The assessment and monitoring of Fontan associated liver disease (FALD) is challenging because of variability in clinical presentation and disease progression. The purpose of this study was to compare the prognostic value of liver biomarker scores to baseline (single) liver biomarker score and other modalities of liver disease assessment.

Retrospective study of adults with Fontan palliation who had ≥3 consecutive assessments of complete blood count and comprehensive metabolic panel. Model for end-stage liver disease excluding international normalized ratio (MELD-XI), fibrosis-4 (FIB-4), and aspartate transaminase to platelet ratio (APRI) scores were calculated at each visit, and average scores were calculated as the mean of the first 3 measurements. The presence of cirrhosis was determined using 4 different modalities: (1) imaging diagnosis of cirrhosis, (2) clinical diagnosis of cirrhosis, (3) histologic diagnosis of cirrhosis, (4) cirrhosis/portal hypertension based on the varices, ascites, splenomegaly, and thrombocytopenia (VAST) score.

Of 344 patients (age 30 ± 9 years, 56% males), the average MELD-XI, FIB-4, and APRI scores were 12.5 ± 4.1, 1.19 ± 0.81, and 0.61 ± 0.46, respectively. All 3 liver biomarker scores were associated with death/transplant, independent of comorbidities and hemodynamic indices. Average liver biomarker scores had superior prognostic performance compared to baseline scores, and multiparametric cirrhosis diagnosis.

These data support the use of average liver biomarker scores for risk stratification in the setting of Fontan associated liver disease (FALD). These scores can be seamlessly integrated into routine clinical practice since they are derived from blood tests that are routinely acquired during clinic visits.