The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction.
Original languageEnglish
Pages (from-to)832-842
JournalEuropean Respiratory Journal
Volume46
Issue number3
DOIs
Publication statusPublished - 2015

Cite this

@article{ab14438263f147398b5a48be14826958,
title = "The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle",
abstract = "Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction.",
author = "E. Manders and S. Rain and H.J. Bogaard and M.L. Handoko and G.J.M. Stienen and {Vonk Noordegraaf}, A. and C.A.C. Ottenheijm and {de Man}, F.S.",
year = "2015",
doi = "10.1183/13993003.02052-2014",
language = "English",
volume = "46",
pages = "832--842",
journal = "European Respiratory Journal",
issn = "0903-1936",
publisher = "European Respiratory Society",
number = "3",

}

The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle. / Manders, E.; Rain, S.; Bogaard, H.J.; Handoko, M.L.; Stienen, G.J.M.; Vonk Noordegraaf, A.; Ottenheijm, C.A.C.; de Man, F.S.

In: European Respiratory Journal, Vol. 46, No. 3, 2015, p. 832-842.

Research output: Contribution to journalArticleAcademicpeer-review

TY - JOUR

T1 - The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle

AU - Manders, E.

AU - Rain, S.

AU - Bogaard, H.J.

AU - Handoko, M.L.

AU - Stienen, G.J.M.

AU - Vonk Noordegraaf, A.

AU - Ottenheijm, C.A.C.

AU - de Man, F.S.

PY - 2015

Y1 - 2015

N2 - Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction.

AB - Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction.

U2 - 10.1183/13993003.02052-2014

DO - 10.1183/13993003.02052-2014

M3 - Article

VL - 46

SP - 832

EP - 842

JO - European Respiratory Journal

JF - European Respiratory Journal

SN - 0903-1936

IS - 3

ER -