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REVIEW article

Front. Med.
Sec. Hepatobiliary Diseases
Volume 11 - 2024 | doi: 10.3389/fmed.2024.1354857
This article is part of the Research Topic Fontan-Associated Liver Disease: Current status and future directions View all 4 articles

Fontan-associated liver disease: The importance of multidisciplinary teamwork in its management

Provisionally accepted
Tomoya Tsuchihashi Tomoya Tsuchihashi Yuki Cho Yuki Cho DAISUKE TOKUHARA DAISUKE TOKUHARA *
  • Wakayama Medical University, Wakayama, Wakayama, Japan

The final, formatted version of the article will be published soon.

    The Fontan operation, which directly connects the 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.

    Keywords: BRTO, balloon-occluded retrograde transvenous obliteration, CHFS, congestive hepatic fibrosis score, CT, computed tomography, CVP, central venous pressure, EASL, European Association for the Study of the Liver, ERN-RARE-LIVER, European Reference Network on Rare Liver Diseases, FALD, Fontan-associated liver disease, FIB-4, fibrosis-4

    Received: 13 Dec 2023; Accepted: 12 Nov 2024.

    Copyright: © 2024 Tsuchihashi, Cho and TOKUHARA. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: DAISUKE TOKUHARA, Wakayama Medical University, Wakayama, 641-8509, Wakayama, Japan

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