Congenital heart disease

Clinical Characteristics Associated With Variability in Biopsy Grade Severity of Liver Fibrosis in Fontan Circulation

JACC AdvPublished Cross-sectional study

Authors' conclusion

Liver fibrosis in FC progresses with duration of exposure, independent of venous pressure, suggesting ongoing fibrogenesis even at acceptable hemodynamics. Younger age at FO is associated with greater fibrosis severity

Study context

Population
Patients with Fontan circulation undergoing standardized evaluation incl. cardiac catheterization, percutaneous liver biopsy, liver elastography and blood laboratories
Participants
173 patients
Outcome
Liver fibrosis severity by Sirius red % staining of liver biopsy specimens, and its associations with clinical and hemodynamic factors

Main finding

As reported in the abstract

Liver fibrosis severity (Sirius red % staining on liver biopsy)
Sirius red % staining correlated with aspartate aminotransferase/platelet ratio
P = 0.026

Observational data: associations, not effects.

Further findings

Patient and hemodynamic characteristics

Median age at Fontan operation (FO), years (range: 0.5-15)
2.2
Median age at biopsy, years (range: 4.7-39.4)
17.5
Dominant RV morphology
67%

Years of Fontan circulation (FC) exposure vs other measures

Years of FC exposure correlated positively with hemoglobin, gamma glutamyl transferase and liver elastography

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Clinical Characteristics Associated With Variability in Biopsy Grade Severity of Liver Fibrosis in Fontan Circulation. JACC Adv. Published 15 December 2025. doi:10.1016/j.jacadv.2025.102421

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