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.
Manders, E., Rain, S., Bogaard, H. J., Handoko, M. L., Stienen, G. J. M., Vonk Noordegraaf, A., ... de Man, F. S. (2015). The striated muscles in pulmonary arterial hypertension: adaptations beyond the right ventricle. European Respiratory Journal, 46(3), 832-842. https://doi.org/10.1183/13993003.02052-2014