{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T01:09:27Z","timestamp":1789175367695,"version":"build-2803163510"},"reference-count":24,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,4,15]],"date-time":"2021-04-15T00:00:00Z","timestamp":1618444800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006492","name":"Division of Intramural Research, National Institute of Allergy and Infectious Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006492","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Sickle cell disease\u2010related pulmonary hypertension (SCD\u2010PH) is a complex disorder with multifactorial contributory mechanisms. Previous trials have evaluated the efficacy of pulmonary arterial hypertension (PAH) therapies in SCD\u2010PH with mixed results. We hypothesized that a subset of patients with right heart catheterization (RHC) confirmed disease may benefit from PAH therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a retrospective chart review of patients with SCD\u2010PH diagnosed by RHC who were treated with phosphodiesterase 5 inhibitor (PDE5\u2010I) therapy for \u22654\u00a0months between 2008 and 2019 at two institutions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010six patients were included in the analysis. The median age (IQR) upon PDE5\u2010I initiation was 47.5\u00a0years (35\u201051.5\u00a0years); 58% were female and twenty\u2010nine (81%) had HbSS disease. Of these, 53% of patients had a history of acute chest syndrome, 42% had a history of venous thromboembolism, and 38% had imaging consistent with chronic thromboembolic PH. Patients were treated for a median duration of 25\u00a0months (IQR 13\u201060\u00a0months). Use of PDE5\u2010I was associated with a significant improvement in symptoms as assessed by NYHA Class (<jats:italic>P<\/jats:italic>\u00a0=\u00a0.002).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In SCD patients with PH defined by RHC, PDE5\u2010I therapy was tolerated long\u2010term and may improve physical activity.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.13612","type":"journal-article","created":{"date-parts":[[2021,3,2]],"date-time":"2021-03-02T07:57:25Z","timestamp":1614671845000},"page":"54-62","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Long\u2010term tolerability of phosphodiesterase\u20105 inhibitors in pulmonary hypertension of sickle cell disease"],"prefix":"10.1111","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5274-0666","authenticated-orcid":false,"given":"Cassondra","family":"Cramer\u2010Bour","sequence":"first","affiliation":[{"name":"Department of Medicine Boston University School of Medicine  Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy Parker","family":"Ruhl","sequence":"additional","affiliation":[{"name":"Laboratory of Malaria and Vector Research National Institute of Allergy and Infectious Diseases National Institutes of Health  Bethesda MD USA"},{"name":"Pulmonary Branch National Heart, Lung, and Blood Institute  Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seyed Mehdi","family":"Nouraie","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Asthma and Critical Care Medicine Department of Medicine University of Pittsburgh  Pittsburgh PA 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S.","family":"Klings","sequence":"additional","affiliation":[{"name":"Department of Medicine Boston University School of Medicine  Boston MA USA"},{"name":"The Pulmonary Center Boston University School of Medicine  Boston MA 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