Fontan-associated liver disease (FALD) may be caused by chronic liver congestion due to high central venous pressure (CVP). Recently, the usefulness of liver native T1 mapping in magnetic resonance imaging (MRI) in adulthood has been reported. To evaluate the usefulness of native liver T1 mapping in children with congenital heart disease (CHD), we investigated the utility of native liver T1 relaxation time (LT1) in pediatric Fontan patients in comparison to other CHDs. Correlations between LT1 and laboratory biomarkers or hemodynamic data were also assessed. A total of 155 patients with CHD (biventricular repair, n = 42; bidirectional Glenn circulation, n = 38; and Fontan circulation, n = 75) underwent blood tests, cardiac catheterization, and cardiac MRI within 48 h. Both CVP and LT1 levels were higher in Fontan patients than in bidirectional Glenn and biventricular patients. There were significant correlation in the overall population and weak correlation in Fontan patients between CVP and LT1(correlation coefficient 0.644 [0.541-0.728] and 0.244 [0.0179-0.446], P < 0.001 and 0.035, respectively). Among the laboratory data, the multiple linear regression analysis revealed that the fibrosis-4 index and alanine aminotransferase were significantly correlated with LT1 in the overall population (P = 0.008,0.012), and the fibrosis-4 index was correlated with LT1 in Fontan patients (P = 0.019). LT1 might have some role to predict elevated CVP and liver injury in children with CHD.
Keywords: Central venous pressure; Congenital heart disease; Fontan-associated liver disease; Liver native T1 mapping.
© 2024. The Author(s), under exclusive licence to Springer Nature B.V.