Fontan-Associated Liver Disease: Trends Over Time and Relationship With Outcomes

Area of Science:

• Cardiology
• Hepatology
• Pediatric Medicine

Background:

• Fontan associated liver disease is a serious complication of single ventricle palliation.
• Understanding serial liver function changes is crucial for predicting outcomes.

Purpose of the Study:

• To describe serial liver function changes in Fontan patients from 2014-2020.
• To determine if these changes predict adverse clinical outcomes.

Main Methods:

• Retrospective chart review of 112 adult Fontan patients (age ≥18).
• Analysis of annual bloodwork (liver enzymes, AFP, creatinine) and liver imaging.
• Assessment of trends and correlation with clinical events (CHF, hospitalization, thrombosis, PLE, death).

Main Results:

• Age-adjusted AFP and creatinine increased; alkaline phosphatase decreased. No other biomarkers changed significantly.
• The proportion of patients with higher liver imaging scores increased.
• Age, but not biomarkers or imaging scores, was significantly associated with adverse events (HR 1.035, P=0.010).

Conclusions:

• Routine liver biomarkers did not worsen over time, despite increased liver imaging abnormalities.
• Current bloodwork and imaging assessments did not correlate with clinical outcomes in Fontan patients.
• Further research is needed to identify reliable predictors of Fontan associated liver disease progression.