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Fontan-Associated Liver Disease (FALD)

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Liver Disease in Clinical Practice

Part of the book series: In Clinical Practice ((ICP))

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Abstract

The Fontan procedure has significantly improved the survival rates of those born with univentricular congenital heart disease. In doing so, patients are left with a chronically elevated central venous pressure and reduced cardiac output. This leads to a spectrum of hepatic dysfunction known as Fontan-associated liver disease (FALD). FALD is progressive, with the risk and therefore incidence of cirrhosis and hepatocellular carcinoma (HCC) increasing over time. This necessitates accurate diagnostic and prognostic assessment. Biochemical markers, non-invasive fibrosis scores, radiological imaging, elastography and liver biopsy are all used in screening, diagnosis and surveillance of FALD, but without national guidelines or validated diagnostic thresholds to follow. Treatment of FALD relies on non-specific medical therapies and consideration of combined heart–liver transplantation. Clearly, further research in the form of large-scale longitudinal studies is vital to provide a stronger evidence basis for all aspects of care for these patients. Validated tools, standardised guidelines and clear referral pathways are yet to be determined. With such progress, we can harmonise the management of FALD and continue to improve patient outcomes.

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Correspondence to Tehreem P. Chaudhry .

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Asad, H., Chaudhry, T.P., Jenkins, P. (2022). Fontan-Associated Liver Disease (FALD). In: Cross, T. (eds) Liver Disease in Clinical Practice. In Clinical Practice. Springer, Cham. https://doi.org/10.1007/978-3-031-10012-3_15

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  • DOI: https://doi.org/10.1007/978-3-031-10012-3_15

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-10011-6

  • Online ISBN: 978-3-031-10012-3

  • eBook Packages: MedicineMedicine (R0)

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