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Fontan-Associated Liver Disease: A Review

Luis Téllez1, Enrique Rodríguez-Santiago1, Agustín Albillos1

  • 1Hospital Universitario Ramón y Cajal. Universidad de Alcalá. Servicio de Gastroenterología y Hepatología.

Annals of Hepatology
|February 23, 2018
PubMed

Insights

Fontan-associated liver disease, a consequence of Fontan surgery, can lead to serious complications. Early diagnosis and management are crucial for preventing conditions like liver cancer and portal hypertension in Fontan survivors.

Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Surgery

Background:

  • Fontan-associated liver disease (FALD) stems from hemodynamic changes post-Fontan surgery.
  • Histological liver changes in FALD resemble but differ from other cardiac liver diseases.
  • The natural history of FALD is not fully understood, but complications are common.

Purpose of the Study:

  • To review current knowledge on Fontan-associated liver disease pathophysiology.
  • To update on the management strategies for FALD.
  • To highlight new diagnostic methods and treatments for FALD.

Main Methods:

  • Literature review of Fontan-associated liver disease.
  • Analysis of hemodynamic changes and systemic venous congestion.
  • Evaluation of diagnostic and treatment modalities.

Main Results:

  • FALD patients face risks of ascites, variceal hemorrhage, and encephalopathy.
  • Fontan survivors have an elevated risk of hepatocellular carcinoma.
  • Early detection of advanced FALD is vital for preventing complications.

Conclusions:

  • FALD requires vigilant monitoring and timely intervention.
  • Advances in diagnostics and treatment offer improved patient outcomes.
  • Understanding FALD pathophysiology is key to effective management.

Keywords:
Fontan surgery. Liver disease. Hepatocarcinoma. Ascites. Fontan failure.

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