Fontan-associated liver disease: paediatric experience in a reference centre in Colombia
Andrés David Aranzazu1, Isabel C Sánchez2, Sydney Goldfeder3
1Pediatric Cardiology Fellow, Clínica CardioVID, Universidad Pontificia Bolivariana, Medellín, Colombia.
Insights
Fontan-associated liver disease affects over 11% of pediatric patients with Fontan circulation, presenting diagnostic and treatment challenges. Early identification and management are crucial for these complex cases.
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.
Abstract:
Fontan-associated liver disease is a condition characterised by structural, functional, and clinical alterations secondary to the haemodynamic changes of this circulation.
Objective:
To describe the experience of a series of paediatric patients with Fontan-associated liver dysfunction.
Methods:
A retrospective study including 12 patients with Fontan-associated liver disease. Patients were selected from the single-ventricle program at a high-complexity centre in Colombia between 2001 and 2024.
Results:
During the study period, 108 patients were in the Fontan stage. Among them, 12 met the criteria for Fontan-associated liver disease (11.1%). The median age at extracardiac Fontan completion was 3.9 years, while the median age at Fontan-associated liver disease diagnosis was 14.5 years. Concomitant protein-losing enteropathy and/or plastic bronchitis were present in 33% of cases. Echocardiographic follow-up showed systolic dysfunction in 41% and diastolic dysfunction in 16% of patients. All patients exhibited some degree of valvular insufficiency, mild in 83.3% and moderate in 16%. Haemodynamic variables at the time of diagnosis did not show significant abnormalities.
Conclusions:
Liver disease is a persistent concern in paediatric patients with Fontan physiology, posing diagnostic and therapeutic challenges. Healthcare professionals managing these patients should be aware of its early identification and appropriate treatment.
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