; catabolism; exercise; mitochondria; oxygen supply; respiratory muscles; skeletal muscles.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Mitochondrial respiratory chain complexes (Modified from [17]).
Figure 2
Figure 2
Mechanisms likely participating in pulmonary arterial hypertension-related skeletal muscle dysfunction.
Figure 3
Figure 3
Mechanisms likely participating in pulmonary arterial hypertension-related mitochondrial dysfunctions in muscles.
See this image and copyright information in PMC

References

    1. Humbert M., Guignabert C., Bonnet S., Dorfmuller P., Klinger J.R., Nicolls M.R., Olschewski A.J., Pullamsetti S.S., Schermuly R.T., Stenmark K.R., et al. Pathology and pathobiology of pulmonary hypertension: State of the art and research perspectives. Eur. Respir. J. 2019;53:1801887. doi: 10.1183/13993003.01887-2018. - DOI - PMC - PubMed
    1. Simonneau G., Montani D., Celermajer D.S., Denton C.P., Gatzoulis M.A., Krowka M., Williams P.G., Souza R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur. Respir. J. 2019;53:1801913. doi: 10.1183/13993003.01913-2018. - DOI - PMC - PubMed
    1. Galie N., Humbert M., Vachiery J.-L., Gibbs S., Lang I., Torbicki A., Simonneau G., Peacock A., Vonk Noordegraaf A., Beghetti M., et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) Eur. Respir. J. 2015;46:903–975. - PubMed
    1. Amsallem M., Sternbach J.M., Adigopula S., Kobayashi Y., Vu T.A., Zamanian R., Liang D., Dhillon G., Schnittger I., McConnell M.V., et al. Addressing the Controversy of Estimating Pulmonary Arterial Pressure by Echocardiography. J. Am. Soc. Echocardiogr. Off. Publ. Am. Soc. Echocardiogr. 2016;29:93–102. doi: 10.1016/j.echo.2015.11.001. - DOI - PubMed
    1. Kovacs G., Herve P., Barbera J.A., Chaouat A., Chemla D., Condliffe R., Garcia G., Grunig E., Howard L., Humbert M., et al. An official European Respiratory Society statement: Pulmonary haemodynamics during exercise. Eur. Respir. J. 2017;50:1700578. doi: 10.1183/13993003.00578-2017. - DOI - PubMed