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

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.