{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T00:52:34Z","timestamp":1760057554110,"version":"build-2065373602"},"reference-count":41,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2025,2,9]],"date-time":"2025-02-09T00:00:00Z","timestamp":1739059200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: The Fontan procedure has provided pediatric patients suffering from severe congenital heart disease the opportunity to reach adulthood. Increasingly, we encounter the liver repercussions of Fontan circulation, alongside a decline in heart function and exercise performance. This study aims to identify the univentricular heart malformations that are most susceptible to liver dysfunction; assess which markers of liver injury are essential for multidisciplinary clinical follow-up of Fontan patients; determine the optimal approach for evaluating liver function in Fontan patients; and explore how a congenital cardiology team can interpret the data and respond effectively to signs of organ failure. Methods: Cross-sectional clinical study including patients who underwent a Fontan procedure at the University Hospital of Padua between 1982 and 2017. Patients were admitted for elective hospitalization between June 2021 and June 2022 and underwent clinical assessment, laboratory tests, and instrumental examinations. Results: Seventy patients were included in the study. On admission, 48 patients (72%) were in New York Heart Association (NYHA) functional class I, and the cardiopulmonary exercise test was normal for age and gender. At laboratory tests, 56% of patients showed changes in NTproBNP values, most of whom had right-sided ventricular morphology. Liver function tests showed abnormal Gamma-Glutamyl Transferase (GGT) blood levels in 68%. On cardiac imaging, at least moderate atrioventricular valve insufficiency was found in 9% of cases. Fibroscan showed altered hepatic stiffness values in 25% of cases. Statistical analysis showed that systemic atrioventricular valve (SAVV) dysfunction was significantly associated with a reduction of maximum oxygen consumption (VO2 max) and hepatic stiffness. Conclusions: SAVV dysfunction is significantly responsible for worse functional outcomes and the development of hepatic fibrosis due to an increase in venous congestion. Setting up a careful multidisciplinary follow-up in these patients is mandatory for early detection of complications, prompt treatment, and better outcomes.<\/jats:p>","DOI":"10.3390\/jcm14041114","type":"journal-article","created":{"date-parts":[[2025,2,10]],"date-time":"2025-02-10T03:39:47Z","timestamp":1739158787000},"page":"1114","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Heart\u2013Liver Interplay in Patients with Fontan Circulation"],"prefix":"10.3390","volume":"14","author":[{"given":"Roberta","family":"Biffanti","sequence":"first","affiliation":[{"name":"Paediatric Cardiology Unit, Department of Woman\u2019s and Child\u2019s Health, University of Padua Medical School, 35121 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1661-5419","authenticated-orcid":false,"given":"Jolanda","family":"Sabatino","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Unit, Department of Woman\u2019s and Child\u2019s Health, University of Padua Medical School, 35121 Padova, Italy"},{"name":"Paediatric Cardiology Unit, Magna Graecia University, 88100 Catanzaro, 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":"Paediatric Cardiology Unit, Department of Woman\u2019s and Child\u2019s Health, University of Padua Medical School, 35121 Padova, Italy"},{"name":"PhD School in Developmental Medicine and Health Planning Sciences, University of Padua, 35122 Padua, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6584-4157","authenticated-orcid":false,"given":"Liliana","family":"Chemello","sequence":"additional","affiliation":[{"name":"Internal Medicine & Hepatology Unit, Department of Medicine, University of Padua Medical School, 35121 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7368-279X","authenticated-orcid":false,"given":"Luisa","family":"Cavalletto","sequence":"additional","affiliation":[{"name":"Internal Medicine & Hepatology Unit, Department of Medicine, University of Padua Medical School, 35121 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrea","family":"Gasperetti","sequence":"additional","affiliation":[{"name":"Sport and Exercise Medicine Unit, Department of Medicine, University of Padua Medical School, 35121 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7535-9670","authenticated-orcid":false,"given":"Massimo","family":"Padalino","sequence":"additional","affiliation":[{"name":"Paediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic & Vascular Sciences and Public Health, University of Padua Medical School, 35121 Padova, Italy"},{"name":"Section of Cardiac Surgery, Department of Precision and Regenerative Medicine, University of Bari \u201cAldo Moro\u201d, 70121 Bari, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0305-0113","authenticated-orcid":false,"given":"Giovanni","family":"Di Salvo","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Unit, Department of Woman\u2019s and Child\u2019s Health, University of Padua Medical School, 35121 Padova, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,2,9]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1161\/CIRCULATIONAHA.105.592378","article-title":"Univentricular heart","volume":"115","author":"Khairy","year":"2007","journal-title":"Circulation"},{"key":"ref_2","first-page":"1111","article-title":"Frequency and spectrum of congenital heart defects among live births in Germany: A study of the Competence Network for Congenital Heart Defects","volume":"100","author":"Schwedler","year":"2011","journal-title":"Clin. 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