{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T17:15:11Z","timestamp":1779124511256,"version":"3.51.4"},"reference-count":47,"publisher":"EDP Sciences","issue":"4","license":[{"start":{"date-parts":[[2023,4,24]],"date-time":"2023-04-24T00:00:00Z","timestamp":1682294400000},"content-version":"vor","delay-in-days":23,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"ANR","award":["ANR-20-CE14-0006"],"award-info":[{"award-number":["ANR-20-CE14-0006"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Med Sci (Paris)"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:p>L\u2019hypertension art\u00e9rielle pulmonaire (HTAP) est une maladie rare affectant principalement le lit vasculaire pulmonaire pr\u00e9-capillaire. Certaines formes de la maladie pr\u00e9sentent n\u00e9anmoins une atteinte veinulaire\/capillaire. Il s\u2019agit d\u2019un remodelage obstructif des art\u00e9rioles pulmonaires coupl\u00e9 \u00e0 une rar\u00e9faction vasculaire, augmentant la post-charge ventriculaire<jats:sup>1<\/jats:sup> droite et conduisant \u00e0 une insuffisance cardiaque droite. La physiopathologie de l\u2019HTAP est complexe. Les traitements sp\u00e9cifiques actuels ciblent la dysfonction endoth\u00e9liale, avec une action essentiellement vasodilatatrice. Des traitements innovants prometteurs ciblant le remodelage vasculaire pulmonaire sont en cours de d\u00e9veloppement.<\/jats:p>","DOI":"10.1051\/medsci\/2023053","type":"journal-article","created":{"date-parts":[[2023,4,24]],"date-time":"2023-04-24T11:54:47Z","timestamp":1682337287000},"page":"359-369","source":"Crossref","is-referenced-by-count":2,"title":["Physiopathologie et traitements de l\u2019hypertension art\u00e9rielle pulmonaire"],"prefix":"10.1051","volume":"39","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7730-2427","authenticated-orcid":false,"given":"Fr\u00e9d\u00e9ric","family":"Perros","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1374-1890","authenticated-orcid":false,"given":"\u00c9tienne-Marie","family":"Jutant","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6862-8975","authenticated-orcid":false,"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Dorfm\u00fcller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0703-2892","authenticated-orcid":false,"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"250","published-online":{"date-parts":[[2023,4,24]]},"reference":[{"key":"R1","doi-asserted-by":"crossref","unstructured":"Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2022; ehac237.","DOI":"10.1183\/13993003.00879-2022"},{"key":"R2","doi-asserted-by":"crossref","unstructured":"Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 2022; 2200879.","DOI":"10.1183\/13993003.00879-2022"},{"key":"R3","doi-asserted-by":"crossref","first-page":"1900038","DOI":"10.1183\/13993003.00038-2019","volume":"53","author":"Hoeper","year":"2019","journal-title":"Eur Respir J"},{"key":"R4","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/S2213-2600(20)30317-9","volume":"8","author":"Maron","year":"2020","journal-title":"Lancet Respir Med"},{"key":"R5","doi-asserted-by":"crossref","first-page":"1801887","DOI":"10.1183\/13993003.01887-2018","volume":"53","author":"Humbert","year":"2019","journal-title":"Eur Respir J"},{"key":"R6","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1038\/nrcardio.2017.84","volume":"14","author":"Lau","year":"2017","journal-title":"Nat Rev Cardiol"},{"key":"R7","doi-asserted-by":"crossref","first-page":"1700217","DOI":"10.1183\/13993003.00217-2017","volume":"50","author":"Weatherald","year":"2017","journal-title":"Eur Respir J"},{"key":"R8","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1183\/09031936.00188611","volume":"40","author":"Savale","year":"2012","journal-title":"Eur Respir J"},{"key":"R9","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1056\/NEJM199608293350901","volume":"335","author":"Abenhaim","year":"1996","journal-title":"N Engl J Med"},{"key":"R10","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.1183\/13993003.00814-2015","volume":"46","author":"Montani","year":"2015","journal-title":"Eur Respir J"},{"key":"R11","doi-asserted-by":"crossref","first-page":"1518","DOI":"10.1183\/13993003.00026-2016","volume":"47","author":"Montani","year":"2016","journal-title":"Eur Respir J"},{"key":"R12","doi-asserted-by":"crossref","first-page":"210161","DOI":"10.1183\/16000617.0161-2021","volume":"30","author":"Perros","year":"2021","journal-title":"Eur Respir Rev"},{"key":"R13","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1161\/CIRCULATIONAHA.114.008750","volume":"131","author":"Ranchoux","year":"2015","journal-title":"Circulation"},{"key":"R14","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.ajpath.2014.10.021","volume":"185","author":"Ranchoux","year":"2015","journal-title":"Am J Pathol"},{"key":"R15","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1161\/CIRCULATIONAHA.115.014207","volume":"132","author":"Perros","year":"2015","journal-title":"Circulation"},{"key":"R16","doi-asserted-by":"crossref","first-page":"2045894020907884","DOI":"10.1177\/2045894020907884","volume":"10","author":"Caliez","year":"2020","journal-title":"Pulm Circ"},{"key":"R17","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1016\/j.healun.2017.09.022","volume":"37","author":"Nossent","year":"2018","journal-title":"J Heart Lung Transplant"},{"key":"R18","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1165\/rcmb.2019-0015OC","volume":"63","author":"Manaud","year":"2020","journal-title":"Am J Respir Cell Mol Biol"},{"key":"R19","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1016\/j.drudis.2018.02.008","volume":"23","author":"Santos-Ribeiro","year":"2018","journal-title":"Drug Discov Today"},{"key":"R20","doi-asserted-by":"crossref","first-page":"E443","DOI":"10.3390\/jcm9020443","volume":"9","author":"Valuparampil Varghese","year":"2020","journal-title":"J Clin Med"},{"key":"R21","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1051\/medsci\/2013296013","volume":"29","author":"Perros","year":"2013","journal-title":"Un parfum d\u2019auto-immunit\u00e9. Med Sci (Paris)"},{"key":"R22","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1183\/09031936.00094706","volume":"29","author":"Perros","year":"2007","journal-title":"Eur Respir J"},{"key":"R23","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1378\/chest.14-1678","volume":"147","author":"Hautefort","year":"2015","journal-title":"Chest"},{"key":"R24","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1164\/rccm.201105-0927OC","volume":"185","author":"Perros","year":"2012","journal-title":"Am J Respir Crit Care Med"},{"key":"R25","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1165\/rcmb.2019-0443OC","volume":"63","author":"Koudstaal","year":"2020","journal-title":"Am J Respir Cell Mol Biol"},{"key":"R26","doi-asserted-by":"crossref","first-page":"1756","DOI":"10.3390\/ijms22041756","volume":"22","author":"van Uden","year":"2021","journal-title":"Int J Mol Sci"},{"key":"R27","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1161\/CIRCULATIONAHA.118.033744","volume":"139","author":"Hautefort","year":"2019","journal-title":"Circulation"},{"key":"R28","doi-asserted-by":"crossref","first-page":"3105","DOI":"10.1161\/CIRCULATIONAHA.104.488486","volume":"111","author":"Sitbon","year":"2005","journal-title":"Circulation"},{"key":"R29","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1093\/eurheartj\/ehq170","volume":"31","author":"Montani","year":"2010","journal-title":"Eur Heart J"},{"key":"R30","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1007\/978-3-642-38664-0_9","volume":"218","author":"Clozel","year":"2013","journal-title":"Handb Exp Pharmacol"},{"key":"R31","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1056\/NEJMoa050010","volume":"353","author":"Gali\u00e8","year":"2005","journal-title":"N Engl J Med"},{"key":"R32","doi-asserted-by":"crossref","first-page":"2894","DOI":"10.1161\/CIRCULATIONAHA.108.839274","volume":"119","author":"Gali\u00e8","year":"2009","journal-title":"Circulation"},{"key":"R33","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1056\/NEJMoa1209655","volume":"369","author":"Ghofrani","year":"2013","journal-title":"N Engl J Med"},{"key":"R34","doi-asserted-by":"crossref","first-page":"485","DOI":"10.7326\/0003-4819-112-7-485","volume":"112","author":"Rubin","year":"1990","journal-title":"Ann Intern Med"},{"key":"R35","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1056\/NEJMoa020204","volume":"347","author":"Olschewski","year":"2002","journal-title":"N Engl J Med"},{"key":"R36","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1164\/ajrccm.165.6.2106079","volume":"165","author":"Simonneau","year":"2002","journal-title":"Am J Respir Crit Care Med"},{"key":"R37","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1183\/09031936.00137511","volume":"40","author":"Simonneau","year":"2012","journal-title":"Eur Respir J"},{"key":"R38","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1016\/j.healun.2015.08.014","volume":"34","author":"Yusen","year":"2015","journal-title":"J Heart Lung Transplant"},{"key":"R39","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1038\/nm.3877","volume":"21","author":"Long","year":"2015","journal-title":"Nat Med"},{"key":"R40","doi-asserted-by":"crossref","first-page":"1801899","DOI":"10.1183\/13993003.01899-2018","volume":"53","author":"Morrell","year":"2019","journal-title":"Eur Respir J"},{"key":"R41","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1051\/medsci\/2021131","volume":"37","author":"Humbert","year":"2021","journal-title":"Med Sci (Paris)"},{"key":"R42","doi-asserted-by":"crossref","first-page":"2002341","DOI":"10.1183\/13993003.02341-2020","volume":"57","author":"Guignabert","year":"2021","journal-title":"Eur Respir J"},{"key":"R43","doi-asserted-by":"crossref","first-page":"1204","DOI":"10.1056\/NEJMoa2024277","volume":"384","author":"Humbert","year":"2021","journal-title":"N Engl J Med"},{"key":"R44","doi-asserted-by":"crossref","first-page":"2201347","DOI":"10.1183\/13993003.01347-2022","volume":"61","author":"Humbert","year":"2023","journal-title":"Eur Respir J"},{"key":"R45","doi-asserted-by":"crossref","unstructured":"Hoeper MM, Badesch DB, Ghofrani HA, et al. Phase 3 Trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med 2023 Mar 6. doi:10.1056\/NEJMoa2213558.","DOI":"10.1056\/NEJMoa2213558"},{"key":"R46","doi-asserted-by":"crossref","first-page":"1128","DOI":"10.1161\/CIRCULATIONAHA.112.000765","volume":"127","author":"Hoeper","year":"2013","journal-title":"Circulation"},{"key":"R47","doi-asserted-by":"crossref","first-page":"2102356","DOI":"10.1183\/13993003.02356-2021","volume":"60","author":"Galkin","year":"2022","journal-title":"Eur Respir J"}],"container-title":["m\u00e9decine\/sciences"],"original-title":[],"link":[{"URL":"https:\/\/www.medecinesciences.org\/10.1051\/medsci\/2023053\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,24]],"date-time":"2023-04-24T11:57:37Z","timestamp":1682337457000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.medecinesciences.org\/10.1051\/medsci\/2023053"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4]]},"references-count":47,"journal-issue":{"issue":"4"},"alternative-id":["msc230012"],"URL":"https:\/\/doi.org\/10.1051\/medsci\/2023053","relation":{},"ISSN":["0767-0974","1958-5381"],"issn-type":[{"value":"0767-0974","type":"print"},{"value":"1958-5381","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4]]}}}