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Noninvasive surrogates are poor predictors of liver fibrosis in patients with Fontan circulation

  • Yu Rim Shin
    Affiliations
    Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea

    Severance Cardiovascular Hospital, Seoul, Republic of Korea
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  • Seung Up Kim
    Affiliations
    Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea

    Institute of Gastroenterology, Yonsei University College of Medicine, Seoul, Republic of Korea

    Yonsei Liver Center, Severance Hospital, Seoul, Republic of Korea
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  • Sak Lee
    Affiliations
    Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea

    Severance Cardiovascular Hospital, Seoul, Republic of Korea
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  • Jae Young Choi
    Affiliations
    Severance Cardiovascular Hospital, Seoul, Republic of Korea

    Department of Pediatric Cardiology, Yonsei University College of Medicine, Seoul, Republic of Korea
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  • Han Ki Park
    Correspondence
    Address for reprints: Han Ki Park, MD, PhD, Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, 50-1 Yonsei-ro, Seodaemun–gu, Seoul, 03722, Republic of Korea.
    Affiliations
    Division of Cardiovascular Surgery, Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea

    Severance Cardiovascular Hospital, Seoul, Republic of Korea
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  • Jeong Eun Yoo
    Affiliations
    Department of Pathology, Yonsei University College of Medicine, Seoul, Republic of Korea
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  • Young Nyun Park
    Affiliations
    Department of Pathology, Yonsei University College of Medicine, Seoul, Republic of Korea
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Published:December 23, 2021DOI:https://doi.org/10.1016/j.jtcvs.2021.12.028

      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

      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)
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      Linked Article

      • Commentary: The future for adult Fontan patients: What is there to see in the liver?
        The Journal of Thoracic and Cardiovascular SurgeryVol. 164Issue 4
        • Preview
          Whereas 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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