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CT and MR imaging findings of the livers in adults with Fontan palliation: an observational study

  • Hepatobiliary
  • Published:
Abdominal Radiology Aims and scope Submit manuscript

Abstract

Purpose

To describe liver imaging findings and complications on computed tomography (CT) or magnetic resonance imaging (MRI) in adults with Fontan palliation and investigate whether imaging features show correlations with clinical and physiological parameters.

Methods

Our Institutional Review Board approved this retrospective study. Two blinded abdominal radiologists reviewed abdominal CT (n = 21) and MRI (n = 16) images between September 2011 and October 2017 in 37 adults (median age 27 years, interquartile range 21–36 years, 14 males [38%]) with a Fontan palliation (median post-Fontan duration 22 years, interquartile range 19–28 years). Correlation between CT/MRI findings and clinical parameters including laboratory results within 6 months of CT/MRI examinations was assessed by Spearman’s rank correlation coefficient.

Results

Lobulated hepatic surface and blunt hepatic edge were seen in 92% (34/37) and 95% (35/37) of patients, respectively. Surface nodularity was noted in 32% (12/37). In 7 patients, there were 11 hepatic nodules which showed arterial-phase hyperenhancement and washout. Among them, 2 were biopsy-proven hepatocellular carcinomas (HCCs), and the remaining 9 were focal nodular hyperplasia (FNH)-like nodules. Suprahepatic inferior vena cava (IVC) diameter showed positive correlations with post-Fontan duration (p < 0.01), serum gamma-glutamyl transferase (p < 0.01), and total bilirubin (p < 0.01).

Conclusion

The livers in post-Fontan adults show a unique morphology of blunt edge and lobulating surface with occasional nodularity. There is a diagnostic challenge in distinguishing HCCs from FNH-like nodules in post-Fontan population due to overlapping imaging findings. Suprahepatic IVC diameter is a potentially useful imaging marker that reflects hepatic dysfunction in Fontan palliation.

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Abbreviations

CT:

Computed tomography

MRI:

Magnetic resonance imaging

BNP:

Brain natriuretic peptide

HCC:

Hepatocellular carcinoma

FNH:

Focal nodular hyperplasia

IVC:

Inferior vena cava

GGT:

Gamma-glutamyl transferase

FALD:

Fontan-associated liver disease

T2WI:

T2-weighted imaging

T1WI:

T1-weighted imaging

Gd-EOB-DTPA:

Gadolinium-ethoxybenzyl-diethylenetriaminepentaacetic acid, gadoxate disodium

HU:

Hounsfield unit

DWI:

Diffusion-weighted imaging

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Acknowledgements

We thank Kyunghee Han (University of Pennsylvania) for providing a statistical advice for this manuscript.

Author information

Authors and Affiliations

  1. Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, 585 University Avenue, Toronto, ON, M5G 2N2, Canada

    Hyun Kyung Yang, Hyun-Jung Jang, Korosh Khalili & Tae Kyoung Kim

  2. Department of Cardiology, University Health Network, University of Toronto, 585 University Avenue, Toronto, ON, M5G 2N2, Canada

    Rachel M. Wald

  3. Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Shi-Joon Yoo

  4. Department of Medical Imaging, St. Michael’s Hospital, University of Toronto, 30 Bond Street, Toronto, ON, M5B 1W8, Canada

    Hyun Kyung Yang

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  1. Hyun Kyung Yang
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Correspondence to Tae Kyoung Kim.

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Institutional Review Board of University Health Network, Toronto, ON, Canada, approved this retrospective study.

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Yang, H.K., Jang, HJ., Khalili, K. et al. CT and MR imaging findings of the livers in adults with Fontan palliation: an observational study. Abdom Radiol 45, 188–202 (2020). https://doi.org/10.1007/s00261-019-02119-8

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  • DOI: https://doi.org/10.1007/s00261-019-02119-8

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