{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T14:19:16Z","timestamp":1772288356226,"version":"3.50.1"},"reference-count":36,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2024,3,17]],"date-time":"2024-03-17T00:00:00Z","timestamp":1710633600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100012166","name":"National Key Research and Development Program of China","doi-asserted-by":"publisher","award":["2016YFC1304400"],"award-info":[{"award-number":["2016YFC1304400"]}],"id":[{"id":"10.13039\/501100012166","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Objective<\/jats:title><jats:p>Off\u2010label pulmonary arterial hypertension (PAH)\u2010targeted drugs are commonly prescribed for non\u2010operated chronic thromboembolic pulmonary hypertension (CTEPH), but their effect on the long\u2010term prognosis of CTEPH remains unknown. This study investigated the effect of off\u2010label PAH\u2010targeted drugs on the long\u2010term survival of CTEPH patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>CTEPH patients were enrolled from a prospective multicentre national registry. Except for licensed riociguat and treprostinil, other PAH\u2010targeted drugs were off\u2010label. In the original and propensity score\u2013matched (PSM) samples, five\u2010year survival was compared in two groups: (a) patients not receiving off\u2010label PAH\u2010targeted drugs (control) versus (b) patients receiving off\u2010label PAH\u2010targeted drugs (treatment). The latter group was investigated for the effect of started off\u2010label PAH\u2010targeted drugs at baselines (initial) or during follow\u2010up (subsequent).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 347 enrolled patients, 212 were treated with off\u2010label PAH\u2010targeted drugs initially (<jats:italic>n<\/jats:italic>\u2009=\u2009173) or subsequently (<jats:italic>n<\/jats:italic>\u2009=\u200939), and 135 were untreated. The 1\u2010, 2\u2010, 3\u2010 and 5\u2010year survival of the treatment group was significantly higher than that of the control group (97.1% vs. 89.4%, 92.3% vs. 82.1%, 83.2% vs. 75.1% and 71.1% vs. 55.3%, respectively, log\u2010rank test, <jats:italic>p<\/jats:italic>\u2009=\u20090.005). Initial treatment was correlated with better 5\u2010year survival after excluding patients with subsequent treatment to reduce the immortal\u2010time bias (hazard ratio: 0.611; 95% CI: 0.397\u20130.940; <jats:italic>p<\/jats:italic>\u2009=\u20090.025). In PSM samples, patients given initial treatment showed significantly better 5\u2010year survival than untreated patients (68.9% vs. 49.3%, log\u2010rank test, <jats:italic>p<\/jats:italic>\u2009=\u20090.008).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Off\u2010label targeted drugs contributed to improved long\u2010term survival in CTEPH patients receiving pharmacotherapies.<\/jats:p><jats:p><jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/resp14700-gra-0001-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text><\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.14700","type":"journal-article","created":{"date-parts":[[2024,3,18]],"date-time":"2024-03-18T06:39:21Z","timestamp":1710743961000},"page":"614-623","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Effect of off\u2010label targeted drugs on long\u2010term survival in chronic thromboembolic pulmonary hypertension: Insights from a national multicentre prospective registry"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-3626-0611","authenticated-orcid":false,"given":"Wanying","family":"Xia","sequence":"first","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Diseases Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuling","family":"Qian","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Diseases Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yangyi","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Diseases Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5194-0420","authenticated-orcid":false,"given":"Ruilin","family":"Quan","sequence":"additional","affiliation":[{"name":"Department of Pulmonary Vascular Disease, National Center for Cardiovascular Diseases Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, State Key Laboratory of Cardiovascular Disease  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuanhua","family":"Yang","sequence":"additional","affiliation":[{"name":"Respiratory and Critical Care Medicine Beijing Institute of Respiratory Medicine, Beijing Chao\u2010Yang Hospital, Capital Medical University  Beijing China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhenwen","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Cardiology Tianjin Medical University General Hospital  Tianjin China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hongyan","family":"Tian","sequence":"additional","affiliation":[{"name":"Department of Peripheral Vascular Diseases The First Affiliated Hospital of Xi'an Jiaotong University  Xi'an China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shengqing","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Xijing Hospital, Fourth Military Medical University  Xi'an China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jieyan","family":"Shen","sequence":"additional","affiliation":[{"name":"Department of Cardiology Renji Hospital, Shanghai Jiaotong University School of Medicine  Shanghai China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yingqun","family":"Ji","sequence":"additional","affiliation":[{"name":"Department of Respiratory First Affiliated Hospital of Dalian Medical University  Dalian 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