Do Hemodynamic and Echocardiographic Derangements after the Fontan Operation Correlate with Severity of Liver Disease on Biopsy?

Background : Liver biopsy remains the gold standard for evaluating Fontan-associated liver disease. Prior studies show that unfavorable Fontan hemodynamics are associated with a higher risk for developing liver disease. We sought to determine whether hemodynamics pre- and post-Fontan, severity of atrioventricular valve regurgitation (AVV), and systemic ventricular dysfunction by echocardiography correlate with the degree of liver disease on biopsy. Methods : In this single-center retrospective study, we included patients with Fontan physiology who had a liver biopsy between January 2008 and May 2024. Pre- and post-Fontan catheterization data, as well as post-Fontan echocardiogram data, were reviewed. Severity of liver fibrosis was classified by the congestive hepatic fibrosis score. Data was analyzed using dichotomization (low grade score 0-2B and high grade 3-4) with chi-square and Fisher’s tests. Results : 154 post-Fontan liver biopsies corresponding to 120 patients were analyzed. The median time between the Fontan operation and liver biopsy was 23 years [IQR 17, 28]. The median Glenn pressure was 14mmHg at pre-Fontan catheterization [IQR 11, 15]. On post-Fontan catheterization, median Fontan pressure was 18mmHg [IQR 16, 22], and median systemic saturation was 92% [IQR 85, 95]. High-grade hepatic fibrosis did not significantly correlate with time from Fontan, elevated Glenn pressure, post-Fontan systemic venous pressure, or post-Fontan systemic ventricular function. The correlation between moderate or worse systemic AVV regurgitation approached but did not reach statistical significance (p = 0.051). Conclusion : Neither pre- or post-Fontan hemodynamics nor significant AVV regurgitation or systemic ventricular dysfunction are significantly associated with high-grade liver disease in patients with Fontan physiology.