Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management
The Fontan operation, which directly connects the superior and inferior vena cava to the pulmonary artery, is a palliative surgery for children with a functional or anatomic single ventricle. This procedure leads to hemodynamic changes (Fontan circulation) in patients, who tend to develop congestive...
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Frontiers Media S.A.
2024-11-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2024.1354857/full |
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| author | Tomoya Tsuchihashi Yuki Cho Daisuke Tokuhara Daisuke Tokuhara |
| author_facet | Tomoya Tsuchihashi Yuki Cho Daisuke Tokuhara Daisuke Tokuhara |
| author_sort | Tomoya Tsuchihashi |
| collection | DOAJ |
| description | The Fontan operation, which directly connects the superior and inferior vena cava to the pulmonary artery, is a palliative surgery for children with a functional or anatomic single ventricle. This procedure leads to hemodynamic changes (Fontan circulation) in patients, who tend to develop congestive hepatic fibrosis characterized by sinusoidal fibrosis and dilatation beginning approximately 10 years after the procedure. In addition, in the context of severe fibrosis and cirrhosis, hepato-gastrointestinal complications including hepatocellular carcinoma, focal nodular hyperplasia, and portal hypertension can arise. Fontan-associated liver disease (FALD) encompasses the broad spectrum of liver alterations secondary to postoperative hemodynamic changes, and the effective management of FALD requires contributions from specialists in hepatology, gastroenterology, surgery, radiology, histopathology, and pediatric and adult cardiology. In this article, we outline the pathogenesis of FALD and discuss the importance of a multidisciplinary collaborative approach to its management. |
| format | Article |
| id | doaj-art-b10e1f82d8ac410e861a3e09fc5f1c4c |
| institution | OA Journals |
| issn | 2296-858X |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj-art-b10e1f82d8ac410e861a3e09fc5f1c4c2025-08-20T01:52:45ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2024-11-011110.3389/fmed.2024.13548571354857Fontan-associated liver disease: the importance of multidisciplinary teamwork in its managementTomoya Tsuchihashi0Yuki Cho1Daisuke Tokuhara2Daisuke Tokuhara3Department of Pediatrics, Wakayama Medical University, Wakayama, JapanDepartment of Pediatrics, Wakayama Medical University, Wakayama, JapanDepartment of Pediatrics, Wakayama Medical University, Wakayama, JapanDepartment of Pediatrics, Osaka Metropolitan University, Osaka, JapanThe Fontan operation, which directly connects the superior and inferior vena cava to the pulmonary artery, is a palliative surgery for children with a functional or anatomic single ventricle. This procedure leads to hemodynamic changes (Fontan circulation) in patients, who tend to develop congestive hepatic fibrosis characterized by sinusoidal fibrosis and dilatation beginning approximately 10 years after the procedure. In addition, in the context of severe fibrosis and cirrhosis, hepato-gastrointestinal complications including hepatocellular carcinoma, focal nodular hyperplasia, and portal hypertension can arise. Fontan-associated liver disease (FALD) encompasses the broad spectrum of liver alterations secondary to postoperative hemodynamic changes, and the effective management of FALD requires contributions from specialists in hepatology, gastroenterology, surgery, radiology, histopathology, and pediatric and adult cardiology. In this article, we outline the pathogenesis of FALD and discuss the importance of a multidisciplinary collaborative approach to its management.https://www.frontiersin.org/articles/10.3389/fmed.2024.1354857/fullFontan procedureFontan-associated liver diseaseliver fibrosishepatocellular carcinomagastroesophageal varixprotein-losing enteropathy |
| spellingShingle | Tomoya Tsuchihashi Yuki Cho Daisuke Tokuhara Daisuke Tokuhara Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management Frontiers in Medicine Fontan procedure Fontan-associated liver disease liver fibrosis hepatocellular carcinoma gastroesophageal varix protein-losing enteropathy |
| title | Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management |
| title_full | Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management |
| title_fullStr | Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management |
| title_full_unstemmed | Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management |
| title_short | Fontan-associated liver disease: the importance of multidisciplinary teamwork in its management |
| title_sort | fontan associated liver disease the importance of multidisciplinary teamwork in its management |
| topic | Fontan procedure Fontan-associated liver disease liver fibrosis hepatocellular carcinoma gastroesophageal varix protein-losing enteropathy |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2024.1354857/full |
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