The Impact of Fontan Type and Timing on Fontan Associated Liver Disease

Document Type

Conference Proceeding

Publication Date

4-2026

Publication Title

Journal of the American College of Cardiology

Abstract

Background: Fontan associated liver disease (FALD) is a nearly universal condition in patients with single-ventricle palliation. We aimed to evaluate whether Fontan surgical technique, age at Fontan completion, presence of fenestration, and single ventricle morphology influence FALD onset. Additionally, we assessed laboratory parameters, hemodynamics and Fibroscan® results for correlations with FALD on cross-sectional imaging.

Methods: This retrospective chart review study included 62 eligible patients (1990-2015) with 116 MRIs and 103 CT scans performed at a single institution. Data on demographics, surgical history, complications, imaging, laboratory results and hemodynamics were collected. FALD was categorized as congestive hepatopathy or advanced disease based on imaging. Statistical analysis included Chi-squared, Fisher’s exact, Wilcoxon Rank Sum, and Kruskal-Wallis test.

Results: The median age at FALD onset differed significantly by surgical technique, with lateral tunnel Fontan patients developing FALD later than those with extra-cardiac Fontan (p< 0.001). A trend suggested later onset in patients who underwent Fontan at an older age, though not statistically significant (p 0.14). No significant difference was observed with or without fenestration (p 0.48). Patients with a systemic LV tended to develop FALD later than those with a systemic right ventricle (p 0.054). Lab values and hemodynamic data did not predict FALD presence. Patients with FALD were more likely to have F4 level of fibrosis, while those without FALD were more likely to have F3 level of fibrosis.

Conclusion: While all patients with Fontan palliation eventually develop FALD, surgical technique and age at palliation may influence the timing of disease onset.

Volume

87

Issue

13 Suppl

First Page

A346

Comments

American College of Cardiology 75th Annual Scientific Session & Expo, March 28-30, 2026, New Orleans, LA

Helen DeVos Children’s Hospital

Last Page

A346

DOI

doi:10.1016/j.jacc.2026.02.856

ISSN

0735-1097

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