Transjugular liver biopsy in Fontan-associated liver disease in children: assessment of safety and diagnostic value compared with noninvasive techniques

Objectives To evaluate transjugular liver biopsy (TJLB) as a safe and effective diagnostic option in pediatric patients with liver disease secondary to Fontan surgery and to compare the histological results with those of noninvasive diagnostic tests in relation to the degree of liver fibrosis (LF). Methods A retrospective, single-center study of children under 18 years, Fontan surgery, underwent TJLB from 2019 to the present. Sociodemographic variables, type of heart disease, age at surgery, laboratory test results, ultrasound and liver elastography results, time to TJLB, hemodynamic study results, and degree of LF were collected. Results From 2019 to 2024, 28 TJLBs were performed on 26 patients: 75% male. The mean age at surgery was 5.5 ± 1 year (range: 3.6–8 years). The mean age at the time of biopsy was 16 ± 1.5 years (12–18 years). Ninety-three percent of patients were receiving antiplatelet therapy. Upper gastrointestinal endoscopy showed 36% grade II esophageal varices and 25% grade I. All patients underwent catheterization and hemodynamic evaluation after the procedure. Thirty-nine percent had platelets less than 150,000, and 35% ALT elevated (30.9 ± 17.4 IU/L, range 9–87 IU/L). Six patients had alpha-fetoprotein levels, all in normal range. Ultrasonography revealed normal results in three patients (11%), 57% homogeneous hepatomegaly, 25% parenchymal heterogeneity, and 7% liver nodules. Fifty-seven percent showed ultrasound-guided splenomegaly (11.6 ± 2.8 cm (range: 8–18 cm)), with a platelet-to-spleen ratio of 16.2 ± 6.7 (range: 4.6–2.7). Normal liver doppler in 36%; only two patients experienced portal flow reversal, and one increased portal vein. Elastography performed in 68% of patients: F4 METAVIR fibrosis in 63% and F2-F3 fibrosis in 37% (13.25 ± 4 kPa; range: 7–20 kPa). No significant differences between liver-free pressure and wedge pressure. Liver biopsies revealed grade 4 fibrosis in only 21.4%, and 64% grade 2 or 3 fibrosis. The most frequent histological involvement was sinusoidal dilatation with perisinusoidal fibrosis (86%), associated with centrocentral fibrous bridges in 43%. There was only one complication after TJLB: a mild encapsulated hepatic hematoma autocontrolled and two mild pneumothoraces after jugular vein cannulation. In the comparative study, the histological grade of LF did not correlate statistically significantly with other noninvasive indices. Conclusions TJLB is a safe and effective technique, performed concurrently with procedures such as gastrointestinal endoscopy and catheterization, with a low complication rate. In our series, the degree of fibrosis measured by elastography overestimated the degree of fibrosis measured by histological study.