Assessment of liver fibrosis using a 3-dimensional high-resolution late gadolinium enhancement sequence in children and adolescents with Fontan circulation

Objectives To assess abnormal liver enhancement on 15–20 min delayed 3D high-resolution late gadolinium enhancement (3D HR LGE) sequence in patients with Fontan circulation. Methods Retrospective study of pediatric Fontan patients (< 18 years old) with combined cardiac-liver MRI from January 2018 to August 2021. Abnormal hepatic enhancement was graded (0–3) for each lobe, summed for a total liver enhancement score (0–6), and compared to repaired tetralogy of Fallot (rTOF) patients. Correlations with other hepatic imaging biomarkers were performed. Temporal relationships of enhancement compared to traditional early portal venous and 5–7-min delayed phase liver imaging were analyzed. Results The Fontan group ( n = 35, 13 ± 3.4 years old, median time from Fontan 10 (9–12) years) had 23/35 (66%) with delayed 3D HR LGE total liver enhancement score > 0 (range 0–5), with greater involvement of the right lobe (1 (0–1) vs 0 (0–1), p < 0.01). The rTOF group ( n = 35, 14 ± 2.6 years old) had no abnormal enhancement. In the Fontan group, total liver enhancement was 3 (2–4) in the early portal venous phase, lower at 1 (1–2) in the 5–7-min delayed phase ( p < 0.01), and lowest at 1 (0–2) in the 15–20-min delayed phase ( p = 0.03). 3D HR LGE enhancement correlated inversely with portal vein flow ( r s = − 0.42, p = 0.01) and positively with left lobe stiffness ( r s = 0.51, p < 0.01). The enhancement score decreased in 13/35 (37%) between the 5–7- and 15–20-min delayed phases. Conclusions Liver fibrosis can be assessed on 3D HR LGE sequences in patients with Fontan circulation, correlates with other imaging biomarkers of Fontan liver disease, and may add information for hepatic surveillance in this population. Key Points • Abnormal liver enhancement on 3D HR LGE sequences in Fontan patients likely represents liver fibrosis and is seen in up to 66% of children and adolescents with variable distribution and severity. • The degree of 3D HR LGE liver enhancement correlates with decreased portal vein flow and increased left hepatic lobe stiffness.