{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T07:40:49Z","timestamp":1790840449557,"version":"4.1.0"},"reference-count":52,"publisher":"European Respiratory Society (ERS)","issue":"4","content-domain":{"domain":["ersjournals.com"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2012,12,19]]},"published-print":{"date-parts":[[2013,4]]},"abstract":"<jats:p>Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease with poor prognosis if not treated. The treatment of choice is surgery with pulmonary endarterectomy. However, a significant percentage of patients are deemed non-operable due to distal distribution of the disease and arteriopathy in the non-occluded areas that is indistinguishable from pulmonary arterial hypertension (PAH). The overlap in clinical presentation, pathological features and pathogenesis between PAH and CTEPH provides a compelling rationale for exploring the efficacy of PAH-targeted therapies in CTEPH. These therapies are often considered for non-operable patients and are also used in operable patients as a bridge to surgery or as post-pulmonary endarterectomy therapy for persistent pulmonary hypertension, despite the fact they are not licensed for CTEPH.<\/jats:p><jats:p>Two randomised clinical trials have been performed in non-operable CTEPH patients. The BENEFiT study, with the endothelin receptor antagonist bosentan, did not show improvement in walking distance. Recently, the CHEST-1 trial, with the soluble guanylate cyclase stimulator riociguat, met study end-point and demonstrated significant improvement in walking distance in patients with non-operable CTEPH.<\/jats:p><jats:p>There is an urgent need for more randomised clinical trials designed to clarify whether administration of PAH-targeted therapies improves clinically meaningful end-points in various CTEPH populations.<\/jats:p>","DOI":"10.1183\/09031936.00201612","type":"journal-article","created":{"date-parts":[[2013,2,9]],"date-time":"2013-02-09T01:49:48Z","timestamp":1360374588000},"page":"985-990","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":96,"title":["Chronic thromboembolic pulmonary hypertension: role of medical therapy"],"prefix":"10.1183","volume":"41","author":[{"given":"Joanna","family":"Pepke-Zaba","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pavel","family":"Jansa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nick H.","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Naeije","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald","family":"Simonneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2013,2,8]]},"reference":[{"key":"2024102021342273000_41.4.985.1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.192230"},{"key":"2024102021342273000_41.4.985.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2010.11.024"},{"key":"2024102021342273000_41.4.985.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra010902"},{"key":"2024102021342273000_41.4.985.4","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.99.10.1325"},{"key":"2024102021342273000_41.4.985.5","doi-asserted-by":"publisher","DOI":"10.1378\/chest.130.1.172"},{"key":"2024102021342273000_41.4.985.6","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000236952.87590.c8"},{"key":"2024102021342273000_41.4.985.7","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa032274"},{"key":"2024102021342273000_41.4.985.8","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2009.018960"},{"key":"2024102021342273000_41.4.985.9","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1160\/TH04-10-0657","article-title":"Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension","volume":"93","author":"Bonderman","year":"2005","journal-title":"Thromb Haemost"},{"key":"2024102021342273000_41.4.985.10","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00087608"},{"key":"2024102021342273000_41.4.985.11","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00092008"},{"key":"2024102021342273000_41.4.985.12","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.015008"},{"key":"2024102021342273000_41.4.985.13","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMp048088"},{"key":"2024102021342273000_41.4.985.14","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200712-1841OC"},{"key":"2024102021342273000_41.4.985.15","unstructured":"The NHS Information Centre. 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