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

Published online by Cambridge University Press:  03 July 2025

Andrés David Aranzazu Open the ORCID record for Andrés David Aranzazu [Opens in a new window] ,
Isabel C. Sánchez Open the ORCID record for Isabel C. Sánchez [Opens in a new window] ,
Sydney Goldfeder Open the ORCID record for Sydney Goldfeder [Opens in a new window]  and
Mónica Isabel Guzmán Open the ORCID record for Mónica Isabel Guzmán [Opens in a new window]
Andrés David Aranzazu*
Affiliation:
Pediatric Cardiology Fellow, Clínica CardioVID, Universidad Pontificia Bolivariana, Medellín, Colombia
Isabel C. Sánchez
Affiliation:
Pediatric Cardiologist, Clínica CardioVID, Medellín, Colombia
Sydney Goldfeder
Affiliation:
Physician Epidemiologist, Clínica CardioVID, Medellín, Colombia
Mónica Isabel Guzmán
Affiliation:
Pediatric Cardiologist, Leader of the Single Ventricle Program, Clínica CardioVID, Medellín, Colombia
*
Corresponding author: Andrés David Aranzazu; Email: aranzazuandres7@gmail.com
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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.

Keywords

Fontan techniqueuniventricular heartliver failureCHD

Information

Type
Original Article
Information
Cardiology in the Young , First View , pp. 1 - 5
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

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