Fontan-Associated Liver Disease: A Single-Center's Experience With Transvenous Hepatic Biopsy and Shear-Wave Elastography

Abstract

Objective: To report our center's experience with hepatic fibrosis scores derived from transvenous hepatic biopsy and ultrasound shear-wave elastography (SWE) values in post-Fontan patients. Methods: We identified patients who underwent a lateral tunnel Fontan (LTF) or an extracardiac Fontan (ECF) Fontan between 1990 and 2020. We further identified those who underwent transvenous hepatic biopsy between February 2013 and February 2025 and SWE between January 2017 and February 2025. We defined a total hepatic fibrosis score (TFS 0-8) as the sum of the portal fibrosis (0-4) and sinusoidal fibrosis (0-4) scores. Results: We identified 318 patients. Of the 318, 204 (64.1%) were male, 266 (83.6%) had ECF, and 52 (16.4%) had LTF, 173 (54.4%) ≥5 years of age underwent 265 transvenous liver biopsies. Of the 265 biopsies, 220 were performed in 143 ECF and 45 in 30 LTF patients. Of the 173 biopsied patients, 76 underwent 115 SWE measurements. Analysis of correlation (TFS vs kPa) between 160 unique biopsy and elastography pairs, showed a correlation coefficient of R = 0.6, P ≤ .00001. Of the 160 comparisons, 67 (41.8%) had TFS 0-2 (mild), average kPa: 10.7 ± 2.3, 76 (47.6%) had TFS 3-5 (moderate), average kPa: 12.6 ± 3.3 and 17 (10.6%) had TFS 6-8 (severe), average kPa: 15.6 ± 3.7, P = .00001. We found no significant correlation between Fontan mean pressures and elastography values, R = 0.1, P = .29. Conclusions: In post-Fontan patients, we found a moderately strong correlation between SWE and TFS. Further, although data overlap, SWE may be clinically useful in differentiating the degree of hepatic fibrosis, especially mild versus severe.