{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T02:36:46Z","timestamp":1760150206207,"version":"build-2065373602"},"reference-count":8,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T00:00:00Z","timestamp":1697068800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Pulmonary arterial hypertension (PAH) is a rare heterogeneous disorder in the paediatric population which is mostly associated with congenital heart disease. The management of paediatric idiopathic PAH (IPAH) is difficult due to insufficient comparative data and depends on the results of evidence-based adult studies with several pulmonary vasodilators, as well as the clinical experiences of paediatric experts. Our aim was to present the case of a 9-year-old girl who underwent several methods of treatment, including pharmacotherapy with a significant reaction to treprostinil, as well as bilateral lung transplantation. The patient\u2019s treatment was distinguished by the fact that the dose escalation was as rapid as that observed in the adult population. Due to the limited current evidence and knowledge, the initiation of treatment for these patients remains an individual choice. On the grounds of the number of non-specific symptoms, the diagnosis of this patient was a long process and based mainly on the differential diagnosis. The purpose of this paper is to study this example in order to highlight the importance of early symptoms and the quick implementation of intensive treatment. The applied methods may be useful in doubtful diagnosis processes and treatment procedures in the paediatric population.<\/jats:p>","DOI":"10.3390\/diagnostics13203185","type":"journal-article","created":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T03:07:47Z","timestamp":1697080067000},"page":"3185","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Rapidly Progressive Idiopathic Pulmonary Arterial Hypertension in a Paediatric Patient Treated with Lung Transplantation"],"prefix":"10.3390","volume":"13","author":[{"given":"Filip","family":"Baszkowski","sequence":"first","affiliation":[{"name":"Department of Pediatric Cardiology, Poznan University of Medical Sciences, 60-572 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1175-1745","authenticated-orcid":false,"given":"Weronika","family":"Pelczar-P\u0142achta","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Poznan University of Medical Sciences, 60-572 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nikola","family":"Pempera","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Poznan University of Medical Sciences, 60-572 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylwia","family":"S\u0142awek-Szmyt","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, 61-848 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Ka\u0142u\u017cna-Oleksy","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, 61-848 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maciej","family":"Lesiak","sequence":"additional","affiliation":[{"name":"1st Department of Cardiology, Poznan University of Medical Sciences, 61-848 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Waldemar","family":"Bobkowski","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Poznan University of Medical Sciences, 60-572 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"2022 ESC\/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension: Developed by the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by the International Society for Heart and Lung Transplantation (ISHLT) and the European Reference Network on Rare Respiratory Diseases (ERN-LUNG)","volume":"43","author":"Humbert","year":"2022","journal-title":"Eur. Heart J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"S89","DOI":"10.1097\/PCC.0000000000000622","article-title":"Pediatric Cardiac Intensive Care Society 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care Pulmonary Hypertension","volume":"17","author":"Kim","year":"2016","journal-title":"Pediatr. Crit. Care Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1016\/j.chest.2019.09.009","article-title":"Risk Reduction and Right Heart Reverse Remodeling by Upfront Triple Combination Therapy in Pulmonary Arterial Hypertension","volume":"157","author":"Badagliacca","year":"2020","journal-title":"Chest"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.1183\/09031936.00116313","article-title":"Upfront triple combination therapy in pulmonary arterial hypertension: A pilot study","volume":"43","author":"Sitbon","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_5","doi-asserted-by":"crossref","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":"ref_6","doi-asserted-by":"crossref","first-page":"2001120","DOI":"10.1183\/13993003.01120-2020","article-title":"Upfront triple combination therapy in severe paediatric pulmonary arterial hypertension","volume":"57","author":"Haarman","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1097\/TP.0000000000000348","article-title":"Lung transplantation for severe pulmonary hypertension\u2014Awake extracorporeal membrane oxygenation for postoperative left ventricular remodelling","volume":"99","author":"Tudorache","year":"2015","journal-title":"Transplantation"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1093\/ehjci\/jev014","article-title":"Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging","volume":"16","author":"Lang","year":"2015","journal-title":"Eur. Heart J.\u2014Cardiovasc. Imaging"}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/20\/3185\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:05:21Z","timestamp":1760130321000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/20\/3185"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,12]]},"references-count":8,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2023,10]]}},"alternative-id":["diagnostics13203185"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics13203185","relation":{},"ISSN":["2075-4418"],"issn-type":[{"type":"electronic","value":"2075-4418"}],"subject":[],"published":{"date-parts":[[2023,10,12]]}}}