Introduction

Fontan-associated liver disease (FALD) is a sequela of Fontan circulation. There is little data informing what proportion of patients will develop clinically significant chronic liver disease (CLD). This study aimed to determine the prevalence and time to development of CLD manifestations in children after Fontan.

Methods

A retrospective analysis was performed in patients post-Fontan at Texas Children’s Hospital between 1/1/2010 and 6/30/2022. Patients were included until last clinic encounter, time of heart and/or liver transplant, or death. Analysis included descriptive statistics, linear mixed regression modeling, and Kaplan-Meier plots for time to development of pre-defined CLD manifestations.

Results

In 493 post-Fontan patients (63.7% male, 53.5% Caucasian), hypoplastic left heart was the most common defect (28.3%) with majority having an extra-cardiac conduit (71.6%). Over a mean follow up of 12.6 years, manifestations included: Varices 1.6% (n=8), ascites 8.3% (n=41), hepatomegaly 14.6% (n=72), splenomegaly 16% (n=79), and thrombocytopenia 58% (n=286). First onset splenomegaly and hepatomegaly were highest 11-15 years post-Fontan, however, 32% (n=158) had thrombocytopenia pre-Fontan (Figure 1A). Mean elastography and time to development of CLD manifestations are shown in Figure 1B. Fifteen-year cumulative incidence of varices was 0.44%.

Conclusion

To our knowledge, this is the largest characterization of liver disease in children post-Fontan. Clinical manifestations of CLD are rare in the first 10 years post-Fontan. Platelet count may be a less reliable marker of CLD in this population than previously recognized. Further studies are warranted to inform which studies and biomarkers should be used when screening and monitoring for CLD.