Related Experiment Video
Updated: Jul 8, 2026
Novel In Vivo Micro-Computed Tomography Imaging Techniques for Assessing the Progression of Non-Alcoholic Fatty Liver Disease
Published on: March 24, 2023
Non-invasive fibrosis tools lack clinical utility for identifying advanced fibrosis in Fontan-associated liver
Paul Armstrong1,2, Aoife Moriarty3,2, Robert Hughes3
1The Liver Centre, Mater Misericordiae University Hospital, Dublin, Ireland paul.armstrong@ucdconnect.ie.
Insights
Most adults with Fontan-associated liver disease have advanced liver fibrosis. A patent Fontan fenestration may reduce fibrosis risk, but liver biopsy remains essential for diagnosis.
Area of Science:
- Cardiology
- Hepatology
- Medical Diagnostics
Background:
- Fontan-associated liver disease (FALD) is a complication of Fontan procedures for congenital heart disease.
- Rising Fontan survival rates increase the prevalence of FALD in adults.
- Predictors of advanced liver fibrosis in adult Fontan patients are not well understood.
Purpose of the Study:
- To evaluate the accuracy of non-invasive fibrosis assessment tools (NIT) in predicting advanced liver fibrosis in adult Fontan patients.
- To identify predictors of advanced liver fibrosis in this cohort.
- To compare the diagnostic performance of NIT with liver biopsy.
Main Methods:
- Retrospective analysis of 71 adult Fontan patients.
- Collection of demographic, cardiac, and fibrosis biomarker data, including liver stiffness measurement (LSM), FIB-4, and APRI scores.
- Histological staging of liver fibrosis using congestive hepatic fibrosis score from biopsies (2017-2024).
Main Results:
- 62% of patients exhibited histological advanced fibrosis.
- Advanced fibrosis was associated with closed Fontan fenestration and higher LSM (weak correlation).
- Non-invasive tools (APRI, FIB-4) showed limited accuracy, though APRI was associated with fibrosis in females.
Conclusions:
- The majority of Fontan patients develop advanced liver fibrosis by their third decade.
- A patent Fontan fenestration may be protective against advanced fibrosis.
- Liver biopsy remains the gold standard for diagnosing advanced fibrosis in FALD due to limitations of NIT.
Objective:
Fontan-associated liver disease (FALD) results from haemodynamic changes following the Fontan procedure for congenital heart disease and is associated with poorer outcomes. The prevalence of Fontan is rising due to improved survival; however, little is known about predictors of advanced liver fibrosis in adult patients. This study aimed to determine the accuracy of non-invasive fibrosis assessment tools (NIT) in predicting histologically confirmed advanced liver fibrosis in an adult Fontan cohort attending Mater Misericordiae University Hospital.
Methods:
Patient demographics, congenital cardiac variables and fibrosis biomarkers were recorded including liver stiffness measurement (LSM) via transient elastography, Fibrosis-4 (FIB-4) and Aspartate aminotransferase-to-Platelet Ratio Index (APRI) scores. Biopsies, taken between 2017 and 2024, were staged using the congestive hepatic fibrosis score. Analysis was performed using SPSS.
Results:
71 patients (58% male) were included. The median age was 25 years. 62% had histological advanced fibrosis. There were no significant bleeding events post biopsy. Overall, advanced fibrosis was associated with a closed Fontan fenestration (p=0.022) and higher LSM, although with a weak correlation (p=0.04, r=0.25, area under the curve (AUC) 0.65), but not with APRI or FIB-4. There was no difference in rates of advanced fibrosis between sex (p=0.84). In females, higher APRI was associated with advanced fibrosis (p=0.045, r=0.41, AUC 0.73).
Conclusions:
The majority of Fontan patients have advanced liver fibrosis in their third decade. A patent Fontan fenestration appears to reduce the risk of advanced fibrosis. Despite an association with higher LSM, there was no cut-off which could negate the need for biopsy in a significant population. Our data suggest that the discriminatory ability of NIT may vary according to sex. Liver biopsy is safe and remains the only method of reliably diagnosing advanced fibrosis in FALD.
More Related Videos
06:09Author Spotlight: Advancing Hepatic Fibrosis Diagnosis Using Magnetic Resonance Elastography and AI
Published on: July 21, 2023
05:56Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
