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Zelluläre Pathophysiologie der pulmonalen Hypertonie
Pulmonary hypertension comprises a group of diseases with heterogeneous etiology characterized by an increase of hydrostatic pressure in the pulmonary vascular bed. While secondary pulmonary hypertension predominantly results from acute or chronic left ventricular failure, characteristic gene defect...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Steinkopff-Verlag
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101801/ https://www.ncbi.nlm.nih.gov/pubmed/32288285 http://dx.doi.org/10.1007/s00398-002-0347-z |
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author | Kuebler, W. M. Kuppe, H. |
author_facet | Kuebler, W. M. Kuppe, H. |
author_sort | Kuebler, W. M. |
collection | PubMed |
description | Pulmonary hypertension comprises a group of diseases with heterogeneous etiology characterized by an increase of hydrostatic pressure in the pulmonary vascular bed. While secondary pulmonary hypertension predominantly results from acute or chronic left ventricular failure, characteristic gene defects or predisposing risk factors lead to various forms of primary pulmonary hypertension. Despite its diverse pathogenesis, pulmonary hypertension exhibits a uniform cellular pathophysiology in the pulmonary microcirculation. The dysfunction of lung vascular endothelial cells, which are the front line in response to hemodynamic changes in the pulmonary circulation, is the pathophysiological driving force of pulmonary hypertension. Endothelial dysfunction is characterized by a reduced production of vasodilative, anti-proliferative mediators and an increased release of vasoconstrictive, proliferative factors. This apparent imbalance not only enhances pulmonary vasoconstriction, but supports pathologic remodeling processes in the vascular intima and media. In addition, the pulmonary endothelium recruits platelets and leukocytes, thus, contributing to further release of vasoconstrictive and proliferative mediators and characteristic thrombus formation. These endothelium-derived pathomechanisms amplify each other, further enhance pulmonary vascular resistance, and finally result in fixation of the hypertensive state. Hence, pulmonary hypertension not only describes an alteration of lung hemodynamics, but comprises a complex set of pathophysiological events in both lung parenchymal cells and circulating blood cells. For development of new therapeutical strategies, the multifactorial character of the disease should be considered. |
format | Online Article Text |
id | pubmed-7101801 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Steinkopff-Verlag |
record_format | MEDLINE/PubMed |
spelling | pubmed-71018012020-03-31 Zelluläre Pathophysiologie der pulmonalen Hypertonie Kuebler, W. M. Kuppe, H. Z Herz Thorax Gefasschir Originalarbeit Pulmonary hypertension comprises a group of diseases with heterogeneous etiology characterized by an increase of hydrostatic pressure in the pulmonary vascular bed. While secondary pulmonary hypertension predominantly results from acute or chronic left ventricular failure, characteristic gene defects or predisposing risk factors lead to various forms of primary pulmonary hypertension. Despite its diverse pathogenesis, pulmonary hypertension exhibits a uniform cellular pathophysiology in the pulmonary microcirculation. The dysfunction of lung vascular endothelial cells, which are the front line in response to hemodynamic changes in the pulmonary circulation, is the pathophysiological driving force of pulmonary hypertension. Endothelial dysfunction is characterized by a reduced production of vasodilative, anti-proliferative mediators and an increased release of vasoconstrictive, proliferative factors. This apparent imbalance not only enhances pulmonary vasoconstriction, but supports pathologic remodeling processes in the vascular intima and media. In addition, the pulmonary endothelium recruits platelets and leukocytes, thus, contributing to further release of vasoconstrictive and proliferative mediators and characteristic thrombus formation. These endothelium-derived pathomechanisms amplify each other, further enhance pulmonary vascular resistance, and finally result in fixation of the hypertensive state. Hence, pulmonary hypertension not only describes an alteration of lung hemodynamics, but comprises a complex set of pathophysiological events in both lung parenchymal cells and circulating blood cells. For development of new therapeutical strategies, the multifactorial character of the disease should be considered. Steinkopff-Verlag 2014-02-18 2002 /pmc/articles/PMC7101801/ /pubmed/32288285 http://dx.doi.org/10.1007/s00398-002-0347-z Text en © Steinkopff Verlag 2002 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic. |
spellingShingle | Originalarbeit Kuebler, W. M. Kuppe, H. Zelluläre Pathophysiologie der pulmonalen Hypertonie |
title | Zelluläre Pathophysiologie der pulmonalen Hypertonie |
title_full | Zelluläre Pathophysiologie der pulmonalen Hypertonie |
title_fullStr | Zelluläre Pathophysiologie der pulmonalen Hypertonie |
title_full_unstemmed | Zelluläre Pathophysiologie der pulmonalen Hypertonie |
title_short | Zelluläre Pathophysiologie der pulmonalen Hypertonie |
title_sort | zelluläre pathophysiologie der pulmonalen hypertonie |
topic | Originalarbeit |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7101801/ https://www.ncbi.nlm.nih.gov/pubmed/32288285 http://dx.doi.org/10.1007/s00398-002-0347-z |
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