{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T06:44:38Z","timestamp":1790750678021,"version":"4.1.0"},"reference-count":112,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,9,30]],"date-time":"2025-09-30T00:00:00Z","timestamp":1759190400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,9,30]],"date-time":"2025-09-30T00:00:00Z","timestamp":1759190400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>Pulmonary hypertension (PH) is characterised by elevated pressures in the pulmonary arterial system. Despite its high mortality, paediatric PH has few approved treatments. Our aim was to review the latest diagnostic and treatment considerations for paediatric PH, with particular emphasis on WHO group 3 chronic lung disease\/hypoxia\u2010related pulmonary hypertension (CLD\u2010PH).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Two subspecialists caring for children with CLD\u2010PH conducted a narrative review of the latest expert guidelines and published pharmacotherapeutic data relevant to CLD\u2010PH.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Bronchopulmonary dysplasia (BPD) is the leading cause of CLD\u2010PH; up to 30% of infants with BPD develop PH. Extreme prematurity, mechanical ventilation, infection and genetic predisposition contribute to vascular growth arrest, remodelling and increased pulmonary vascular resistance. Other conditions with lung hypoplasia and CLD\u2010PH include congenital diaphragmatic hernia (CDH) and Down syndrome. Children with PH should be monitored by a specialised team experienced in cardiac monitoring, lung function\/exercise testing, PH pharmacotherapy and the typical CLD\u2010PH comorbidities. The most common therapy options include supplemental oxygen, phosphodiesterase inhibitors, endothelin receptor antagonists and prostacyclin agonists.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>CLD\u2010PH in children is a complex condition that requires a thorough understanding of its aetiology, assessment methods, management strategies and supportive, family\u2010centred care to improve outcomes for children with CLD\u2010PH.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/apa.70320","type":"journal-article","created":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T06:30:32Z","timestamp":1759300232000},"page":"16-31","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Chronic Lung Disease\u2010Related Pulmonary Hypertension in Children"],"prefix":"10.1111","volume":"115","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9115-5312","authenticated-orcid":false,"given":"Jason E.","family":"Lang","sequence":"first","affiliation":[{"name":"Duke Clinical Research Institute  Durham North Carolina USA"},{"name":"Division of Pulmonary and Sleep Medicine, Department of Pediatrics Duke University School of Medicine  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4451-3580","authenticated-orcid":false,"given":"Kevin D.","family":"Hill","sequence":"additional","affiliation":[{"name":"Duke Clinical Research Institute  Durham North Carolina USA"},{"name":"Division of Pediatric Cardiology, Department of Pediatrics Duke University Pediatric and Congenital Heart Center, Duke University School of Medicine  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,9,30]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2020.06.005"},{"key":"e_1_2_13_3_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.03337-2020"},{"key":"e_1_2_13_4_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202110-2428OC"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01222-2024"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.4103\/2045-8932.83445"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01916-2018"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.3928\/19382359-20190325-03"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-024-02850-x"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2009-2959"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201812-2348OC"},{"issue":"17","key":"e_1_2_13_12_1","first-page":"1","article-title":"Births: Final Data for 2020","volume":"70","author":"Osterman M.","year":"2021","journal-title":"National Vital Statistics Reports"},{"key":"e_1_2_13_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2015.10244"},{"key":"e_1_2_13_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-019-0127-7"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/fn.83.1.F1"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2017-314264"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jp.7211369"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.105.6.1216"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000884"},{"key":"e_1_2_13_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2016.10.082"},{"key":"e_1_2_13_21_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12955-022-02018-5"},{"key":"e_1_2_13_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2024-327547"},{"key":"e_1_2_13_23_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.jp.7211673"},{"key":"e_1_2_13_24_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2007-0971"},{"key":"e_1_2_13_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.07.035"},{"key":"e_1_2_13_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2017.05.029"},{"key":"e_1_2_13_27_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.164.10.2101140"},{"key":"e_1_2_13_28_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201305-124OC"},{"key":"e_1_2_13_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clp.2015.08.010"},{"key":"e_1_2_13_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41390-020-0993-4"},{"key":"e_1_2_13_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/jpc.14878"},{"key":"e_1_2_13_32_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200701-051OC"},{"key":"e_1_2_13_33_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa072788"},{"key":"e_1_2_13_34_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.163.7.2011060"},{"key":"e_1_2_13_35_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202003-0601OC"},{"key":"e_1_2_13_36_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201910-1975OC"},{"key":"e_1_2_13_37_1","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-198510000-00023"},{"key":"e_1_2_13_38_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.16863"},{"key":"e_1_2_13_39_1","doi-asserted-by":"publisher","DOI":"10.1159\/000327891"},{"key":"e_1_2_13_40_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41372-019-0549-9"},{"key":"e_1_2_13_41_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2021.695610"},{"key":"e_1_2_13_42_1","doi-asserted-by":"publisher","DOI":"10.1038\/jp.2016.255"},{"key":"e_1_2_13_43_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045894018760166"},{"key":"e_1_2_13_44_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2020-216485"},{"key":"e_1_2_13_45_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41390-024-03268-3"},{"key":"e_1_2_13_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semperi.2023.151811"},{"key":"e_1_2_13_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semperi.2023.151817"},{"key":"e_1_2_13_48_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2020-030007"},{"key":"e_1_2_13_49_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202203-0570OC"},{"key":"e_1_2_13_50_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201702-178OC"},{"key":"e_1_2_13_51_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41372-024-01881-y"},{"key":"e_1_2_13_52_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00229.2015"},{"key":"e_1_2_13_53_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-025-06099-4"},{"issue":"1","key":"e_1_2_13_54_1","first-page":"4","article-title":"Pulmonary Arterial Hypertension in Children: Diagnostic Work\u2010Up and Challenges","volume":"27","author":"Rosenzweig E. B.","year":"2009","journal-title":"Progress in Pediatric Cardiology"},{"key":"e_1_2_13_55_1","doi-asserted-by":"publisher","DOI":"10.1111\/apa.70019"},{"key":"e_1_2_13_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clp.2023.10.001"},{"key":"e_1_2_13_57_1","doi-asserted-by":"publisher","DOI":"10.1111\/jir.12371"},{"key":"e_1_2_13_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.06.031"},{"key":"e_1_2_13_59_1","doi-asserted-by":"publisher","DOI":"10.4250\/jcu.2015.23.2.72"},{"key":"e_1_2_13_60_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-010-1200-0"},{"key":"e_1_2_13_61_1","doi-asserted-by":"publisher","DOI":"10.5665\/sleep.5554"},{"key":"e_1_2_13_62_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00455-016-9725-7"},{"key":"e_1_2_13_63_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10332"},{"key":"e_1_2_13_64_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1754.1992.tb02697.x"},{"key":"e_1_2_13_65_1","doi-asserted-by":"publisher","DOI":"10.1161\/HHF.0000000000000080"},{"key":"e_1_2_13_66_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2022-057010"},{"key":"e_1_2_13_67_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022"},{"key":"e_1_2_13_68_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000329"},{"key":"e_1_2_13_69_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2019.06.022"},{"key":"e_1_2_13_70_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2014-307340"},{"key":"e_1_2_13_71_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2015-308246"},{"key":"e_1_2_13_72_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2008.03.009"},{"key":"e_1_2_13_73_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2020.01.001"},{"key":"e_1_2_13_74_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-017-1575-z"},{"key":"e_1_2_13_75_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2011.06.014"},{"key":"e_1_2_13_76_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201907-1342OC"},{"key":"e_1_2_13_77_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2015-308591"},{"key":"e_1_2_13_78_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.2040-4603.2021.tb00176.x"},{"key":"e_1_2_13_79_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1413687"},{"key":"e_1_2_13_80_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2014.02.575"},{"key":"e_1_2_13_81_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.110.016667"},{"key":"e_1_2_13_82_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.113.005698"},{"key":"e_1_2_13_83_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2018.09.067"},{"key":"e_1_2_13_84_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-012-0180-4"},{"issue":"1","key":"e_1_2_13_85_1","first-page":"7","article-title":"Oral Tadalafil in Children With Pulmonary Arterial Hypertension","volume":"66","author":"Shiva A.","year":"2016","journal-title":"Drug Research (Stuttg)"},{"key":"e_1_2_13_86_1","doi-asserted-by":"publisher","DOI":"10.1080\/03007995.2017.1354832"},{"key":"e_1_2_13_87_1","unstructured":"M. R.Southworth \u201cSupplement Approval NDA 022081\/S\u2010045 (Ambrisentan) Research OoND\u2010CfDEa \u201d(2025) https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2025\/022081s045lbl.pdf."},{"key":"e_1_2_13_88_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0009-9236(03)00005-5"},{"key":"e_1_2_13_89_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2125.2009.03532.x"},{"key":"e_1_2_13_90_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40272-013-0052-2"},{"key":"e_1_2_13_91_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22555"},{"key":"e_1_2_13_92_1","unstructured":"Actelion Pharmaceuticals US I \u201cOPSUMIT (Macitentan) Tablets for Oral Use \u201d(2025)."},{"key":"e_1_2_13_93_1","unstructured":"Actavis Pharma I \u201cAmbrisentan Tablets \u201d(2025)."},{"issue":"5","key":"e_1_2_13_94_1","article-title":"Prostacyclin for Pulmonary Arterial Hypertension","volume":"5","author":"Barnes H.","year":"2019","journal-title":"Cochrane Database of Systematic Reviews"},{"key":"e_1_2_13_95_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24442"},{"issue":"1","key":"e_1_2_13_96_1","first-page":"37","article-title":"Prostacyclins and Pulmonary Arterial Hypertension in Children","volume":"26","author":"Wu Y.","year":"2022","journal-title":"European Review for Medical and Pharmacological Sciences"},{"key":"e_1_2_13_97_1","doi-asserted-by":"publisher","DOI":"10.1177\/2045894019862138"},{"key":"e_1_2_13_98_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.healun.2020.03.029"},{"key":"e_1_2_13_99_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-017-1677-7"},{"key":"e_1_2_13_100_1","article-title":"Sotatercept in Pulmonary Arterial Hypertension","author":"Cracowski J. L.","year":"2025","journal-title":"Trends in Pharmacological Sciences"},{"key":"e_1_2_13_101_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2024277"},{"key":"e_1_2_13_102_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2213558"},{"key":"e_1_2_13_103_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2415160"},{"key":"e_1_2_13_104_1","doi-asserted-by":"publisher","DOI":"10.1159\/000444210"},{"key":"e_1_2_13_105_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2021.808317"},{"key":"e_1_2_13_106_1","doi-asserted-by":"publisher","DOI":"10.1136\/wjps-2024-000789"},{"key":"e_1_2_13_107_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.99.6.838"},{"key":"e_1_2_13_108_1","doi-asserted-by":"publisher","DOI":"10.4137\/CMC.S10237"},{"key":"e_1_2_13_109_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2009.01.021"},{"key":"e_1_2_13_110_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2011.07.009"},{"key":"e_1_2_13_111_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12872-017-0569-3"},{"key":"e_1_2_13_112_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01345-2024"},{"key":"e_1_2_13_113_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2015-307792"}],"container-title":["Acta Paediatrica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.70320","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/apa.70320","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/apa.70320","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T14:24:57Z","timestamp":1768919097000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/apa.70320"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9,30]]},"references-count":112,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1111\/apa.70320"],"URL":"https:\/\/doi.org\/10.1111\/apa.70320","archive":["Portico"],"relation":{},"ISSN":["0803-5253","1651-2227"],"issn-type":[{"value":"0803-5253","type":"print"},{"value":"1651-2227","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,9,30]]},"assertion":[{"value":"2025-06-03","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-09-23","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-09-30","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}