{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T15:13:10Z","timestamp":1787497990803,"version":"build-2736575974"},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T00:00:00Z","timestamp":1561939200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T00:00:00Z","timestamp":1561939200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/100007150","name":"United Therapeutics Corporation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007150","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007150","name":"United Therapeutics Corporation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007150","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pulm. circ."],"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:p>Treprostinil, a prostacyclin analogue, is approved for the treatment of pulmonary arterial hypertension (PAH) in adults. Transition from parenteral to oral treprostinil has been successfully accomplished in adults with PAH but not in children. In this multicenter study, pediatric patients treated with parenteral (Cohort 1) or inhaled (Cohort 2) treprostinil were transitioned to oral treprostinil. Prostacyclin\u2010na\u00efve individuals on background oral PAH therapy received oral treprostinil as add\u2010on therapy (Cohort 3). Successful transition was oral treprostinil dose maintenance through week 24. Patients were monitored for adverse events (AEs), 6\u2010min walk distance (6MWD), PAH symptoms, World Health Organization (WHO) Functional Class (FC), cardiac magnetic resonance imaging (cMRI), cardiopulmonary exercise testing (CPET), and quality of life through 24 weeks. A total of 32 patients were enrolled in the study; 23 (72%) were girls (mean age\u2009=\u200912.2 years). All patients were on background oral PAH therapy. Overall, patients (96.9%) maintained transition to oral treprostinil; one patient (Cohort 1) transitioned to oral treprostinil, then back to parenteral after experiencing syncope and WHO FC change from II to III. Cohorts 1, 2, and 3 received a final mean oral treprostinil dose of 5.6, 3.3, and 4.5\u2009mg t.i.d., respectively. All cohorts had variable changes in 6MWD, cMRI, and CPET. Overall, 12 serious AEs were reported. All patients had drug\u2010related AEs including headache (81%), diarrhea (69%), nausea (66%), vomiting (66%), and flushing (56%). Pediatric patients maintained transition to oral treprostinil with preservation of exercise capacity and WHO FC. Prostanoid\u2010related AEs were most common and similar to those reported in adults.<\/jats:p>","DOI":"10.1177\/2045894019856471","type":"journal-article","created":{"date-parts":[[2019,6,19]],"date-time":"2019-06-19T08:00:16Z","timestamp":1560931216000},"page":"1-8","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":15,"title":["Oral treprostinil in transition or as add\u2010on therapy in pediatric pulmonary arterial hypertension"],"prefix":"10.1002","volume":"9","author":[{"given":"D. Dunbar","family":"Ivy","sequence":"first","affiliation":[{"name":"Children\u2019s Hospital of Colorado Aurora CO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey A.","family":"Feinstein","sequence":"additional","affiliation":[{"name":"Lucile Packard Children\u2019s Hospital Stanford and Stanford University School of Medicine Palo Alto CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Yung","sequence":"additional","affiliation":[{"name":"Seattle Children\u2019s Hospital Seattle WA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary P.","family":"Mullen","sequence":"additional","affiliation":[{"name":"Boston Children\u2019s Hospital Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward C.","family":"Kirkpatrick","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Wisconsin Milwaukee WI USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russel","family":"Hirsch","sequence":"additional","affiliation":[{"name":"Cincinnati Children\u2019s Hospital Cincinnati OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric D.","family":"Austin","sequence":"additional","affiliation":[{"name":"Monroe Carell Jr. Children\u2019s Hospital at Vanderbilt Nashville TN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey","family":"Fineman","sequence":"additional","affiliation":[{"name":"University of California San Francisco San Francisco CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uyen","family":"Truong","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Colorado Aurora CO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Derek","family":"Solum","sequence":"additional","affiliation":[{"name":"United Therapeutics Research Triangle Park NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.Q.","family":"Deng","sequence":"additional","affiliation":[{"name":"United Therapeutics Research Triangle Park NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel K.","family":"Hopper","sequence":"additional","affiliation":[{"name":"Lucile Packard Children\u2019s Hospital Stanford and Stanford University School of Medicine Palo Alto CA USA"},{"name":"Children\u2019s Hospital of Philadelphia Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,7]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.028"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000329"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.114.006974"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2016.06.019"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199602013340504"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2009.09.005"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1086\/674304"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2010.06.013"},{"key":"e_1_2_11_11_1","first-page":"A2460","article-title":"Safety and tolerability of transitioning from parenteral treprostinil to oral treprostinil in patients with pulmonary arterial hypertension","volume":"189","author":"White RJ","year":"2014","journal-title":"Am J Respir Crit Care Med"},{"key":"e_1_2_11_12_1","unstructured":"Ivy DD. Pulmonary arterial hypertension assessment in pediatric cardiology. In: Beghetti M Barst RJ Naeije R et al. eds.Pulmonary Arterial Hypertension Related to Congenital Heart Disease. Atlanta GA: Elsevier Urban and Fischer; 2006 pp.93\u2013111."},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1586\/ers.09.16"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00056110"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.109931"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.carj.2011.12.016"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-9236(03)00005-5"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.126.3.808"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.130.5.1471"}],"container-title":["Pulmonary Circulation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894019856471","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1177\/2045894019856471","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1177\/2045894019856471","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,28]],"date-time":"2023-08-28T05:14:09Z","timestamp":1693199649000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1177\/2045894019856471"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7]]},"references-count":18,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2019,7]]}},"alternative-id":["10.1177\/2045894019856471"],"URL":"https:\/\/doi.org\/10.1177\/2045894019856471","archive":["Portico"],"relation":{},"ISSN":["2045-8940","2045-8940"],"issn-type":[{"value":"2045-8940","type":"print"},{"value":"2045-8940","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,7]]}}}