Related Experiment Video

Updated: May 20, 2026

Wireless Telemetry Device Implantation in a Fontan Ovine Model for Continuous and Long-Term Hemodynamic Monitoring
06:29

Wireless Telemetry Device Implantation in a Fontan Ovine Model for Continuous and Long-Term Hemodynamic Monitoring

Published on: May 2, 2025

Long-Term Care in Fontan Circulation: Surveillance and Management of Fontan-Associated Liver Disease and

Chaowapong Jarasvaraparn1, Gary R Schooler2, Iván A González3

  • 1Division of Pediatric Gastroenterology, Hepatology and Nutrition, Indiana University, 705 Riley Hospital Drive, ROC 4210, Indianapolis, IN, 46202, USA. cjarasva@iu.edu.

Current Cardiology Reports
|May 19, 2026
PubMed

Insights

Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE) are common after the Fontan procedure. Early recognition and multidisciplinary management are crucial for improving outcomes in these patients.

Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Gastroenterology

Background:

  • The Fontan procedure creates a unique physiology leading to chronic venous hypertension.
  • This venous hypertension results in multisystem injury, notably affecting the liver and gastrointestinal tract.

Purpose of the Study:

  • To review the clinical spectrum and management of Fontan-associated liver disease (FALD).
  • To examine protein-losing enteropathy (PLE) in the context of Fontan physiology.
  • To understand how chronic venous hypertension contributes to multisystem injury.

Main Methods:

  • Literature review of clinical studies and case reports.
  • Analysis of current understanding of Fontan circulation pathophysiology.
  • Synthesis of management strategies for FALD and PLE.

Main Results:

  • Liver fibrosis is a nearly universal complication, with cirrhosis affecting 43% by 30 years post-Fontan.
  • Standard biomarkers and imaging may underestimate FALD severity due to hepatic congestion.
  • Liver biopsy is the gold standard for staging FALD.
  • PLE results from multifactorial gastrointestinal protein loss.
  • A multidisciplinary approach is essential, especially for combined heart-liver transplantation in advanced cases.

Conclusions:

  • Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE) are inevitable consequences of Fontan physiology.
  • Standard diagnostic tools may be insufficient for accurate FALD staging.
  • Future research needs standardized staging and targeted therapies for lymphatic dysfunction and fibrosis progression.
Abstract

Keywords:
Fontan procedureFontan-associated liver diseaseProtein losing enteropathy

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