{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T22:10:55Z","timestamp":1793916655575,"version":"4.8.0"},"reference-count":24,"publisher":"European Respiratory Society (ERS)","issue":"6","license":[{"start":{"date-parts":[[2021,11,4]],"date-time":"2021-11-04T00:00:00Z","timestamp":1635984000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2021,10,28]]},"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Contemporary risk assessment tools categorise patients with pulmonary arterial hypertension (PAH) as low, intermediate or high risk. A minority of patients achieve low risk status with most remaining intermediate risk. Our aim was to validate a four-stratum risk assessment approach categorising patients as low, intermediate-low, intermediate-high or high risk, as proposed by the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) investigators.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We evaluated incident patients from the French PAH Registry and applied a four-stratum risk method at baseline and at first reassessment. We applied refined cut-points for three variables: World Health Organization functional class, 6-min walk distance and N-terminal pro-brain natriuretic peptide. We used Kaplan\u2013Meier survival analyses and Cox proportional hazards regression to assess survival according to three-stratum and four-stratum risk approaches.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>At baseline (n=2879), the four-stratum approach identified four distinct risk groups and performed slightly better than a three-stratum method for predicting mortality. Four-stratum model discrimination was significantly higher than the three-stratum method when applied during follow-up and refined risk categories among subgroups with idiopathic PAH, connective tissue disease-associated PAH, congenital heart disease and portopulmonary hypertension. Using the four-stratum approach, 53% of patients changed risk category from baseline compared to 39% of patients when applying the three-stratum approach. Those who achieved or maintained a low risk status had the best survival, whereas there were more nuanced differences in survival for patients who were intermediate-low and intermediate-high risk.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The four-stratum risk assessment method refined risk prediction, especially within the intermediate risk category of patients, performed better at predicting survival and was more sensitive to change than the three-stratum approach.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1183\/13993003.02419-2021","type":"journal-article","created":{"date-parts":[[2021,11,4]],"date-time":"2021-11-04T13:00:32Z","timestamp":1636030832000},"page":"2102419","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":200,"title":["External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry"],"prefix":"10.1183","volume":"59","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6246-5557","authenticated-orcid":false,"given":"Ath\u00e9na\u00efs","family":"Boucly","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0615-4575","authenticated-orcid":false,"given":"Jason","family":"Weatherald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6862-8975","authenticated-orcid":false,"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"de 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COMPERA 2.0: a refined 4-stratum risk assessment model for pulmonary arterial hypertension. Eur Respir J 2021; in press [https:\/\/doi.org\/10.1183\/13993003.02311-2021].","DOI":"10.1183\/13993003.02311-2021"},{"key":"2024102102443574000_59.6.2102419.16","doi-asserted-by":"publisher","DOI":"10.1177\/1536867X1001000303"},{"key":"2024102102443574000_59.6.2102419.17","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01889-2018"},{"key":"2024102102443574000_59.6.2102419.18","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1164\/rccm.201709-1840PP","article-title":"The low-risk profile in pulmonary arterial hypertension. 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