The Importance of Hepatic Surveillance After Single-Ventricle Palliation: An Interventional Study Validating Liver...

Predictors of Fontan-associated liver disease (FALD) are not well defined. We hypothesized that magnetic resonance elastography liver stiffness measurement (LSM) would correlate with cardiac MRI findings, and hemodynamic, clinical, radiographic, and laboratory measures of FALD and Fontan failure. Patients undergoing pre- and post- Fontan assessment with same-day MRI and cardiac catheterization were enrolled. Data was collected retrospectively. 22 Fontan (mean age 12.1 ± 7.5 years) and 17 Glenn patients (mean age 4.0 ± 0.9 years) were retrospectively enrolled. LSM was higher in Fontan vs Glenn patients (3.8 ± 0.6 kPa vs 2.2 ± 0.4 kPa; p < 0.0001), as were all fibrosis scores (FIB- 4 = 0.4 $\pm$ 0.2 vs 0.1 $\pm$ 0.1, respectively, p = 0.002). LSM positively correlated with hepatic wedge pressure (r = 0.75; p < 0.0001), and all fibrosis scores (FIB- 4; r = 0.55; p = 0.001). There was negative correlation with LSM and ejection fraction (r = - 0.64; p < 0.0001). Comparing those with Fontan failure (n = 8) and those without (n = 14), only ejection fraction was statically significant (41 ± 12% vs 51 ± 4%, respectively; p = 0.012). Of the patients in Fontan failure, there was no difference in LSM between those with FALD (n = 3), and those without (n = 5), (3.7 $\pm$ 0.3 kPa vs 3.8 $\pm$ 0.6 kPa; p = 0.72). On multivariable regression analysis, LSM and GGT were independently associated with elevated hepatic venous pressure (p = 0.004 and p = 0.016, respectively). There was no correlation with LSM and Fontan duration (r = 0.15; p = 0.517). Liver stiffness rises significantly after Fontan palliation and positively correlates with hemodynamic and clinical markers of hepatic congestion and fibrosis, however its ability to predict Fontan failure and FALD remains uncertain.