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Purpose of Review <p>This manuscript reviews the clinical spectrum and management of Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE), examining how chronic venous hypertension leads to multisystem injury.</p> Recent Findings <p>Liver fibrosis is now recognized as an early and nearly universal complication after the Fontan procedure, with cirrhosis affecting approximately 43% of patients by 30&#xa0;years post-operation. Although post-2001 survival exceeds 90%, standard biomarkers and imaging frequently underestimate disease severity, as liver stiffness measurements are confounded by hepatic congestion. Liver biopsy remains the gold standard for staging.</p> Summary <p>FALD is an inevitable consequence of Fontan physiology, characterized by sinusoidal congestion and progressive fibrosis, while PLE results from multifactorial gastrointestinal protein loss. The key clinical implication is the importance of a multidisciplinary approach, particularly when considering transition from isolated heart transplantation to combined heart–liver transplantation in advanced disease. Future research should prioritize standardized staging systems and targeted therapies to reduce lymphatic dysfunction and fibrosis progression.</p>

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

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年5月19日

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  • Chaowapong Jarasvaraparn,
  • Gary R. Schooler,
  • Iván A. González,
  • Salil Ginde,
  • Alisha Mavis

摘要

Purpose of Review

This manuscript reviews the clinical spectrum and management of Fontan-associated liver disease (FALD) and protein-losing enteropathy (PLE), examining how chronic venous hypertension leads to multisystem injury.

Recent Findings

Liver fibrosis is now recognized as an early and nearly universal complication after the Fontan procedure, with cirrhosis affecting approximately 43% of patients by 30 years post-operation. Although post-2001 survival exceeds 90%, standard biomarkers and imaging frequently underestimate disease severity, as liver stiffness measurements are confounded by hepatic congestion. Liver biopsy remains the gold standard for staging.

Summary

FALD is an inevitable consequence of Fontan physiology, characterized by sinusoidal congestion and progressive fibrosis, while PLE results from multifactorial gastrointestinal protein loss. The key clinical implication is the importance of a multidisciplinary approach, particularly when considering transition from isolated heart transplantation to combined heart–liver transplantation in advanced disease. Future research should prioritize standardized staging systems and targeted therapies to reduce lymphatic dysfunction and fibrosis progression.