{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T23:53:09Z","timestamp":1792799589064,"version":"4.5.0"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T00:00:00Z","timestamp":1769817600000},"content-version":"vor","delay-in-days":91,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Assistance Publique \u2013 H\u00f4pitaux de Paris","id":[{"id":"https:\/\/ror.org\/00pg5jh14","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100002738","name":"Assistance Publique \u2013 H\u00f4pitaux de Paris","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002738","id-type":"DOI","asserted-by":"publisher"},{"id":"https:\/\/ror.org\/00pg5jh14","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2025,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Pulmonary hypertension is a serious cardiopulmonary complication of sickle cell disease, but the prognostic impact of hemodynamic parameters remains poorly defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>This study aimed to assess the clinical and long-term prognostic relevance of hemodynamic parameters in sickle cell disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data were analyzed from the French ETENDARD cohort. All 398 participants underwent echocardiography; those with tricuspid regurgitation velocity \u22652.5 m\/s proceeded to right heart catheterization. Over a minimum 10-year follow-up, we examined the clinical and prognostic significance of hemodynamic variables.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>Pulmonary hypertension defined by a mean pulmonary arterial pressure (mPAP) &amp;gt;20 mm Hg was identified in 44 patients (11%). Among them, 26 (6.5%) had a pulmonary arterial wedge pressure \u226415 mm Hg, and 18 (4.5%) had a pulmonary arterial wedge pressure \u2009&amp;gt;15 mm Hg. Mortality during follow-up was 11.1%. A significant association was found between pulmonary vascular resistance (PVR) levels and mortality risk, with a threshold identified at 1.5 Wood units (WU) through receiver operating characteristic curve analyses. In patients with mPAP &amp;gt;20 mm Hg and PVR \u22651.5 WU, the adjusted hazard ratio for mortality was 4.27 (95% confidence interval, 1.88\u20139.74; P\u2009&amp;lt;\u20090.001). A hemolytic phenotype and the presence of systemic complications, including hypertension, left ventricular diastolic dysfunction, renal impairment, and leg ulcers, were associated with elevated PVR and increased mortality risk.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>A PVR threshold of 1.5 WU emerges as a key predictor of mortality in patients with an mPAP above 20 mm Hg, particularly when assessed in conjunction with markers of hemolysis and systemic complications.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.202502-0353oc","type":"journal-article","created":{"date-parts":[[2025,9,12]],"date-time":"2025-09-12T15:26:12Z","timestamp":1757690772000},"page":"2096-2104","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":13,"title":["Cardiopulmonary Hemodynamic Determinants of Long-Term Survival in Sickle Cell Disease: Insights from the ETENDARD Study"],"prefix":"10.1093","volume":"211","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6862-8975","authenticated-orcid":false,"given":"Laurent","family":"Savale","sequence":"first","affiliation":[{"name":"INSERM, UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies (HPPIT), Le Kremlin-Bic\u00eatre 94276, France"},{"name":"Facult\u00e9 de M\u00e9decine, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Service de Pneumologie et Soins Intensifs Respiratoires, Assistance Publique \u2013 H\u00f4pitaux de Paris, H\u00f4pital Bic\u00eatre, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0414-0962","authenticated-orcid":false,"given":"Thomas","family":"d\u2019Humi\u00e8res","sequence":"additional","affiliation":[{"name":"Physiology Department, FHU SENCODE\/REDGENE, Henri Mondor Hospital, Assistance Publique \u2013 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