{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,10]],"date-time":"2026-08-10T12:36:48Z","timestamp":1786365408614,"version":"build-2736575974"},"reference-count":93,"publisher":"European Respiratory Society (ERS)","issue":"130","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir Rev"],"accepted":{"date-parts":[[2013,10,12]]},"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:p>Pulmonary arterial hypertension (PAH) is a fatal disease associated with vasoconstriction and vascular remodelling. There are three well-known pathways that contribute to the pathogenesis of PAH: endothelin, nitric oxide and prostacyclin. Treatments targeting these pathways are well established in clinical practice, such as endothelin receptor antagonists, phosphodiesterase type-5 inhibitors and epoprostenol. New treatments have been developed with the aim of improving efficacy and ease of administration of therapies targeting these three established pathways, and several of these new treatments have recently undergone phase III investigation. Ongoing pre-clinical studies are also beginning to uncover other mechanisms that play a role in the complex pathobiology of PAH. These include genetic targets, transcription factors and signalling pathways. The discovery of new treatment targets may change the landscape of PAH therapy in the future. Herein, we present some of the promising future treatments and interesting new therapeutic targets.<\/jats:p>","DOI":"10.1183\/09059180.00006613","type":"journal-article","created":{"date-parts":[[2013,11,30]],"date-time":"2013-11-30T01:42:15Z","timestamp":1385775735000},"page":"503-514","source":"Crossref","is-referenced-by-count":34,"title":["New horizons in pulmonary arterial hypertension therapies"],"prefix":"10.1183","volume":"22","author":[{"given":"Nazzareno","family":"Gali\u00e8","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ardeschir-Hossein","family":"Ghofrani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2013,11,29]]},"reference":[{"key":"2025042406003429000_22.130.503.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra040291"},{"key":"2025042406003429000_22.130.503.2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00046612"},{"key":"2025042406003429000_22.130.503.3","doi-asserted-by":"publisher","DOI":"10.1183\/09059180.00004812"},{"key":"2025042406003429000_22.130.503.4","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)06250-X"},{"key":"2025042406003429000_22.130.503.5","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa012212"},{"key":"2025042406003429000_22.130.503.6","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200307-957OC"},{"key":"2025042406003429000_22.130.503.7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2006.01.057"},{"key":"2025042406003429000_22.130.503.8","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.742510"},{"key":"2025042406003429000_22.130.503.9","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa050010"},{"key":"2025042406003429000_22.130.503.10","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.839274"},{"key":"2025042406003429000_22.130.503.11","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-112-7-485"},{"key":"2025042406003429000_22.130.503.12","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"2025042406003429000_22.130.503.13","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa020204"},{"key":"2025042406003429000_22.130.503.14","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.165.6.2106079"},{"key":"2025042406003429000_22.130.503.15","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2010.01.027"},{"key":"2025042406003429000_22.130.503.16","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(03)00463-7"},{"key":"2025042406003429000_22.130.503.17","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)01786-2"},{"key":"2025042406003429000_22.130.503.18","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.911818"},{"key":"2025042406003429000_22.130.503.19","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201203-0383OC"},{"key":"2025042406003429000_22.130.503.20","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-1460"},{"key":"2025042406003429000_22.130.503.21","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0047662"},{"key":"2025042406003429000_22.130.503.22","doi-asserted-by":"publisher","DOI":"10.1124\/jpet.108.142976"},{"key":"2025042406003429000_22.130.503.23","doi-asserted-by":"publisher","DOI":"10.1007\/s40262-013-0063-8"},{"key":"2025042406003429000_22.130.503.24","doi-asserted-by":"publisher","DOI":"10.1208\/s12248-011-9316-3"},{"key":"2025042406003429000_22.130.503.25","doi-asserted-by":"publisher","DOI":"10.1021\/jm3009103"},{"key":"2025042406003429000_22.130.503.26","first-page":"1066","article-title":"Macitentan, a tissue-targeting endothelin receptor antagonist for the potential oral treatment of pulmonary arterial hypertension and idiopathic pulmonary fibrosis","volume":"11","author":"Raja","year":"2010","journal-title":"Curr Opin Investig Drugs"},{"key":"2025042406003429000_22.130.503.27","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213917"},{"key":"2025042406003429000_22.130.503.28","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2009.04.007"},{"key":"2025042406003429000_22.130.503.29","doi-asserted-by":"publisher","DOI":"10.1124\/jpet.107.124248"},{"key":"2025042406003429000_22.130.503.30","doi-asserted-by":"publisher","DOI":"10.1124\/jpet.112.197152"},{"key":"2025042406003429000_22.130.503.31","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00137511"},{"key":"2025042406003429000_22.130.503.32","unstructured":"Clinicaltrials.gov. Selexipag (ACT-293987) in pulmonary arterial hypertension, GRIPHON Trial. NCT01106014. http:\/\/clinicaltrials.gov\/ct2\/show\/NCT01106014?term=GRIPHON&rank=1 Date last accessed: August 28, 2013. Date last updated: September 3, 2013."},{"key":"2025042406003429000_22.130.503.33","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(03)00463-7"},{"key":"2025042406003429000_22.130.503.34","doi-asserted-by":"publisher","DOI":"10.1378\/chest.11-2212"},{"key":"2025042406003429000_22.130.503.35","doi-asserted-by":"publisher","DOI":"10.1517\/13543784.2011.565048"},{"key":"2025042406003429000_22.130.503.36","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00182909"},{"key":"2025042406003429000_22.130.503.37","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1209655"},{"key":"2025042406003429000_22.130.503.38","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1209657"},{"key":"2025042406003429000_22.130.503.39","first-page":"A3531","article-title":"Riociguat for the treatment of pulmonary arterial hypertension (PAH): a phase III long-term extension study (PATENT-2)","volume":"187","author":"Lewis","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"key":"2025042406003429000_22.130.503.40","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2008.08.059"},{"key":"2025042406003429000_22.130.503.41","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.112.000765"},{"key":"2025042406003429000_22.130.503.42","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.98.11030554"},{"key":"2025042406003429000_22.130.503.43","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201006-0905OC"},{"key":"2025042406003429000_22.130.503.44","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200707-1037OC"},{"key":"2025042406003429000_22.130.503.45","doi-asserted-by":"publisher","DOI":"10.1172\/JCI24838"},{"key":"2025042406003429000_22.130.503.46","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2011.02.024"},{"key":"2025042406003429000_22.130.503.47","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc051946"},{"key":"2025042406003429000_22.130.503.48","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201001-0123OC"},{"key":"2025042406003429000_22.130.503.49","first-page":"A6304","article-title":"Comparison of effects of imatinib and nilotinib in a rodent model of pulmonary arterial hypertension","volume":"181","author":"Nicholas","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"2025042406003429000_22.130.503.50","unstructured":"Clinicaltrials.gov. Efficacy, safety, tolerability and pharmacokinetics (PK) of nilotinib (AMN107) in pulmonary arterial hypertension (PAH). NCT01179737 http:\/\/clinicaltrials.gov\/ct2\/show\/NCT01179737?term=NCT01179737&rank=1 Date last accessed: August 30, 2013. Date last updated: March 25, 2013."},{"key":"2025042406003429000_22.130.503.51","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.776831"},{"key":"2025042406003429000_22.130.503.52","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.109940"},{"key":"2025042406003429000_22.130.503.53","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-12-141"},{"key":"2025042406003429000_22.130.503.54","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00217.2009"},{"key":"2025042406003429000_22.130.503.55","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00050008"},{"key":"2025042406003429000_22.130.503.56","doi-asserted-by":"publisher","DOI":"10.1172\/JCI200317500"},{"key":"2025042406003429000_22.130.503.57","first-page":"A2516","article-title":"Effects of inhaled aviptadil (vasoactive intestinal peptide) in patients with pulmonary arterial hypertension (PAH)","volume":"181","author":"Gali\u00e8","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"2025042406003429000_22.130.503.58","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.185.7.786"},{"key":"2025042406003429000_22.130.503.59","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2011.06.009"},{"key":"2025042406003429000_22.130.503.60","doi-asserted-by":"publisher","DOI":"10.3109\/01902148.2010.512972"},{"key":"2025042406003429000_22.130.503.61","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.01318.2010"},{"key":"2025042406003429000_22.130.503.62","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00944.2011"},{"key":"2025042406003429000_22.130.503.63","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00130209"},{"key":"2025042406003429000_22.130.503.64","doi-asserted-by":"publisher","DOI":"10.1007\/s00380-009-1176-8"},{"key":"2025042406003429000_22.130.503.65","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcad.2012.07.006"},{"key":"2025042406003429000_22.130.503.66","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000127375.56172.92"},{"key":"2025042406003429000_22.130.503.67","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00126010"},{"key":"2025042406003429000_22.130.503.68","first-page":"A2496","article-title":"Proof-of-concept study to investigate the efficacy, hemodynamics and tolerability of terguride vs. placebo in subjects with pulmonary arterial hypertension: results of a double blind, randomised, prospective phase IIa study","volume":"185","author":"Ghofrani","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"2025042406003429000_22.130.503.69","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.87299"},{"key":"2025042406003429000_22.130.503.70","doi-asserted-by":"publisher","DOI":"10.1172\/JCI60658"},{"key":"2025042406003429000_22.130.503.71","doi-asserted-by":"publisher","DOI":"10.1172\/JCI43382"},{"key":"2025042406003429000_22.130.503.72","first-page":"1","article-title":"[Effect of apelin on vasodilatation of isolated pulmonary arteries in rats is concerned with the nitric oxide pathway]","volume":"27","author":"Huang","year":"2011","journal-title":"Zhongguo Ying Yong Sheng Li Xue Za Zhi"},{"key":"2025042406003429000_22.130.503.73","doi-asserted-by":"publisher","DOI":"10.1016\/j.peptides.2010.06.004"},{"key":"2025042406003429000_22.130.503.74","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.00089.2009"},{"key":"2025042406003429000_22.130.503.75","doi-asserted-by":"crossref","first-page":"720305","DOI":"10.1155\/2010\/720305","article-title":"Interleukin-6 as a potential therapeutic target for pulmonary arterial hypertension","volume":"2010","author":"Furuya","year":"2010","journal-title":"Int J Rheumatol"},{"key":"2025042406003429000_22.130.503.76","doi-asserted-by":"publisher","DOI":"10.1186\/1465-9921-8-15"},{"key":"2025042406003429000_22.130.503.77","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200811-1682OC"},{"key":"2025042406003429000_22.130.503.78","doi-asserted-by":"publisher","DOI":"10.1172\/JCI6539"},{"key":"2025042406003429000_22.130.503.79","doi-asserted-by":"publisher","DOI":"10.1172\/JCI32503"},{"key":"2025042406003429000_22.130.503.80","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00199.2009"},{"key":"2025042406003429000_22.130.503.81","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1843.2010.01756.x"},{"key":"2025042406003429000_22.130.503.82","doi-asserted-by":"publisher","DOI":"10.1007\/s00210-013-0846-3"},{"key":"2025042406003429000_22.130.503.83","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-010-0679-1"},{"key":"2025042406003429000_22.130.503.84","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201108-1562OC"},{"key":"2025042406003429000_22.130.503.85","doi-asserted-by":"publisher","DOI":"10.1096\/fj.09-131664"},{"key":"2025042406003429000_22.130.503.86","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-009-0524-6"},{"key":"2025042406003429000_22.130.503.87","doi-asserted-by":"publisher","DOI":"10.3109\/01902148.2012.758191"},{"key":"2025042406003429000_22.130.503.88","doi-asserted-by":"publisher","DOI":"10.1126\/science.275.5302.964"},{"key":"2025042406003429000_22.130.503.89","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.769646"},{"key":"2025042406003429000_22.130.503.90","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2007.12.030"},{"key":"2025042406003429000_22.130.503.91","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2008.070705"},{"key":"2025042406003429000_22.130.503.92","doi-asserted-by":"publisher","DOI":"10.21693\/1933-088X-11.1.33"},{"key":"2025042406003429000_22.130.503.93","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2006.12.037"}],"container-title":["European Respiratory Review"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/09059180.00006613","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T13:01:08Z","timestamp":1745499668000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/09059180.00006613"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,11,29]]},"references-count":93,"journal-issue":{"issue":"130","published-online":{"date-parts":[[2013,11,29]]},"published-print":{"date-parts":[[2013,12]]}},"alternative-id":["10.1183\/09059180.00006613"],"URL":"https:\/\/doi.org\/10.1183\/09059180.00006613","relation":{},"ISSN":["0905-9180","1600-0617"],"issn-type":[{"value":"0905-9180","type":"print"},{"value":"1600-0617","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,11,29]]}}}