{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T11:38:17Z","timestamp":1790422697355,"version":"4.1.0"},"reference-count":41,"publisher":"European Respiratory Society (ERS)","issue":"4","funder":[{"DOI":"10.13039\/100008593","name":"European Respiratory Society","doi-asserted-by":"publisher","award":["LTRF 2015 \u2013 4780"],"award-info":[{"award-number":["LTRF 2015 \u2013 4780"]}],"id":[{"id":"10.13039\/100008593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["ANR-15-RHUS-0002"],"award-info":[{"award-number":["ANR-15-RHUS-0002"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2018,8,12]]},"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:p>The prognostic importance of follow-up haemodynamics and the validity of multidimensional risk assessment are not well established for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH).<\/jats:p><jats:p>We assessed incident SSc-PAH patients to determine the association between clinical and haemodynamic variables at baseline and first follow-up right heart catheterisation (RHC) with transplant-free survival. RHC variables included cardiac index, stroke volume index (SVI), pulmonary arterial compliance and pulmonary vascular resistance. Risk assessment was performed according to the number of low-risk criteria: functional class I or II, 6-min walking distance (6MWD) &gt;440\u2005m, right atrial pressure &lt;8\u2005mmHg and cardiac index \u22652.5\u2005L\u00b7min<jats:sup>\u22121<\/jats:sup>\u00b7m<jats:sup>\u22122<\/jats:sup>.<\/jats:p><jats:p>Transplant-free survival from diagnosis (n=513) was 87%, 55% and 35% at 1, 3 and 5 years, respectively. At baseline, 6MWD was the only independent predictor. A follow-up RHC was available for 353 patients (median interval 4.6\u2005months, interquartile range 3.9\u20136.4\u2005months). The 6MWD, functional class, cardiac index, SVI, pulmonary arterial compliance and pulmonary vascular resistance were independently associated with transplant-free survival at follow-up, with SVI performing better than other haemodynamic variables. 1-year outcomes were better with increasing number of low-risk criteria at baseline (area under the curve (AUC) 0.63, 95% CI 0.56\u20130.69) and at first follow-up (AUC 0.71, 95% CI 0.64\u20130.78).<\/jats:p><jats:p>Follow-up haemodynamics and multidimensional risk assessment had greater prognostic significance than at baseline in SSc-PAH.<\/jats:p>","DOI":"10.1183\/13993003.00678-2018","type":"journal-article","created":{"date-parts":[[2018,9,12]],"date-time":"2018-09-12T09:15:17Z","timestamp":1536743717000},"page":"1800678","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":81,"title":["Haemodynamics and serial risk assessment in systemic sclerosis associated pulmonary arterial hypertension"],"prefix":"10.1183","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0615-4575","authenticated-orcid":false,"given":"Jason","family":"Weatherald","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ath\u00e9na\u00efs","family":"Boucly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Launay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-0955","authenticated-orcid":false,"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gr\u00e9goire","family":"Pr\u00e9vot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Bourlier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Dauphin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ari","family":"Chaouat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Ja\u00efs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mitja","family":"Jevnikar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Traclet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"De Groote","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rald","family":"Simonneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Mouthon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1942-1951","authenticated-orcid":false,"given":"Olivier","family":"Sitbon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2018,9,12]]},"reference":[{"key":"2024102100355445000_52.4.1800678.1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.62.11.1088"},{"key":"2024102100355445000_52.4.1800678.2","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2009.114264"},{"key":"2024102100355445000_52.4.1800678.3","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"2024102100355445000_52.4.1800678.4","doi-asserted-by":"publisher","DOI":"10.1002\/art.38029"},{"key":"2024102100355445000_52.4.1800678.5","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200912-1820OC"},{"key":"2024102100355445000_52.4.1800678.6","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1016\/j.chest.2017.02.010","article-title":"Idiopathic and systemic sclerosis-associated pulmonary arterial hypertension: a comparison of demographic, hemodynamic, and MRI characteristics and outcomes","volume":"152","author":"Ramjug","year":"2017","journal-title":"Chest"},{"key":"2024102100355445000_52.4.1800678.7","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00092311"},{"key":"2024102100355445000_52.4.1800678.8","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1093\/eurheartj\/ehx025","article-title":"SERAPHIN haemodynamic substudy: the effect of the dual endothelin receptor antagonist macitentan on haemodynamic parameters and NT-proBNP levels and their association with disease progression in patients with pulmonary arterial hypertension","volume":"38","author":"Gali\u00e8","year":"2017","journal-title":"Eur Heart J"},{"key":"2024102100355445000_52.4.1800678.9","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.ijcard.2016.12.099","article-title":"Prognostic relevance of pulmonary arterial compliance after therapy initiation or escalation in patients with pulmonary arterial hypertension","volume":"230","author":"Ghio","year":"2017","journal-title":"Int J Cardiol"},{"key":"2024102100355445000_52.4.1800678.10","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.117.029254"},{"key":"2024102100355445000_52.4.1800678.11","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00889-2017"},{"key":"2024102100355445000_52.4.1800678.12","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-204424"},{"key":"2024102100355445000_52.4.1800678.13","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehl336"},{"key":"2024102100355445000_52.4.1800678.14","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.10.047"},{"key":"2024102100355445000_52.4.1800678.15","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.112.000008"},{"key":"2024102100355445000_52.4.1800678.16","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00135407"},{"key":"2024102100355445000_52.4.1800678.17","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.117.033147"},{"key":"2024102100355445000_52.4.1800678.18","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2011.06.068"},{"key":"2024102100355445000_52.4.1800678.19","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2016.05.015"},{"key":"2024102100355445000_52.4.1800678.20","doi-asserted-by":"crossref","first-page":"3771","DOI":"10.1007\/s00330-016-4217-6","article-title":"Cardiac magnetic resonance findings predicting mortality in patients with pulmonary arterial hypertension: a systematic review and meta-analysis","volume":"26","author":"Baggen","year":"2016","journal-title":"Eur Radiol"},{"key":"2024102100355445000_52.4.1800678.21","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":"2024102100355445000_52.4.1800678.22","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehl477"},{"key":"2024102100355445000_52.4.1800678.23","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2014-306142"},{"key":"2024102100355445000_52.4.1800678.24","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1016\/j.echo.2018.04.013","article-title":"Three-dimensional echocardiography for the assessment of right ventriculo-arterial coupling","volume":"31","author":"Aubert","year":"2018","journal-title":"J Am Soc Echocardiogr"},{"key":"2024102100355445000_52.4.1800678.25","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200604-547OC"},{"key":"2024102100355445000_52.4.1800678.26","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.110512"},{"key":"2024102100355445000_52.4.1800678.27","doi-asserted-by":"publisher","DOI":"10.1002\/art.24267"},{"key":"2024102100355445000_52.4.1800678.28","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2005.069484"},{"key":"2024102100355445000_52.4.1800678.29","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kep398"},{"key":"2024102100355445000_52.4.1800678.30","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"2024102100355445000_52.4.1800678.31","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2012.06.011"},{"key":"2024102100355445000_52.4.1800678.32","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(02)02012-0"},{"key":"2024102100355445000_52.4.1800678.33","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2009.120303"},{"key":"2024102100355445000_52.4.1800678.34","doi-asserted-by":"crossref","first-page":"v33","DOI":"10.1093\/rheumatology\/kex197","article-title":"Pulmonary hypertension related to systemic sclerosis: points to consider for clinical trials","volume":"56","author":"Humbert","year":"2017","journal-title":"Rheumatology"},{"key":"2024102100355445000_52.4.1800678.35","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1186\/s13075-017-1489-4","article-title":"Factors associated with the 6-minute walk distance in patients with systemic sclerosis","volume":"19","author":"Sanges","year":"2017","journal-title":"Arthritis Res Ther"},{"key":"2024102100355445000_52.4.1800678.36","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1007\/s00408-017-0034-1","article-title":"Six-minute walk test as a predictor of diagnosis, disease severity, and clinical outcomes in scleroderma-associated pulmonary hypertension: the DIBOSA study","volume":"195","author":"Gadre","year":"2017","journal-title":"Lung"},{"key":"2024102100355445000_52.4.1800678.37","doi-asserted-by":"crossref","unstructured":"Kylhammar D , Kjellstr\u00f6m B , Hjalmarsson C , et\u00a0al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J 2017; doi: 10.1093\/eurheartj\/ehx257.","DOI":"10.1093\/eurheartj\/ehx257"},{"key":"2024102100355445000_52.4.1800678.38","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00740-2017"},{"key":"2024102100355445000_52.4.1800678.39","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02606-2017"},{"key":"2024102100355445000_52.4.1800678.40","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200806-953OC"},{"key":"2024102100355445000_52.4.1800678.41","doi-asserted-by":"crossref","first-page":"e0197112","DOI":"10.1371\/journal.pone.0197112","article-title":"Clinical phenotypes and survival of pre-capillary pulmonary hypertension in systemic sclerosis","volume":"13","author":"Launay","year":"2018","journal-title":"PLoS One"}],"container-title":["European Respiratory Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1183\/13993003.00678-2018","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T06:59:55Z","timestamp":1740380395000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.ersnet.org\/lookup\/doi\/10.1183\/13993003.00678-2018"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9,12]]},"references-count":41,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2018,10,18]]},"published-print":{"date-parts":[[2018,10]]}},"alternative-id":["10.1183\/13993003.00678-2018"],"URL":"https:\/\/doi.org\/10.1183\/13993003.00678-2018","relation":{},"ISSN":["0903-1936","1399-3003"],"issn-type":[{"value":"0903-1936","type":"print"},{"value":"1399-3003","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9,12]]}}}