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Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension
Pulmonary arterial hypertension (PAH) is a syndrome diagnosed by increased mean pulmonary artery (PA) pressure and resistance and normal pulmonary capillary wedge pressure. PAH is characterized pathologically by distal pulmonary artery remodeling, increased pulmonary vascular resistance, and plexifo...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7422804/ https://www.ncbi.nlm.nih.gov/pubmed/32786064 http://dx.doi.org/10.14814/phy2.14532 |
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author | Siamwala, Jamila H. Zhao, Alexander Barthel, Haley Pagano, Francesco S. Gilbert, Richard J. Rounds, Sharon |
author_facet | Siamwala, Jamila H. Zhao, Alexander Barthel, Haley Pagano, Francesco S. Gilbert, Richard J. Rounds, Sharon |
author_sort | Siamwala, Jamila H. |
collection | PubMed |
description | Pulmonary arterial hypertension (PAH) is a syndrome diagnosed by increased mean pulmonary artery (PA) pressure and resistance and normal pulmonary capillary wedge pressure. PAH is characterized pathologically by distal pulmonary artery remodeling, increased pulmonary vascular resistance, and plexiform lesions (PLs). Right ventricular fibrosis and hypertrophy, leading to right ventricular failure, are the main determinants of mortality in PAH. Recent work suggests that right ventricular fibrosis results from resident cardiac fibroblast activation and conversion to myofibroblasts, leading to replacement of contractile cardiomyocytes with nondistensible tissue incapable of conductivity or contractility. However, the origins, triggers, and consequences of myofibroblast expansion and its pathophysiological relationship with PAH are unclear. Recent advances indicate that signals generated by adaptive and innate immune cells may play a role in right ventricular fibrosis and remodeling. This review summarizes recent insights into the mechanisms by which adaptive and innate immune signals participate in the transition of cardiac fibroblasts to activated myofibroblasts and highlights the existing gaps of knowledge as relates to the development of right ventricular fibrosis. |
format | Online Article Text |
id | pubmed-7422804 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-74228042020-08-13 Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension Siamwala, Jamila H. Zhao, Alexander Barthel, Haley Pagano, Francesco S. Gilbert, Richard J. Rounds, Sharon Physiol Rep Reviews Pulmonary arterial hypertension (PAH) is a syndrome diagnosed by increased mean pulmonary artery (PA) pressure and resistance and normal pulmonary capillary wedge pressure. PAH is characterized pathologically by distal pulmonary artery remodeling, increased pulmonary vascular resistance, and plexiform lesions (PLs). Right ventricular fibrosis and hypertrophy, leading to right ventricular failure, are the main determinants of mortality in PAH. Recent work suggests that right ventricular fibrosis results from resident cardiac fibroblast activation and conversion to myofibroblasts, leading to replacement of contractile cardiomyocytes with nondistensible tissue incapable of conductivity or contractility. However, the origins, triggers, and consequences of myofibroblast expansion and its pathophysiological relationship with PAH are unclear. Recent advances indicate that signals generated by adaptive and innate immune cells may play a role in right ventricular fibrosis and remodeling. This review summarizes recent insights into the mechanisms by which adaptive and innate immune signals participate in the transition of cardiac fibroblasts to activated myofibroblasts and highlights the existing gaps of knowledge as relates to the development of right ventricular fibrosis. John Wiley and Sons Inc. 2020-08-12 /pmc/articles/PMC7422804/ /pubmed/32786064 http://dx.doi.org/10.14814/phy2.14532 Text en © 2020 The Authors. Physiological Reports published by Wiley Periodicals LLC on behalf of The Physiological Society and the American Physiological Society This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Reviews Siamwala, Jamila H. Zhao, Alexander Barthel, Haley Pagano, Francesco S. Gilbert, Richard J. Rounds, Sharon Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
title | Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
title_full | Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
title_fullStr | Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
title_full_unstemmed | Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
title_short | Adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
title_sort | adaptive and innate immune mechanisms in cardiac fibrosis complicating pulmonary arterial hypertension |
topic | Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7422804/ https://www.ncbi.nlm.nih.gov/pubmed/32786064 http://dx.doi.org/10.14814/phy2.14532 |
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