{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T03:48:36Z","timestamp":1747194516084,"version":"3.40.5"},"reference-count":19,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"vor","delay-in-days":62,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,7,1]],"date-time":"2020-07-01T00:00:00Z","timestamp":1593561600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100009240","name":"Sveriges Kommuner och Landsting","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009240","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004334","name":"Merck","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004334","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004326","name":"Bayer","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004326","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004334","name":"Merck","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004334","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2020,7]]},"abstract":"<jats:p>Phosphodiesterase\u20105\u00a0inhibitors are commonly used in pulmonary arterial hypertension but, as suggested by the RESPITE study, phosphodiesterase\u20105\u00a0inhibitor therapy (mono\u2010\/combination) does not always have a satisfactory treatment effect. This study aimed to investigate the clinical course of pulmonary arterial hypertension patients not at treatment goal after at least 90\u00a0days of treatment with phosphodiesterase\u20105\u00a0inhibitors, alone or in combination with other pulmonary arterial hypertension therapies. The study included 106\u00a0incident patients from the Swedish Pulmonary Arterial Hypertension Registry, treated with phosphodiesterase\u20105\u00a0inhibitors for \u226590\u00a0days, who were not at a pre\u2010specified treatment goal, i.e. in World Health Organisation functional class III, with 6\u2010min walking distance 165\u2013440\u2009m, and N\u2010terminal prohormone of brain natriuretic peptide &gt;300\u2009ng\/L. Changes in World Health Organisation functional class, 6\u2010min walking distance, N\u2010terminal prohormone of brain natriuretic peptide, and risk group between index and follow\u2010up were assessed. Of patients with complete follow\u2010up data, (<jats:italic>n<\/jats:italic>\u2009=\u200953) 77% were on combination therapy and risk assessment yielded 98% at intermediate risk at index. At follow\u2010up, 11 patients transitioned from World Health Organisation functional class III to World Health Organisation functional class II, the median (Q1; Q3) change in 6\u2010min walking distance was 6 (\u221230; 42) meters and in N\u2010terminal prohormone of brain natriuretic peptide 47 (\u2212410; 603) ng\/L, while 89% remained at an intermediate risk. Of those without complete follow\u2010up data, 11 patients died and 2 underwent lung transplantation. In conclusion, pulmonary arterial hypertension patients treated with phosphodiesterase\u20105\u00a0inhibitors, as single or combination therapy and not achieving the pre\u2010specified treatment goals after \u226590\u00a0days have an unfavourable clinical course.<\/jats:p>","DOI":"10.1177\/2045894020958557","type":"journal-article","created":{"date-parts":[[2020,9,28]],"date-time":"2020-09-28T09:31:54Z","timestamp":1601285514000},"page":"1-9","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Poor outcome of patients with pulmonary arterial hypertension with insufficient response to phosphodiesterase\u20105\u00a0inhibitors alone or in combination with other specific therapy: a registry\u2010based study"],"prefix":"10.1002","volume":"10","author":[{"given":"Clara","family":"Hjalmarsson","sequence":"first","affiliation":[{"name":"Department of Molecular and Clinical Medicine Sahlgrenska Academy Gothenburg University Department of Cardiology Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oisin","family":"Butler","sequence":"additional","affiliation":[{"name":"Bayer AG Berlin Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roger","family":"Hesselstrand","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, Rheumatology Lund University Sk\u00e5ne University Hospital Lund Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsiaryna","family":"Holl","sequence":"additional","affiliation":[{"name":"Bayer AG Berlin Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kjell","family":"Jansson","sequence":"additional","affiliation":[{"name":"Department of Cardiology and Department of Clinical Physiology Institution of Medicine and Health Sciences Link\u00f6ping University Link\u00f6ping Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6584-4886","authenticated-orcid":false,"given":"Rogier","family":"Klok","sequence":"additional","affiliation":[{"name":"Merck &amp; Co., Inc. Kenilworth NJ USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00f6ran","family":"R\u00e5degran","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, Cardiology The Section for Heart Failure and Valvular Disease Lund University Sk\u00e5ne University Hospital Lund Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9225-1306","authenticated-orcid":false,"given":"Stefan","family":"S\u00f6derberg","sequence":"additional","affiliation":[{"name":"Department of Public Health and Clinical Medicine, and Heart Centre Ume\u00e5 University Ume\u00e5 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7936-1209","authenticated-orcid":false,"given":"Barbro","family":"Kjellstr\u00f6m","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, Clinical Physiology Lund University Sk\u00e5ne University Hospital Lund Sweden"},{"name":"Cardiology Unit Department of Medicine Karolinska Institute Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,9]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehv317"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1080\/14017431.2016.1185532"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01889-2018"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehx257"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00889-2017"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02425-2016"},{"key":"e_1_2_12_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehp297"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-130-6-199903160-00002"},{"key":"e_1_2_12_11_1","unstructured":"Swedish pulmoary arterial hypertension registry (SPAHR). Annual report (\u00c5rsrapport)www.ucr.uu.se\/spahr\/arsrapporter(2019 accessed 2 September 2020)."},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213917"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02310-2017"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2012.10.026"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045894018790316"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045894018769874"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39478"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0197112"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38029"},{"key":"e_1_2_12_20_1","first-page":"34645","article-title":"Identifying potential parameters associated with response to switching from a PDE5i to riociguat in RESPITE","volume":"0167","author":"Benza RL","year":"2020","journal-title":"Int J Cardiol"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894020958557","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1177\/2045894020958557","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894020958557","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T13:17:30Z","timestamp":1693228650000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1177\/2045894020958557"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,7]]},"references-count":19,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2020,7]]}},"alternative-id":["10.1177\/2045894020958557"],"URL":"https:\/\/doi.org\/10.1177\/2045894020958557","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"type":"print","value":"2045-8940"},{"type":"electronic","value":"2045-8940"}],"subject":[],"published":{"date-parts":[[2020,7]]}}}