{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T13:23:15Z","timestamp":1790428995256,"version":"4.1.0"},"reference-count":27,"publisher":"European Respiratory Society (ERS)","issue":"4","content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2018,8,10]]},"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:p>The long-term effects of chronic blood exchange transfusions (BETs) on pre-capillary pulmonary hypertension complicating sickle cell disease (SCD) are unknown.<\/jats:p><jats:p>13 homozygous SS SCD patients suffering from pre-capillary pulmonary hypertension and treated by chronic BETs were evaluated retrospectively. Assessments included haemodynamics, New\u00a0York Heart Association Functional Class (NYHA FC), 6-min walk distance (6MWD) and blood tests.<\/jats:p><jats:p>Before initiating BETs, all patients were NYHA FC III or IV, median (range) 6MWD was 223 (0\u2013501)\u2005m and median (range) pulmonary vascular resistance (PVR) was 3.7 (2\u201312.5)\u2005Wood Units. After a median number of 4 BET sessions, all patients had improved to NYHA FC II or III. Significant improvements in haemodynamics were observed, including a decrease in PVR (p=0.01). There was a trend to higher 6MWD (p=0.09). Median (range) follow-up time after initiation of BETs was 25 (6\u201353)\u2005months. During this period, two patients decided to stop BETs. One of them died from acute right heart failure and the other experienced worsening pulmonary hypertension. Two other patients died during follow-up at 25 and 54\u2005months after BET initiation.<\/jats:p><jats:p>Chronic BETs may be a potential therapeutic option in pre-capillary pulmonary hypertension complicating SCD, leading to significant clinical and haemodynamic improvements. These data must be confirmed in a prospective study.<\/jats:p>","DOI":"10.1183\/13993003.00272-2018","type":"journal-article","created":{"date-parts":[[2018,10,10]],"date-time":"2018-10-10T23:25:16Z","timestamp":1539213916000},"page":"1800272","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":34,"title":["Chronic blood exchange transfusions in the management of pre-capillary pulmonary hypertension complicating sickle cell disease"],"prefix":"10.1183","volume":"52","author":[{"given":"Matthieu","family":"Turpin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christelle","family":"Chantalat-Auger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Parent","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7oise","family":"Driss","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Lionnet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anoosha","family":"Habibi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Ma\u00eetre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8545-747X","authenticated-orcid":false,"given":"Alice","family":"Huertas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Ja\u00efs","sequence":"additional","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\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","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"}]},{"given":"G\u00e9rald","family":"Simonneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9deric","family":"Galact\u00e9ros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0703-2892","authenticated-orcid":false,"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pablo","family":"Bartolucci","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"}]}],"member":"81","published-online":{"date-parts":[[2018,10,10]]},"reference":[{"key":"2024101909575755000_52.4.1800272.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1005565"},{"key":"2024101909575755000_52.4.1800272.2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00134410"},{"key":"2024101909575755000_52.4.1800272.3","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-08-618561"},{"key":"2024101909575755000_52.4.1800272.4","doi-asserted-by":"publisher","DOI":"10.1038\/nm799"},{"key":"2024101909575755000_52.4.1800272.5","doi-asserted-by":"publisher","DOI":"10.1001\/jama.294.1.81"},{"key":"2024101909575755000_52.4.1800272.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra0804411"},{"key":"2024101909575755000_52.4.1800272.7","doi-asserted-by":"crossref","first-page":"1436","DOI":"10.5858\/2001-125-1436-PTAIPW","article-title":"Pulmonary thrombotic arteriopathy in patients with sickle cell disease","volume":"125","author":"Adedeji","year":"2001","journal-title":"Arch Pathol Lab Med"},{"key":"2024101909575755000_52.4.1800272.8","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200610-1498OC"},{"key":"2024101909575755000_52.4.1800272.9","doi-asserted-by":"crossref","unstructured":"Mehari A , Igbineweka N , Allen D , et\u00a0al. 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