EASL-ERN position paper on liver involvement in patients with Fontan-type circulation

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.