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Updated: Jul 12, 2025
Heterotopic Auxiliary Rat Liver Transplantation With Flow-regulated Portal Vein Arterialization in Acute Hepatic Failure
Published on: September 13, 2014
EASL-ERN position paper on liver involvement in patients with Fontan-type circulation
Luis Téllez1, Audrey Payancé2, Eric Tjwa3
1Gastroenterology and Hepatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain; Instituto Ramón y Cajal de Investigación Sanitaria (IRYCIS), CIBEREHD (Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas), University of Alcalá, Madrid, Spain.
Insights
Fontan-associated liver disease (FALD) affects nearly all patients with Fontan circulation. This paper defines FALD, aids staging, and offers management recommendations for improved Fontan patient outcomes.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Surgery
Background:
- Fontan-type surgery is a critical palliative treatment for univentricular heart defects.
- Long-term hemodynamic changes post-Fontan surgery invariably lead to liver damage, termed Fontan-associated liver disease (FALD).
- The precise impact of FALD on Fontan patient prognosis is unclear due to undefined criteria and competing risks.
Framework:
- This position paper establishes a clear definition and description of FALD.
- It outlines clinical, analytical, radiological, hemodynamic, and histological features of FALD.
- The paper provides guidance for staging liver disease in Fontan patients.
Implementation:
- The paper offers evidence- and experience-based recommendations for managing FALD.
- It addresses various clinical scenarios encountered in Fontan-associated liver disease.
- The goal is to standardize FALD assessment and treatment.
Implications:
- Standardizing FALD definition and staging will clarify its prognostic impact.
- Evidence-based management recommendations aim to improve long-term outcomes for Fontan survivors.
- This paper supports better clinical decision-making for patients with Fontan circulation and liver disease.
Abstract:
Fontan-type surgery is the final step in the sequential palliative surgical treatment of infants born with a univentricular heart. The resulting long-term haemodynamic changes promote liver damage, leading to Fontan-associated liver disease (FALD), in virtually all patients with Fontan circulation. Owing to the lack of a uniform definition of FALD and the competitive risk of other complications developed by Fontan patients, the impact of FALD on the prognosis of these patients is currently debatable. However, based on the increasing number of adult Fontan patients and recent research interest, the European Association for The Study of the Liver and the European Reference Network on Rare Liver Diseases thought a position paper timely. The aims of the current paper are: (1) to provide a clear definition and description of FALD, including clinical, analytical, radiological, haemodynamic, and histological features; (2) to facilitate guidance for staging the liver disease; and (3) to provide evidence- and experience-based recommendations for the management of different clinical scenarios.
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