Abstract
Objectives
Patients with Fontan circulation exhibit a high incidence of liver fibrosis and cirrhosis.
Transient elastography (TE) and the enhanced liver fibrosis (ELF) test have proven
useful as noninvasive surrogate markers of liver fibrosis for other chronic liver
diseases. We evaluated whether TE and the ELF score can predict the degree of liver
fibrosis in patients with Fontan circulation.
Methods
We retrospectively reviewed the medical records of 45 adult patients with at least
10 years of Fontan duration who had undergone liver biopsy and investigated the relation
between the fibrosis stage and TE and the ELF test results. Additionally, the association
of these variables and other biochemical and hemodynamic parameters was assessed.
Results
The mean age was 25.9 years and the mean Fontan duration was 20.8 years. Advanced
liver fibrosis was present in 36 (80.0%) patients. TE or ELF score are comparable
for patients with and without advanced liver fibrosis (mean 23.3 vs 24.8 kPa [P = .85] for TE; mean 8.94 vs 9.25 [P = .44] for the ELF score). However, N-terminal pro-brain natriuretic peptide level
and ventricular end-diastolic pressure were higher in patients with advanced liver
fibrosis (mean 224 vs 80 pg/mL [P < .01]; and mean 12 vs 9 mm Hg [P = .04], respectively). No independent predictor of advanced liver fibrosis was found
in multivariate analysis.
Conclusions
TE and the ELF score were unable to predict the degree of liver fibrosis in Fontan
patients. Liver biopsy remains as the only valid method to assess fibrotic burden
in this population.
Graphical abstract
Graphical Abstract
Key Words
Abbreviations and Acronyms:
ELF (enhanced liver fibrosis), HA (hyaluronic acid), LS (liver stiffness), NT-proBNP (N-terminal pro-brain natriuretic peptide), PIIINP (propeptide of type III procollagen), TE (transient elastography), TIMP-1 (tissue inhibitor of metalloproteinases-1)To read this article in full you will need to make a payment
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Article info
Publication history
Published online: December 23, 2021
Accepted:
December 16,
2021
Received in revised form:
December 2,
2021
Received:
May 10,
2021
Footnotes
This study was supported by the Basic Science Research Program through the National Research Foundation of Korea funded by the Ministry of Science, ICT and Future Planning (2019R1A2C4070136). The funders had no role in the study design, data collection and analysis, decision to publish, or preparation of the report.
Yu Rim Shin and Seung Up Kim contributed equally to this work.
Han Ki Park, Jae Young Choi, and Young Nyun Park are co-corresponding authors. All of the corresponding authors have equally contributed to this work.
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© 2021 by The American Association for Thoracic Surgery
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- Commentary: The future for adult Fontan patients: What is there to see in the liver?The Journal of Thoracic and Cardiovascular SurgeryVol. 164Issue 4
- PreviewWhereas single ventricle palliation has allowed many infants to survive to adulthood with various univentricular physiologies, the last stage of palliation, Fontan physiology, inevitably results in chronic elevated central venous pressures and relatively low cardiac output.1 As a result, end organs such as the liver sustain supraphysiologic venous pressures resulting in chronic congestion. It ultimately leads to fibrosis, cirrhosis, dysfunction, and increased risk for hepatocellular carcinoma.1,2
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