Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type...

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Main Author: M. Humbert
Format: Article
Language:English
Published: European Respiratory Society 2010-03-01
Series:European Respiratory Review
Subjects:
Online Access:http://err.ersjournals.com/cgi/content/full/19/115/59
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spelling doaj-8ca4011fcc814d21a87d02c8192635402020-11-25T02:43:20ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172010-03-01191155963Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiologyM. HumbertPulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis), and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy. http://err.ersjournals.com/cgi/content/full/19/115/59Chronic thromboembolic pulmonary hypertensionepidemiologypathophysiologypulmonary arterial hypertension
collection DOAJ
language English
format Article
sources DOAJ
author M. Humbert
spellingShingle M. Humbert
Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
European Respiratory Review
Chronic thromboembolic pulmonary hypertension
epidemiology
pathophysiology
pulmonary arterial hypertension
author_facet M. Humbert
author_sort M. Humbert
title Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
title_short Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
title_full Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
title_fullStr Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
title_full_unstemmed Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
title_sort pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
publisher European Respiratory Society
series European Respiratory Review
issn 0905-9180
1600-0617
publishDate 2010-03-01
description Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis), and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy.
topic Chronic thromboembolic pulmonary hypertension
epidemiology
pathophysiology
pulmonary arterial hypertension
url http://err.ersjournals.com/cgi/content/full/19/115/59
work_keys_str_mv AT mhumbert pulmonaryarterialhypertensionandchronicthromboembolicpulmonaryhypertensionpathophysiology
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