{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T08:59:24Z","timestamp":1792400364723,"version":"4.4.0"},"reference-count":36,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2023,2,21]],"date-time":"2023-02-21T00:00:00Z","timestamp":1676937600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"ALF"},{"name":"GoRadCare AB and Nordic Infucare"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,3,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Estimation of prognosis in pulmonary arterial hypertension (PAH) has been influenced by that various risk stratification models use different numbers of prognostic parameters, as well as the lack of a comprehensive and time-saving risk assessment calculator. We therefore evaluated the various European Society of Cardiology (ESC)-\/European Respiratory Society (ERS)-based three- and four-strata risk stratification models and established a comprehensive internet-based calculator to facilitate risk assessment.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>Between 1 January 2000 and 26 July 2021, 773 clinical assessments on 169 incident PAH patients were evaluated at diagnosis and follow-ups. Risk scores were calculated using the original Swedish Pulmonary Arterial Hypertension Registry (SPAHR)\/Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) three-strata model, the updated SPAHR three-strata model with divided intermediate risk, and the simplified three-parameter COMPERA 2.0 four-strata model. The original SPAHR\/COMPERA and the updated SPAHR models were tested for both 3\u20136 and 7\u201311 available parameters, respectively. Prognostic accuracy [area under the receiver operating characteristic (ROC) curve (AUC)] and Uno\u2019s cumulative\/time-dependent C-statistics (uAUC) were calculated for 1-, 3-, and 5-year mortality. At baseline, both the original SPAHR\/COMPERA and the updated SPAHR models, using up to six parameters, provided the highest accuracy (uAUC = 0.73 for both models) in predicting 1-, 3-, and 5-year mortality. At follow-ups, the updated SPAHR model with divided intermediate risk (7\u201311 parameters) provided the highest accuracy for 1-, 3-, and 5-year mortality (uAUC = 0.90), followed by the original SPAHR\/COMPERA model (7\u201311 parameters) (uAUC = 0.88) and the COMPERA 2.0 model (uAUC = 0.85).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>The present study facilitates risk assessment in PAH by introducing a comprehensive internet-based risk score calculator (https:\/\/www.svefph.se\/risk-stratification). At baseline, utilizing the original or the updated SPAHR models using up to six parameters was favourable, the latter model additionally offering sub-characterization of the intermediate risk group. Our findings support the 2022 ESC\/ERS pulmonary hypertension guidelines' strategy for risk stratification suggesting the utilization of a three-strata model at baseline and a simplified four-strata model at follow-ups. Our findings furthermore support the utility of the updated SPAHR model with divided intermediate risk, when a more comprehensive assessment is needed at follow-ups, complementing the three-parameter COMPERA 2.0 model. Larger multi-centre studies are encouraged to validate the utility of the updated SPAHR model.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Take home message<\/jats:title>\n                  <jats:p>By introducing an internet-based risk score calculator (https:\/\/www.svefph.se\/risk-stratification), risk assessment is facilitated. Our results support the 2022 ESC\/ERS pulmonary hypertension guidelines' risk stratification strategy, additionally suggesting the updated SPAHR three-strata model with divided intermediate risk, as a promising complement to the new simplified three-parameter COMPERA 2.0 four-strata strategy, when a more comprehensive overview is needed.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjopen\/oead012","type":"journal-article","created":{"date-parts":[[2023,2,21]],"date-time":"2023-02-21T19:06:32Z","timestamp":1677006392000},"source":"Crossref","is-referenced-by-count":16,"title":["Evaluation of the European Society of Cardiology\/European Respiratory Society derived three- and four-strata risk stratification models in pulmonary arterial hypertension: introducing an internet-based risk stratification calculator"],"prefix":"10.1093","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6005-5300","authenticated-orcid":false,"given":"Abdulla","family":"Ahmed","sequence":"first","affiliation":[{"name":"Department of Clinical Sciences Lund, The Section for Cardiology, Lund University , Lund ,","place":["Sweden"]},{"name":"The Haemodynamic Lab, The Section for Heart Failure and Valvular Disease, Heart and Lung Medicine, Sk\u00e5ne University Hospital , Lund ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7086-6262","authenticated-orcid":false,"given":"Salaheldin","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, The Section for Cardiology, Lund University , Lund ,","place":["Sweden"]},{"name":"The Haemodynamic Lab, The Section for Heart Failure and Valvular Disease, Heart and Lung Medicine, Sk\u00e5ne University Hospital , Lund ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Kempe","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, The Section for Cardiology, Lund University , Lund ,","place":["Sweden"]},{"name":"The Haemodynamic Lab, The Section for Heart Failure and Valvular Disease, Heart and Lung Medicine, Sk\u00e5ne University Hospital , Lund ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4919-2771","authenticated-orcid":false,"given":"G\u00f6ran","family":"R\u00e5degran","sequence":"additional","affiliation":[{"name":"Department of Clinical Sciences Lund, The Section for Cardiology, Lund University , Lund ,","place":["Sweden"]},{"name":"The Haemodynamic Lab, The Section for Heart Failure and Valvular Disease, Heart and Lung Medicine, Sk\u00e5ne University Hospital , Lund ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,2,21]]},"reference":[{"key":"2025012214022828400_oead012-B1","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1016\/S2213-2600(15)00543-3","article-title":"A global view of pulmonary hypertension","volume":"4","author":"Hoeper","year":"2016","journal-title":"Lancet Respir Med"},{"key":"2025012214022828400_oead012-B2","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1378\/chest.14-0701","article-title":"Signs of right ventricular deterioration in clinically stable patients with pulmonary arterial hypertension","volume":"147","author":"van de Veerdonk","year":"2015","journal-title":"Chest"},{"key":"2025012214022828400_oead012-B3","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT)","volume":"37","author":"Gali\u00e8","year":"2016","journal-title":"Eur Heart J"},{"key":"2025012214022828400_oead012-B4","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1183\/13993003.01032-2015","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"46","author":"Gali\u00e8","year":"2015","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B5","doi-asserted-by":"crossref","first-page":"1801889","DOI":"10.1183\/13993003.01889-2018","article-title":"Risk stratification and medical therapy of pulmonary arterial hypertension","volume":"53","author":"Gali\u00e8","year":"2019","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B6","doi-asserted-by":"crossref","first-page":"1285","DOI":"10.1378\/chest.10-1293","article-title":"Integration of clinical and hemodynamic parameters in the prediction of long-term survival in patients with pulmonary arterial hypertension","volume":"139","author":"Kane","year":"2011","journal-title":"Chest"},{"key":"2025012214022828400_oead012-B7","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1161\/CIRCULATIONAHA.109.898122","article-title":"Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)","volume":"122","author":"Benza","year":"2010","journal-title":"Circulation"},{"key":"2025012214022828400_oead012-B8","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1183\/09059180.00006711","article-title":"Prognostic factors in pulmonary arterial hypertension: assessing the course of the disease","volume":"20","author":"Howard","year":"2011","journal-title":"Eur Respir Rev"},{"key":"2025012214022828400_oead012-B9","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1183\/09031936.00057010","article-title":"Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension","volume":"36","author":"Humbert","year":"2010","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B10","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1164\/rccm.201709-1840PP","article-title":"The low-risk profile in pulmonary arterial hypertension. Time for a paradigm shift to goal-oriented clinical trial endpoints?","volume":"197","author":"Weatherald","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"2025012214022828400_oead012-B11","doi-asserted-by":"crossref","first-page":"843","DOI":"10.1164\/rccm.201801-0042ED","article-title":"Risk assessment tools in pulmonary arterial hypertension. Prognosis for prospective trials?","volume":"197","author":"Farber","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"2025012214022828400_oead012-B12","first-page":"ehac237","article-title":"2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on rare respiratory diseases (ERN-LUNG)","author":"Humbert","year":"2022","journal-title":"Eur Heart J"},{"key":"2025012214022828400_oead012-B13","first-page":"2200879","article-title":"2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"2022","author":"Humbert","year":"2022","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B14","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/j.healun.2022.03.011","article-title":"Prognostic value of improvement endpoints in pulmonary arterial hypertension trials: a COMPERA analysis","volume":"41","author":"Hoeper","year":"2022","journal-title":"J Heart Lung Transplant"},{"key":"2025012214022828400_oead012-B15","doi-asserted-by":"crossref","first-page":"1700889","DOI":"10.1183\/13993003.00889-2017","article-title":"Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension","volume":"50","author":"Boucly","year":"2017","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B16","doi-asserted-by":"crossref","first-page":"4175","DOI":"10.1093\/eurheartj\/ehx257","article-title":"A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension","volume":"39","author":"Kylhammar","year":"2018","journal-title":"Eur Heart J"},{"key":"2025012214022828400_oead012-B17","doi-asserted-by":"crossref","first-page":"1700740","DOI":"10.1183\/13993003.00740-2017","article-title":"Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model","volume":"50","author":"Hoeper","year":"2017","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B18","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1378\/chest.11-0676","article-title":"The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension","volume":"141","author":"Benza","year":"2012","journal-title":"Chest"},{"key":"2025012214022828400_oead012-B19","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/j.chest.2019.02.004","article-title":"Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 2.0 and comparison with ESC\/ERS-based risk assessment strategies","volume":"156","author":"Benza","year":"2019","journal-title":"Chest"},{"key":"2025012214022828400_oead012-B20","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1016\/j.chest.2020.08.2069","article-title":"Development and validation of an abridged version of the REVEAL 2.0 risk score calculator, REVEAL Lite 2, for use in patients with pulmonary arterial hypertension","volume":"159","author":"Benza","year":"2021","journal-title":"Chest"},{"key":"2025012214022828400_oead012-B21","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/j.healun.2020.03.026","article-title":"Incorporation of renal function in mortality risk assessment for pulmonary arterial hypertension","volume":"39","author":"Zelt","year":"2020","journal-title":"J Heart Lung Transplant"},{"key":"2025012214022828400_oead012-B22","doi-asserted-by":"crossref","first-page":"2102311","DOI":"10.1183\/13993003.02311-2021","article-title":"COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension","volume":"60","author":"Hoeper","year":"2022","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B23","doi-asserted-by":"crossref","first-page":"00837","DOI":"10.1183\/23120541.00837-2020","article-title":"Predicting mortality during long-term follow-up in pulmonary arterial hypertension","volume":"7","author":"Kylhammar","year":"2021","journal-title":"ERJ Open Res"},{"key":"2025012214022828400_oead012-B24","first-page":"1","article-title":"Advanced risk stratification of intermediate risk group in pulmonary arterial hypertension","volume":"10","author":"Yogeswaran","year":"2020","journal-title":"Pulm Circ"},{"key":"2025012214022828400_oead012-B25","doi-asserted-by":"crossref","first-page":"2102419","DOI":"10.1183\/13993003.02419-2021","article-title":"External validation of a refined four-stratum risk assessment score from the French Pulmonary Hypertension Registry","volume":"59","author":"Boucly","year":"2022","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B26","doi-asserted-by":"crossref","first-page":"2351","DOI":"10.1007\/s12325-019-01030-4","article-title":"Assessment of risk of disease progression in pulmonary arterial hypertension: insights from an international survey of clinical practice","volume":"36","author":"Simons","year":"2019","journal-title":"Adv Ther"},{"key":"2025012214022828400_oead012-B27","doi-asserted-by":"crossref","first-page":"e12106","DOI":"10.1002\/pul2.12106","article-title":"Clinical application of risk assessment in PAH: expert center APRN recommendations","volume":"12","author":"Wilson","year":"2022","journal-title":"Pulm Circ"},{"key":"2025012214022828400_oead012-B28"},{"key":"2025012214022828400_oead012-B29","doi-asserted-by":"crossref","first-page":"1702606","DOI":"10.1183\/13993003.02606-2017","article-title":"Risk assessment in pulmonary arterial hypertension","volume":"51","author":"Hoeper","year":"2018","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B30","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1002\/sim.4154","article-title":"On the c-statistics for evaluating overall adequacy of risk prediction procedures with censored survival data","volume":"30","author":"Uno","year":"2011","journal-title":"Stat Med"},{"key":"2025012214022828400_oead012-B31","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1183\/09031936.00092311","article-title":"The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension","volume":"39","author":"Nickel","year":"2012","journal-title":"Eur Respir J"},{"key":"2025012214022828400_oead012-B32","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1164\/rccm.201909-1771OC","article-title":"Identification of cardiac magnetic resonance imaging thresholds for risk stratification in pulmonary arterial hypertension","volume":"201","author":"Lewis","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"key":"2025012214022828400_oead012-B33","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1164\/rccm.201611-2365OC","article-title":"Magnetic resonance imaging in the prognostic evaluation of patients with pulmonary arterial hypertension","volume":"196","author":"Swift","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"2025012214022828400_oead012-B34","doi-asserted-by":"crossref","first-page":"1575","DOI":"10.1016\/j.chest.2020.10.077","article-title":"The value of hemodynamic measurements or cardiac MRI in the follow-up of patients with idiopathic pulmonary arterial hypertension","volume":"159","author":"van der Bruggen","year":"2021","journal-title":"Chest"},{"key":"2025012214022828400_oead012-B35","doi-asserted-by":"crossref","first-page":"S178","DOI":"10.1513\/AnnalsATS.201312-443LD","article-title":"Pulmonary hypertension: NHLBI workshop on the primary prevention of chronic lung diseases","volume":"11","author":"Austin","year":"2014","journal-title":"Ann Am Thorac Soc"},{"key":"2025012214022828400_oead012-B36","doi-asserted-by":"crossref","first-page":"170095","DOI":"10.1183\/16000617.0095-2017","article-title":"Pulmonary arterial hypertension: tailoring treatment to risk in the current era","volume":"26","author":"Gaine","year":"2017","journal-title":"Eur Respir Rev"}],"container-title":["European Heart Journal Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ehjopen\/advance-article-pdf\/doi\/10.1093\/ehjopen\/oead012\/49286045\/oead012.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjopen\/article-pdf\/3\/2\/oead012\/49572170\/oead012.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjopen\/article-pdf\/3\/2\/oead012\/49572170\/oead012.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,22]],"date-time":"2025-01-22T14:02:56Z","timestamp":1737554576000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjopen\/article\/doi\/10.1093\/ehjopen\/oead012\/7049581"}},"subtitle":[],"editor":[{"given":"Denis","family":"Wahl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2023,2,21]]},"references-count":36,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2023,3,2]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjopen\/oead012","relation":{},"ISSN":["2752-4191"],"issn-type":[{"value":"2752-4191","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,3,1]]},"published":{"date-parts":[[2023,2,21]]},"article-number":"oead012"}}