Intrahepatic Transcriptomics Differentiate Advanced Fibrosis and Clinical Outcomes in Adults With Fontan Circulation
Authors' conclusion
Patients with FALD and advanced fibrosis or the CCO exhibited upregulated genes related to inflammation, congestion, and angiogenesis.
Study context
- Population
- Adults with Fontan circulation and Fontan-associated liver disease (FALD) with liver biopsy48% male
- Participants
- 106 adults
- Exposure
- Advanced vs early liver fibrosis; Composite clinical outcome (CCO) vs no CCO
- CCOn = 23
- Outcome
- Intrahepatic transcriptomic differences by degree of liver fibrosis and clinical outcomes
Main finding
As reported in the abstract
Upregulated intrahepatic genes
Upregulated genes, advanced vs early fibrosis
228
Upregulated genes, CCO vs no CCO
894
Upregulated genes in both comparisons (enriched in cytokine/oxidative stress response, VEGFA-VEGFR2 and TGF-β signaling, vasculature development)
136
Observational data: associations, not effects.
Further findings
CCO occurrence and predictors
- CCO present
- 23 (22%)
- CCO not predicted by advanced fibrosis, RV morphology, aortopulmonary collaterals or Fontan pressures on multivariable analysis
Clinical features of advanced fibrosis
- Advanced fibrosis: higher BNP and Fontan, mean pulmonary artery and capillary wedge pressures
Original publication
Intrahepatic Transcriptomics Differentiate Advanced Fibrosis and Clinical Outcomes in Adults With Fontan Circulation. J Am Coll Cardiol. Published 20 February 2024. doi:10.
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