{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T06:10:16Z","timestamp":1758867016607,"version":"3.44.0"},"reference-count":25,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,7,18]],"date-time":"2025-07-18T00:00:00Z","timestamp":1752796800000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Evidence on the predictive ability of risk assessment models for event\u2010free survival (EFS) in patients with pulmonary arterial hypertension is scarce. We aimed to investigate the relationship between risk status at 6 months after diagnosis (6\u2009M) and EFS, by three risk models: Multicomponent Improvement (MCI), ESC\/ERS 4\u2010Strata Risk (4SR), and noninvasive French PH Registry Score (FRS). Data collected in the Swedish PAH Registry 2008\u20132021 were used. The study population was risk\u2010stratified at 6\u2009M according to each model. Information on PAH\u2010related hospitalization (HOSP) was collected from the National Patient Register. EFS was defined as survival without occurrence of: (1) HOSP; (2) initiation of parenteral prostacyclin therapy or dose increase \u2265\u200910%; (3) lung transplantation. The association between risk and EFS was evaluated by Kaplan\u2013Meier estimates and Cox proportional models. The analysis included 411 incident patients, median age 66\u2009y [50, 73]. Median survival time was 3.5\u2009y [1.7; 5.4], and cumulative EFS was 55%. In a Cox proportional regression adjusted for age, eGFR, obesity, atrial fibrillation, and systemic hypertension, EFS was higher in patients who: (1) achieved two or three MCI criteria compared to one or no MCI criterion (HR 0.58; CI 0.39\u20130.84, <jats:italic>p<\/jats:italic>\u2009=\u20090.005); (2) were assessed as low, intermediate\u2013low, or intermediate\u2013high compared to high risk (HR 0.16; CI 0.09\u20130.28, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001); or (3) fulfilled one, two, or three low\u2010risk FRS criteria, compared to no low\u2010risk criterion (HR 0.29; CI 0.19\u20130.43, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Performing a risk assessment 6 months after diagnosis effectively predicts the likelihood of EFS in the studied population, highlighting its prognostic value.<\/jats:p>","DOI":"10.1002\/pul2.70132","type":"journal-article","created":{"date-parts":[[2025,7,18]],"date-time":"2025-07-18T13:07:34Z","timestamp":1752844054000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Risk Assessment Models and Event\u2010Free Survival in Pulmonary Arterial Hypertension"],"prefix":"10.1002","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2395-8989","authenticated-orcid":false,"given":"Clara","family":"Hjalmarsson","sequence":"first","affiliation":[{"name":"Department of Cardiology Sahlgrenska University Hospital Gothenburg Sweden"},{"name":"Institute of Medicine at Sahlgrenska Academy University of Gothenburg Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tanvee","family":"Thakur","sequence":"additional","affiliation":[{"name":"Value and Implementation Outcomes Research, Merck &amp; Co. Inc. Rahway New Jersey USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00f6ran","family":"R\u00e5degran","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, Cardiology, Sk\u00e5ne University Hospital Lund University Lund Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Bj\u00f6rklund","sequence":"additional","affiliation":[{"name":"Department of Cardiology University Hospital Uppsala Uppsala Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4753-3811","authenticated-orcid":false,"given":"H\u00e5kan","family":"W\u00e5hlander","sequence":"additional","affiliation":[{"name":"Pediatric Heart Center, The Queen Silvia Children's Hospital Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magnus","family":"Nisell","sequence":"additional","affiliation":[{"name":"Department of Medicine Solna Karolinska Institute Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna\u2010Maria","family":"Papageorgiou","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Institution of Medicine and Health Sciences Link\u00f6ping University Link\u00f6ping Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"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"}]},{"given":"Dominik","family":"Lautsch","sequence":"additional","affiliation":[{"name":"Value and Implementation Outcomes Research, Merck &amp; Co. Inc. Rahway New Jersey USA"}],"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, Sk\u00e5ne University Hospital Lund University Lund Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,7,18]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehac237"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0221211"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1177\/1753466621995040"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/pul2.12190"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcha.2024.101534"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-022-01906-2"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.14-0193"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02419-2021"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2022.03.011"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02311-2021"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00889-2017"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00740-2017"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00837-2020"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehx257"},{"key":"e_1_2_11_16_1","unstructured":"Svenska Pulmonell Arteriell Hypertension Registret (SPAHR) \u00c5rsrapport 2023(Svensk F\u00f6rening f\u00f6r Pulmonell Hypertension 2023) https\/wwwucruuse\/spahr."},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-145-4-200608150-00004"},{"key":"e_1_2_11_18_1","unstructured":"The National Board of Health and Welfare (SoS).National Patient Register(National Board of Health and Welfare 2023) https:\/\/wwwsocialstyrelsense\/en\/statistics-and-data\/registers\/national-patient-register\/."},{"key":"e_1_2_11_19_1","unstructured":"The National Board of Health and Welfare (SoS).National Prescribed Drug Register(National Board of Health and Welfare 2023) https:\/\/wwwsocialstyrelsense\/en\/statistics-and-data\/registers\/national-prescribed-drug-register\/."},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2213558"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2020.08.2069"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2017.12.010"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.026"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02310-2017"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1540-8167.2011.02255.x"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0033902"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pul2.70132","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T05:48:21Z","timestamp":1758865701000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pul2.70132"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7]]},"references-count":25,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,7]]}},"alternative-id":["10.1002\/pul2.70132"],"URL":"https:\/\/doi.org\/10.1002\/pul2.70132","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"type":"print","value":"2045-8940"},{"type":"electronic","value":"2045-8940"}],"subject":[],"published":{"date-parts":[[2025,7]]},"assertion":[{"value":"2025-02-05","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-07-18","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70132"}}