TY - JOUR
T1 - Skeletal and respiratory muscle dysfunctions in pulmonary arterial hypertension
AU - Riou, Marianne
AU - Pizzimenti, Mégane
AU - Enache, Irina
AU - Charloux, Anne
AU - Canuet, Mathieu
AU - Andres, Emmanuel
AU - Talha, Samy
AU - Meyer, Alain
AU - Geny, Bernard
N1 - Publisher Copyright:
© 2020 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2020/2
Y1 - 2020/2
N2 - 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.
AB - 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.
KW - Catabolism
KW - Exercise
KW - Mitochondria
KW - Oxygen supply
KW - Pulmonary arterial hypertension
KW - Respiratory muscles
KW - Skeletal muscles
UR - http://www.scopus.com/inward/record.url?scp=85085481348&partnerID=8YFLogxK
U2 - 10.3390/jcm9020410
DO - 10.3390/jcm9020410
M3 - Review article
AN - SCOPUS:85085481348
SN - 2077-0383
VL - 9
JO - Journal of Clinical Medicine
JF - Journal of Clinical Medicine
IS - 2
M1 - 410
ER -