{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:56:14Z","timestamp":1790834174071,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"s3","license":[{"start":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T00:00:00Z","timestamp":1154476800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Clin Investigation"],"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Background\u2002<\/jats:bold> Pulmonary arterial hypertension (PAH) is a devastating disease of the small pulmonary arteries and arterioles, characterized by intimal fibrosis, medial hypertrophy and plexiform lesions. When untreated both the idiopathic form (IPAH, formerly termed primary pulmonary hypertension, PPH) and PAH related to various other conditions such as scleroderma (SSc) often take a progressive course with high mortality. There is ongoing search for disease\u2010specific treatments that are able to improve survival in these patients. The oral dual endothelin (ET<jats:sub>A<\/jats:sub>\/ET<jats:sub>B<\/jats:sub>) antagonist bosentan has been shown to improve exercise capacity, time to clinical worsening, haemodynamics and quality of life in short\u2010term studies.<\/jats:p><jats:p><jats:bold>Materials and methods\u2002<\/jats:bold> To determine the long\u2010term effects of bosentan on survival, patients from the two double\u2010blind, randomized trials and their open\u2010label extensions, treated with first\u2010line bosentan, were followed for up to 3\u00a0years. Data on survival were collected between September 1999 (first patient included in the placebo\u2010controlled trials) and December 2002. Vital status was verified in each patient. The survival cohorts of these patients were compared with either the predicted survival for each patient based on an equation from the National Institutes of Health (NIH) PPH registry or with historical controls.<\/jats:p><jats:p><jats:bold>Results\u2002<\/jats:bold> Observed survival up to 36\u00a0months was reported as a Kaplan\u2010Meier estimate in three cohorts: (1) In 169 PPH patients treated with first\u2010line bosentan, 1\u2010 and 2\u2010year survival was 96% and 89%, respectively, vs. predicted untreated survival at 1 and 2\u00a0years of 69% and 57%, respectively; (2) in 50 patients with PAH associated with SSc (PAH\u2010SSc), 1\u2010, 2\u2010 and 3\u2010year survival was 82%, 67% and 64%, respectively, vs. \u223c45%, \u223c35% and \u223c28%, respectively, from registry data of untreated PAH\u2010SSc patients; and (3) in 139 PPH patients in WHO functional class III, 1\u2010 and 2\u2010year survival was 97% and 91%, respectively, vs. 91% and 84% in a historical cohort of 346 patients treated with epoprostenol in five major referral centres.<\/jats:p><jats:p><jats:bold>Conclusions\u2002<\/jats:bold> The present analyses suggest that first\u2010line bosentan therapy, followed by the addition of other disease\u2010specific therapies as required, improves survival in patients with advanced PAH.<\/jats:p>","DOI":"10.1111\/j.1365-2362.2006.01688.x","type":"journal-article","created":{"date-parts":[[2006,8,2]],"date-time":"2006-08-02T23:14:53Z","timestamp":1154560493000},"page":"10-15","source":"Crossref","is-referenced-by-count":82,"title":["Survival in patients with pulmonary arterial hypertension treated with first\u2010line bosentan"],"prefix":"10.1111","volume":"36","author":[{"given":"V. V.","family":"McLaughlin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,8,2]]},"reference":[{"key":"e_1_2_4_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ehj.2004.09.014"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.126.1_suppl.78S"},{"key":"e_1_2_4_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(03)00463-7"},{"key":"e_1_2_4_5_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehi283"},{"key":"e_1_2_4_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.010"},{"key":"e_1_2_4_7_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.04.00072104"},{"key":"e_1_2_4_8_2","first-page":"A48","article-title":"Relation of endothelin\u20101 to survival in patients with primary pulmonary hypertension [abstract]","volume":"26","author":"Galie N","year":"1996","journal-title":"Eur J Clin Invest"},{"key":"e_1_2_4_9_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.120.5.1562"},{"key":"e_1_2_4_10_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.103.2.314"},{"key":"e_1_2_4_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)06250-X"},{"key":"e_1_2_4_12_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa012212"},{"key":"e_1_2_4_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(03)00121-9"},{"key":"e_1_2_4_14_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.124.1.247"},{"key":"e_1_2_4_15_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00054804"},{"key":"e_1_2_4_16_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_2_4_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.89.4.1733"},{"issue":"2","key":"e_1_2_4_18_2","article-title":"Dual endothelin receptor antagonism in pulmonary arterial hypertension related to systemic sclerosis","volume":"35","author":"Denton C","year":"2005","journal-title":"Eur J Clin Invest"},{"key":"e_1_2_4_19_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/35.10.989"},{"key":"e_1_2_4_20_2","doi-asserted-by":"publisher","DOI":"10.1136\/thx.2005.040618"},{"key":"e_1_2_4_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2003.11.037"},{"key":"e_1_2_4_22_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.2503002"},{"key":"e_1_2_4_23_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199207093270203"},{"key":"e_1_2_4_24_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.99.9.1197"},{"key":"e_1_2_4_25_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000138104.83366.E9"},{"key":"e_1_2_4_26_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486"},{"key":"e_1_2_4_27_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000029100.82385.58"},{"key":"e_1_2_4_28_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)02012-0"},{"key":"e_1_2_4_29_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(97)00187-3"},{"key":"e_1_2_4_30_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"e_1_2_4_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.02.036"},{"key":"e_1_2_4_32_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200006223422503"},{"key":"e_1_2_4_33_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00129604"},{"key":"e_1_2_4_34_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.123.2.344"}],"container-title":["European Journal of Clinical Investigation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1365-2362.2006.01688.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1365-2362.2006.01688.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,27]],"date-time":"2023-09-27T08:57:35Z","timestamp":1695805055000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1365-2362.2006.01688.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,8,2]]},"references-count":33,"journal-issue":{"issue":"s3","published-print":{"date-parts":[[2006,9]]}},"alternative-id":["10.1111\/j.1365-2362.2006.01688.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1365-2362.2006.01688.x","archive":["Portico"],"relation":{},"ISSN":["0014-2972","1365-2362"],"issn-type":[{"value":"0014-2972","type":"print"},{"value":"1365-2362","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,8,2]]}}}