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The pathophysiology of chronic thromboembolic pulmonary hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that is usually a consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organised thrombi. Failure of t...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
European Respiratory Society
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488693/ https://www.ncbi.nlm.nih.gov/pubmed/28356405 http://dx.doi.org/10.1183/16000617.0112-2016 |
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author | Simonneau, Gérald Torbicki, Adam Dorfmüller, Peter Kim, Nick |
author_facet | Simonneau, Gérald Torbicki, Adam Dorfmüller, Peter Kim, Nick |
author_sort | Simonneau, Gérald |
collection | PubMed |
description | Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that is usually a consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organised thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis or underlying haematological or autoimmune disorders. It is now known that small-vessel abnormalities also contribute to haemodynamic compromise, functional impairment and disease progression in CTEPH. Small-vessel disease can occur in obstructed areas, possibly triggered by unresolved thrombotic material, and downstream from occlusions, possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries. The molecular processes underlying small-vessel disease are not completely understood and further research is needed in this area. The degree of small-vessel disease has a substantial impact on the severity of CTEPH and postsurgical outcomes. Interventional and medical treatment of CTEPH should aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle and prevent or treat small-vessel disease. It requires early, reliable identification of patients with CTEPH and use of optimal treatment modalities in expert centres. |
format | Online Article Text |
id | pubmed-9488693 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | European Respiratory Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-94886932022-11-14 The pathophysiology of chronic thromboembolic pulmonary hypertension Simonneau, Gérald Torbicki, Adam Dorfmüller, Peter Kim, Nick Eur Respir Rev Reviews Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that is usually a consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organised thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis or underlying haematological or autoimmune disorders. It is now known that small-vessel abnormalities also contribute to haemodynamic compromise, functional impairment and disease progression in CTEPH. Small-vessel disease can occur in obstructed areas, possibly triggered by unresolved thrombotic material, and downstream from occlusions, possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries. The molecular processes underlying small-vessel disease are not completely understood and further research is needed in this area. The degree of small-vessel disease has a substantial impact on the severity of CTEPH and postsurgical outcomes. Interventional and medical treatment of CTEPH should aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle and prevent or treat small-vessel disease. It requires early, reliable identification of patients with CTEPH and use of optimal treatment modalities in expert centres. European Respiratory Society 2017-03-29 /pmc/articles/PMC9488693/ /pubmed/28356405 http://dx.doi.org/10.1183/16000617.0112-2016 Text en Copyright ©ERS 2017. https://creativecommons.org/licenses/by-nc/4.0/ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. |
spellingShingle | Reviews Simonneau, Gérald Torbicki, Adam Dorfmüller, Peter Kim, Nick The pathophysiology of chronic thromboembolic pulmonary hypertension |
title | The pathophysiology of chronic thromboembolic pulmonary hypertension |
title_full | The pathophysiology of chronic thromboembolic pulmonary hypertension |
title_fullStr | The pathophysiology of chronic thromboembolic pulmonary hypertension |
title_full_unstemmed | The pathophysiology of chronic thromboembolic pulmonary hypertension |
title_short | The pathophysiology of chronic thromboembolic pulmonary hypertension |
title_sort | pathophysiology of chronic thromboembolic pulmonary hypertension |
topic | Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488693/ https://www.ncbi.nlm.nih.gov/pubmed/28356405 http://dx.doi.org/10.1183/16000617.0112-2016 |
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