{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T19:08:48Z","timestamp":1792868928158,"version":"4.5.0"},"reference-count":64,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2023,9,1]],"date-time":"2023-09-01T00:00:00Z","timestamp":1693526400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":["www.atsjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Precapillary pulmonary hypertension (PH) is a rare and largely unrecognized complication of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (MF).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To describe characteristics and outcomes of MPN-associated PH.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We report clinical, functional, and hemodynamic characteristics, classification, and outcomes of patients with PV, ET, or primary MF in the French PH registry.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Measurements and Main Results<\/jats:title>\n                    <jats:p>Ninety patients with MPN (42 PV, 35 ET, 13 primary MF) presented with precapillary PH with severe hemodynamic impairment, with a median mean pulmonary arterial pressure and pulmonary vascular resistance of 42\u2009mm\u2009Hg and 6.7 Wood units, respectively, and impaired clinical conditions, with 71% in New York Heart Association functional classes III\/IV and having a median 6-minute-walk distance of 310\u2009m. Half of the patients were diagnosed with chronic thromboembolic PH (CTEPH); the other half were considered to have group 5 PH. MF was preferentially associated with group 5 PH, whereas PV and ET were generally related to CTEPH. Proximal lesions were diagnosed in half of the patients with CTEPH. Thromboendarterectomy was performed in 18 selected patients with high risk of complications (5 early deaths). Overall survival at 1, 3, and 5\u2009years was 67%, 50%, and 34% in group 5 PH and 81%, 66%, and 42% in CTEPH, respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>PH is a life-threatening condition potentially occurring in MPN. There are multiple mechanisms, with equal diagnoses of CTEPH and group 5 PH. Physicians should be aware that PH strongly affects the burden of patients with MPN, especially in group 5 PH, with unknown pathophysiological mechanisms.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1164\/rccm.202210-1941oc","type":"journal-article","created":{"date-parts":[[2023,6,13]],"date-time":"2023-06-13T17:23:55Z","timestamp":1686677035000},"page":"600-612","update-policy":"https:\/\/doi.org\/10.1164\/crossmarkpolicies","source":"Crossref","is-referenced-by-count":32,"title":["Clinical Phenotype and Outcomes of Pulmonary Hypertension Associated with Myeloproliferative Neoplasms: A Population-based Study"],"prefix":"10.1093","volume":"208","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","sequence":"first","affiliation":[{"name":"School of Medicine, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Assistance Publique - H\u00f4pitaux de Paris, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, H\u00f4pital Bic\u00eatre, Le Kremlin-Bic\u00eatre, France"},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 Mixte de Recherche 999 Pulmonary Hypertension: Pathophysiology and Novel Therapies and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7378-2419","authenticated-orcid":false,"given":"Pierre","family":"Thor\u00e9","sequence":"additional","affiliation":[{"name":"Assistance Publique - H\u00f4pitaux de Paris, Department of Respiratory Medicine, Histiocytosis National Referral Center, H\u00f4pital Saint-Louis, Paris, France"},{"name":"School of Medicine, Universit\u00e9 Paris Cit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5458-7770","authenticated-orcid":false,"given":"Xavier","family":"Mignard","sequence":"additional","affiliation":[{"name":"School of Medicine, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Assistance Publique - H\u00f4pitaux de Paris, Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, H\u00f4pital Bic\u00eatre, Le Kremlin-Bic\u00eatre, France"},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale Unit\u00e9 Mixte de Recherche 999 Pulmonary Hypertension: Pathophysiology and Novel Therapies and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4104-7994","authenticated-orcid":false,"given":"Xavier","family":"Ja\u00efs","sequence":"additional","affiliation":[{"name":"School of Medicine, Universit\u00e9 Paris-Saclay, Le Kremlin-Bic\u00eatre, France"},{"name":"Assistance Publique - 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