{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T05:38:07Z","timestamp":1774330687982,"version":"3.50.1"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,3,3]],"date-time":"2023-03-03T00:00:00Z","timestamp":1677801600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:p>Pulmonary hypertension (PH) with developmental lung disease is a life-threatening disease and accounts for 10%\u201312% of pediatric PH patients. Administration of specific pulmonary vasodilators to pediatric PH patients has brought about improvement of their long-term prognosis. Intravenous epoprostenol therapy is a gold standard therapy for severe idiopathic pulmonary arterial hypertension (IPAH), but there are few reports demonstrating the efficacy of epoprostenol for pediatric PH patients with developmental lung disease, especially when treating with high doses of epoprostenol. Two cases of pediatric PH patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD\/MPV) and congenital diaphragmatic hernia (CDH) with bronchopulmonary dysplasia (BPD), respectively, treated with epoprostenol above 100\u2005ng\/kg\/min are presented. In these two cases, severe PH was improved significantly by an aggressive increase of the epoprostenol infusion rate with administration of oral pulmonary vasodilators and appropriate respiratory management, without any significant adverse effects. High-dose epoprostenol therapy may be one of the therapeutic options in pediatric PH patients with developmental lung disease.<\/jats:p>","DOI":"10.3389\/fped.2023.1116434","type":"journal-article","created":{"date-parts":[[2023,3,3]],"date-time":"2023-03-03T05:38:13Z","timestamp":1677821893000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Case report: High-dose epoprostenol therapy in pediatric patients with pulmonary hypertension and developmental lung disease"],"prefix":"10.3389","volume":"11","author":[{"given":"Yoshie","family":"Fukasawa","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hidenori","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miharu","family":"Ito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akiko","family":"Saito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyotaka","family":"Go","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshihito","family":"Morimoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazushi","family":"Yasuda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiaki","family":"Sato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masahiro","family":"Hayakawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Taichi","family":"Kato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,3,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1801916","DOI":"10.1183\/13993003.01916-2018","article-title":"Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management","volume":"53","author":"Rosenzweig","year":"2019","journal-title":"Eur Respir J"},{"key":"B2","doi-asserted-by":"publisher","first-page":"2003337","DOI":"10.1183\/13993003.03337-2020","article-title":"Characterisation of paediatric pulmonary hypertensive vascular disease from the PPHNet registry","volume":"59","author":"Abman","year":"2022","journal-title":"Eur Respir J"},{"key":"B3","doi-asserted-by":"publisher","first-page":"1785","DOI":"10.1253\/circj.71.1785","article-title":"Efficacy and limitations of continuous intravenous epoprostenol therapy for idiopathic pulmonary arterial hypertension in Japanese children","volume":"71","author":"Nakayama","year":"2007","journal-title":"Circ J"},{"key":"B4","doi-asserted-by":"publisher","first-page":"739","DOI":"10.1136\/hrt.2006.096412","article-title":"Epoprostenol treatment in children with severe pulmonary hypertension","volume":"93","author":"Lammers","year":"2007","journal-title":"Heart"},{"key":"B5","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1016\/j.healun.2019.06.022","article-title":"2019 Updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: the European pediatric pulmonary vascular disease network (EPPVDN), endorsed by AEPC, ESPR and ISHLT","volume":"38","author":"Hansmann","year":"2019","journal-title":"J Heart Lung Transplant"},{"key":"B6","doi-asserted-by":"publisher","first-page":"2200","DOI":"10.1253\/circj.cj-10-0190","article-title":"Marked hemodynamic improvements by high-dose epoprostenol therapy in patients with idiopathic pulmonary arterial hypertension","volume":"74","author":"Akagi","year":"2010","journal-title":"Circ J"},{"key":"B7","doi-asserted-by":"publisher","first-page":"542","DOI":"10.1016\/j.jjcc.2015.11.012","article-title":"Rapid and high-dose titration of epoprostenol improves pulmonary hemodynamics and clinical outcomes in patients with idiopathic and heritable pulmonary arterial hypertension","volume":"68","author":"Tokunaga","year":"2016","journal-title":"J Cardiol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"37","author":"Gali\u00e8","year":"2016","journal-title":"Eur Heart J"},{"key":"B9","doi-asserted-by":"publisher","first-page":"923","DOI":"10.1111\/j.1442-200X.2012.03712.x","article-title":"Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: effect of sildenafil, beraprost and bosentan","volume":"54","author":"Kodama","year":"2012","journal-title":"Pediatr Int"},{"key":"B10","doi-asserted-by":"publisher","first-page":"296","DOI":"10.1056\/NEJM199602013340504","article-title":"A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension","volume":"334","author":"Barst","year":"1996","journal-title":"N Engl J Med"},{"key":"B11","doi-asserted-by":"publisher","first-page":"2037","DOI":"10.1161\/CIR.0000000000000329","article-title":"Pediatric pulmonary hypertension: guidelines from the American heart association and American thoracic society","volume":"132","author":"Abman","year":"2015","journal-title":"Circulation"},{"key":"B12","doi-asserted-by":"publisher","first-page":"1197","DOI":"10.1161\/01.cir.99.9.1197","article-title":"Vasodilator therapy for primary pulmonary hypertension in children","volume":"99","author":"Barst","year":"1999","journal-title":"Circulation"},{"key":"B13","doi-asserted-by":"publisher","first-page":"660","DOI":"10.1161\/01.CIR.0000138104.83366.E9","article-title":"Outcomes in children with idiopathic pulmonary arterial hypertension","volume":"110","author":"Yung","year":"2004","journal-title":"Circulation"},{"key":"B14","doi-asserted-by":"publisher","first-page":"92","DOI":"10.1164\/rccm.201011-1874OC","article-title":"BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis","volume":"184","author":"King","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"B15","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1183\/09031936.00011308","article-title":"A randomised, controlled trial of bosentan in severe COPD","volume":"32","author":"Stolz","year":"2008","journal-title":"Eur Respir J"},{"key":"B16","doi-asserted-by":"publisher","first-page":"620","DOI":"10.1056\/NEJMoa1002110","article-title":"A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis","volume":"363","author":"Zisman","year":"2010","journal-title":"N Engl J Med"},{"key":"B17","doi-asserted-by":"publisher","first-page":"2551","DOI":"10.1016\/j.jacc.2017.03.575","article-title":"Pulmonary hypertension in infants, children, and young adults","volume":"69","author":"Hansmann","year":"2017","journal-title":"J Am Coll Cardiol"},{"key":"B18","doi-asserted-by":"publisher","first-page":"ii49","DOI":"10.1136\/heartjnl-2015-308591","article-title":"Pulmonary hypertension associated with acute or chronic lung diseases in the preterm and term neonate and infant. The European paediatric pulmonary vascular disease network, endorsed by ISHLT and DGPK","volume":"102","author":"Hilgendorff","year":"2016","journal-title":"Heart"},{"key":"B19","doi-asserted-by":"publisher","first-page":"2045894018795143","DOI":"10.1177\/2045894018795143","article-title":"Alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical, histological, and genetic aspects","volume":"8","author":"Slot","year":"2018","journal-title":"Pulm Circ"},{"key":"B20","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1016\/s0022-3476(97)70203-8","article-title":"Inhaled nitric oxide enhances oxygenation but not survival in infants with alveolar capillary dysplasia","volume":"130","author":"Steinhorn","year":"1997","journal-title":"J Pediatr"},{"key":"B21","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1016\/j.jpeds.2019.01.047","article-title":"Histopathologic and genetic features of alveolar capillary dysplasia with atypical late presentation and prolonged survival","volume":"210","author":"Edwards","year":"2019","journal-title":"J Pediatr"},{"key":"B22","doi-asserted-by":"publisher","first-page":"158","DOI":"10.1016\/j.jpeds.2017.10.026","article-title":"Infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins who underwent bilateral lung transplantation","volume":"194","author":"Towe","year":"2018","journal-title":"J Pediatr"},{"key":"B23","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1136\/archdischild-2019-318531","article-title":"Fate of pulmonary hypertension associated with bronchopulmonary dysplasia beyond 36 weeks postmenstrual age","volume":"106","author":"Arjaans","year":"2021","journal-title":"Arch Dis Child Fetal Neonatal Ed"},{"key":"B24","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1016\/j.jpeds.2012.12.036","article-title":"Outcomes of congenital diaphragmatic hernia in the modern era of management","volume":"163","author":"Wynn","year":"2013","journal-title":"J Pediatr"},{"key":"B25","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1002\/ppul.23508","article-title":"Pulmonary hypertension specific treatment in infants with bronchopulmonary dysplasia","volume":"52","author":"Kadmon","year":"2017","journal-title":"Pediatr Pulmonol"},{"key":"B26","doi-asserted-by":"publisher","first-page":"97","DOI":"10.3390\/children7080097","article-title":"Targeted therapy for pulmonary hypertension in premature infants","volume":"7","author":"Nees","year":"2020","journal-title":"Children (Basel)"},{"key":"B27","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1002\/ppul.20238","article-title":"Epoprostenol and home mechanical ventilation for pulmonary hypertension associated with chronic lung disease","volume":"40","author":"Zaidi","year":"2005","journal-title":"Pediatr Pulmonol"},{"key":"B28","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1086\/674762","article-title":"Safety of epoprostenol and treprostinil in children less than 12 months of age","volume":"3","author":"McIntyre","year":"2013","journal-title":"Pulm Circ"},{"key":"B29","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1016\/j.lfs.2014.01.077","article-title":"Long-term patient survival with idiopathic\/heritable pulmonary arterial hypertension treated at a single center in Japan","volume":"118","author":"Ogawa","year":"2014","journal-title":"Life Sci"},{"key":"B30","doi-asserted-by":"publisher","first-page":"525","DOI":"10.1046\/j.1442-200X.2002.01594.x","article-title":"Intravenous prostacyclin combined with inhaled nitric oxide therapy for an infant with alveolar capillary dysplasia","volume":"44","author":"Kinugasa","year":"2002","journal-title":"Pediatr Int"},{"key":"B31","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/s1084-2756(02)00191-4","article-title":"The pulmonary circulation in bronchopulmonary dysplasia","volume":"8","author":"Parker","year":"2003","journal-title":"Semin Neonatol"},{"key":"B32","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1089\/ped.2018.0984","article-title":"Pulmonary hypertension associated with bronchopulmonary dysplasia: a review","volume":"32","author":"Varghese","year":"2019","journal-title":"Pediatr Allergy Immunol Pulmonol"}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2023.1116434\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,3,3]],"date-time":"2023-03-03T05:38:17Z","timestamp":1677821897000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2023.1116434\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3,3]]},"references-count":32,"alternative-id":["10.3389\/fped.2023.1116434"],"URL":"https:\/\/doi.org\/10.3389\/fped.2023.1116434","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,3,3]]},"article-number":"1116434"}}