Journal article

Mechanisms of disease: pulmonary arterial hypertension


Authors listSchermuly, Ralph T.; Ghofrani, Hossein A.; Wilkins, Martin R.; Grimminger, Friedrich

Publication year2011

Pages443-455

JournalNature Reviews Cardiology

Volume number8

Issue number8

ISSN1759-5002

eISSN1759-5010

Open access statusGreen

DOI Linkhttps://doi.org/10.1038/nrcardio.2011.87

PublisherNature Research


Abstract
Our understanding of, and approach to, pulmonary arterial hypertension has undergone a paradigm shift in the past decade. Once a condition thought to be dominated by increased vasoconstrictor tone and thrombosis, pulmonary arterial hypertension is now seen as a vasculopathy in which structural changes driven by excessive vascular cell growth and inflammation, with recruitment and infiltration of circulating cells, play a major role. Perturbations of a number of molecular mechanisms have been described, including pathways involving growth factors, cytokines, metabolic signaling, elastases, and proteases, that may underlie the pathogenesis of the disease. Elucidating their contribution to the pathophysiology of pulmonary arterial hypertension could offer new drug targets. The role of progenitor cells in vascular repair is also under active investigation. The right ventricular response to increased pressure load is recognized as critical to survival and the molecular mechanisms involved are attracting increasing interest. The challenge now is to integrate this new knowledge and explore how it can be used to categorize patients by molecular phenotype and tailor treatment more effectively.



Citation Styles

Harvard Citation styleSchermuly, R., Ghofrani, H., Wilkins, M. and Grimminger, F. (2011) Mechanisms of disease: pulmonary arterial hypertension, Nature Reviews Cardiology, 8(8), pp. 443-455. https://doi.org/10.1038/nrcardio.2011.87

APA Citation styleSchermuly, R., Ghofrani, H., Wilkins, M., & Grimminger, F. (2011). Mechanisms of disease: pulmonary arterial hypertension. Nature Reviews Cardiology. 8(8), 443-455. https://doi.org/10.1038/nrcardio.2011.87



Keywords


ACTIVATED-RECEPTOR-GAMMAENDOTHELIAL GROWTH-FACTORMARROW-DERIVED CELLSMATRIX METALLOPROTEINASESMUSCLE-CELL PROLIFERATIONPLEXIFORM LESIONSPROGENITOR CELLSRIGHT-VENTRICULAR DYSFUNCTION

Last updated on 2025-10-06 at 10:01