Limited association between liver stiffness and clinical outcomes in Fontan-associated liver disease: a retrospective analysis.

Cai L, Choudhary P, Tanous D, George J.

research-articleOpen access

AI summary — for doctors, generated from the abstract only

In a study of adult Fontan patients monitored for liver disease, a simple non-invasive liver stiffness test did not reliably predict which patients would develop serious liver complications, and the researchers concluded that this test alone, along with standard echocardiogram monitoring, is insufficient for identifying at-risk patients.

What was studied

A retrospective case series of 65 adult Fontan circulation patients (111 total liver stiffness tests, 2015-2024) at a single tertiary hospital, examining the evolution of Fontan-associated liver disease over time and the correlation between liver stiffness measurements, echocardiographic findings, and blood test results with clinically significant portal hypertension and hepatic decompensation.

Key findings

There was no significant change in liver stiffness measurements when comparing two consecutive decade periods after Fontan surgery, suggesting relative stability of this measure over the broad time periods examined.
On echocardiography, only inferior vena cava diameter and degree of heart pumping function correlated significantly with liver stiffness scores (p=0.032 and p<0.001, respectively).
Twelve patients had clinically significant portal hypertension, which correlated with several specific blood test abnormalities (liver enzymes, bilirubin, clotting measures); 4 patients had decompensated liver cirrhosis and 8 warranted specialist multidisciplinary review, but these more serious outcomes did not correlate with liver stiffness or fat content measurements.

Clinical relevance

These findings suggest that relying solely on liver stiffness measurement and standard echocardiogram monitoring is insufficient for identifying Fontan patients at risk for serious liver complications, supporting the need for more comprehensive, regular, multidisciplinary testing approaches and further multicenter research to develop better predictive models for this understudied complication.

Who should care

Pediatric Cardiology

Limitations

This is a retrospective, single-center case series without a standardized testing protocol across all patients, which could introduce variability in how and when measurements were obtained; the relatively small number of patients with serious outcomes (4 with decompensated cirrhosis) limits statistical power for detecting predictive associations.

A simple liver stiffness test commonly used to monitor Fontan patients for liver problems failed to reliably identify which patients would develop serious liver complications, suggesting doctors need more comprehensive monitoring approaches for this common but poorly understood complication.

This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.

Abstract

The Fontan procedure is a palliative operation for patients with univentricular physiology. Liver complications post-surgery are inevitable and include liver cirrhosis and hepatocellular carcinoma (HCC). Routine surveillance includes transient elastography (TE) and clinical assessment, however longitudinal data on Fontan-associated liver disease (FALD) is scarce. This study aimed to describe the evolution of FALD and assess the correlation between liver stiffness measurement (LSM) and hepatic decompensation. This retrospective case series comprised all adult post-Fontan patients presenting to a tertiary hospital between January 2015 and December 2024. Demographic and clinical information, serum tests, TE and echocardiograms were extracted from medical records. Endpoints assessed were decompensated cirrhosis and findings that warrant HCC multi-disciplinary team review. A derived surrogate outcome, clinically significant portal hypertension (CSPH) was assessed using the Baveno VII criteria. There were a total of 65 patients with 111 Fibroscans™. There was no significant change in LSM by TE when comparing two consecutive decade years post Fontan surgery. On echocardiography, only inferior vena-cava diameter and degree of ventricular function correlated with LSM scores (p = 0.032, p < 0.001). Twelve patients had CSPH, correlating with increased gamma-glutamyltransferase, bilirubin, international normalised ratio and aspartate aminotransferase levels (p = 0.047, 0.034, 0.012, 0.049 respectively). Four patients exhibited features of decompensated cirrhosis, eight warranted multi-disciplinary team discussion however these did not correlate with LSM or steatosis results. LSM and echocardiogram monitoring alone is insufficient for identifying FALD patients at risk of hepatic decompensation. Regular testing and multicenter research is required to develop predictive models that accurately identifies these at-risk patients.

DOI

10.1016/j.ijcchd.2026.100685

PMID

42293360

PMCID

PMC13264328

Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.

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