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Fontan Hepatopathy-Managing Unknowns

Dominica Zentner1, Khoa Phan2, Alexandra Gorelik3

  • 1Department of Medicine (RMH), Faculty of Medicine, Dentistry and Health Sciences, University of Melbourne, Melbourne, Vic, Australia; Department of Cardiology, Royal Melbourne Hospital, Melbourne, Vic, Australia.

Heart, Lung & Circulation
|January 15, 2023
PubMed

Insights

Monitoring Fontan-associated liver disease (FALD) in adults is challenging. Acoustic Radiation Force Impulse imaging (ARFI) can indicate cirrhosis and guide hepatocellular carcinoma surveillance, but serial ARFI offers no clear benefit once cirrhosis is established.

Area of Science:

  • Cardiology
  • Hepatology
  • Medical Imaging

Background:

  • Fontan-associated liver disease (FALD) monitoring in adults requires optimized strategies.
  • A prospective liver care pathway was implemented for 84 adults with Fontan circulation.

Purpose of the Study:

  • To evaluate the utility of Acoustic Radiation Force Impulse imaging (ARFI) in monitoring FALD.
  • To assess the correlation between ARFI, clinical endpoints, and patient characteristics.

Main Methods:

  • Prospective monitoring of 84 adults with Fontan circulation.
  • Liver assessment using ARFI and ultrasound.
  • Collated data included liver biochemistry, ventricular function, and clinical outcomes (varices, HCC, transplantation, death).

Main Results:

  • Most patients showed cirrhotic range ARFI results.
  • ARFI values correlated with hepatic nodularity and inversely with platelet count.
  • Clinical endpoints were more frequent in patients with ventricular dysfunction.

Conclusions:

  • ARFI serves as a useful non-invasive indicator for cirrhosis in FALD.
  • ARFI can identify patients needing closer hepatocellular carcinoma surveillance.
  • Serial ARFI measurements do not provide additional benefit once cirrhosis is detected.
Abstract

Keywords:
Acoustic radiation force impulse imagingCongestive hepatopathyFontan procedureLiver disease

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