Fontan-associated liver disease: paediatric experience in a reference centre in Colombia

Area of Science:

• Pediatric Cardiology
• Hepatology
• Congenital Heart Disease

Background:

• Fontan-associated liver disease (FALD) arises from altered hemodynamics in patients with single-ventricle physiology.
• It involves structural, functional, and clinical liver changes.
• Understanding FALD is critical for long-term patient outcomes.

Purpose of the Study:

• To detail the clinical experience with Fontan-associated liver dysfunction in pediatric patients.
• To highlight the prevalence and characteristics of FALD in a Colombian cohort.

Main Methods:

• Retrospective analysis of 12 pediatric patients diagnosed with Fontan-associated liver disease.
• Data collected from a single-ventricle program between 2001 and 2024.
• Inclusion criteria based on established FALD diagnostic standards.

Main Results:

• 11.1% (12/108) of patients in the Fontan stage developed FALD.
• Median age at diagnosis was 14.5 years (range not specified), with Fontan completion at median 3.9 years.
• 33% had protein-losing enteropathy/plastic bronchitis; 41% showed systolic dysfunction; 16% diastolic dysfunction; 100% had some valvular insufficiency.

Conclusions:

• Fontan-associated liver disease remains a significant clinical issue in pediatric patients with Fontan physiology.
• Diagnostic and therapeutic approaches for FALD require ongoing attention.
• Healthcare providers must prioritize early FALD detection and management.