Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology
Open Access
- 28 February 2010
- journal article
- review article
- Published by European Respiratory Society (ERS) in European Respiratory Review
- Vol. 19 (115) , 59-63
- https://doi.org/10.1183/09059180.00007309
Abstract
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.Keywords
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