{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T21:41:45Z","timestamp":1778190105550,"version":"3.51.4"},"reference-count":21,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2025,6,10]],"date-time":"2025-06-10T00:00:00Z","timestamp":1749513600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background: The aim of this study was to describe our centre experience in the use of pulmonary vasodilator therapy in Fontan patients. Methods: We retrospectively enrolled patients that underwent Fontan operation between 2000 and 2024, reporting demographic and operative data and noting complications and the use of pulmonary vasodilators. Results: A total of 117 patients were followed for a median time of 150 months (90\u2013207). In total, 36.7% were female, and the median age during the intervention was 50 months (37\u201364), and 53% had a single left ventricle physiology. In 20 of these 117 patients (17.1%), at least one pulmonary vasodilator drug was used during their life for the following reasons: 6 elevated pressures in the circuit, 3 low oxygen saturation, 2 plastic bronchitis, 2 pleural effusion, 1 chylothorax, 1 persistent pericardial effusion, 1 haemoptysis, 1 protein losing enteropathy, 1 poor exercise tolerance, 1 pulmonary arterial hypertension present since birth and 1 diastolic dysfunction. They had a significantly higher prevalence of single right ventricle physiology (65% vs. 37%, p = 0.03), pulmonary hypertension (60% vs. 0, p = 0.0001), plastic bronchitis (10% vs. 0, p = 0.03) and declivous oedema in the follow-up period (10% vs. 0, p = 0.03), with a higher assumption of warfarin (35% vs. 6.2%, p = 0.001). Conclusions: We found that in the absence of a standardise protocol, we usually use pulmonary vasodilator therapy in Fontan patients, as it is guided by clinical aspects and hemodynamic conditions, which lead us to start and stop this therapy.<\/jats:p>","DOI":"10.3390\/children12060751","type":"journal-article","created":{"date-parts":[[2025,6,10]],"date-time":"2025-06-10T05:11:49Z","timestamp":1749532309000},"page":"751","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["The Use of Vasodilator Therapy in Fontan Patients: A Single-Centre Experience"],"prefix":"10.3390","volume":"12","author":[{"given":"Alessia","family":"Faccini","sequence":"first","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4970-4562","authenticated-orcid":false,"given":"Martina","family":"Avesani","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberta","family":"Biffanti","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1104-9398","authenticated-orcid":false,"given":"Elettra","family":"Pomiato","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6444-2292","authenticated-orcid":false,"given":"Domenico","family":"Sirico","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-4799-5551","authenticated-orcid":false,"given":"Alice","family":"Pozza","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessia","family":"Cerutti","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Reffo","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8140-6798","authenticated-orcid":false,"given":"Biagio","family":"Castaldi","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Di Salvo","sequence":"additional","affiliation":[{"name":"Division of Paediatric Cardiology, Department of Women\u2019s and Children\u2019s Health, University Hospital Padua, 35128 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,6,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1136\/thx.26.3.240","article-title":"Surgical repair of tricuspid atresia","volume":"26","author":"Fontan","year":"1971","journal-title":"Thorax"},{"key":"ref_2","unstructured":"Avesani, M., Thambo, J., Roubertie, F., and Iriart, X. 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Ther."},{"key":"ref_9","doi-asserted-by":"crossref","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":"ref_10","doi-asserted-by":"crossref","first-page":"e00080","DOI":"10.1161\/HHF.0000000000000080","article-title":"Pulmonary Hypertension in Congenital Heart Disease: A Scientific Statement from the American Heart Association","volume":"16","author":"Jone","year":"2023","journal-title":"Circ. 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