Background and Aims
How to best monitor Fontan associated liver disease (FALD) remains unclear. We describe
results from a prospective liver care pathway in adults (n=84) with a Fontan circulation.
Methods
Routine assessment of the liver, by acoustic radiation force frequency and ultrasound
was undertaken. Results, including liver biochemistry, systemic ventricular function
(echocardiography), functional class, medication use and clinical endpoints (varices,
hepatocellular carcinoma, heart transplantation and death) were collated.
Results
Most individuals returned a cirrhotic range acoustic radiation force impulse imaging
(ARFI) result. ARFI values were greater in the proportion of individuals with hepatic
nodularity (p=0.024). Univariate analysis demonstrated moderate correlation with platelet
number (Spearmans rho= -0.376, p=0.049). Patients with clinical endpoints had lower
platelets (p=0.012) but only a trend to hepatic nodularity (p=0.057). Clinical endpoints
were more common in those with ventricular dysfunction (p=0.011).
Multivariate analysis revealed that age at Fontan and being on angiotensin converting
enzyme inhibitors (ACEI) predicted ARFI score (β=0.06 (95% CI 0.01–0.09), p=0.007
and β=0.53 (95% CI 0.17–0.89), p=0.005, respectively). However, these associations
were not significant once adjusted for Fontan type, age at ARFI, systemic ventricle
morphology, ventricle function, or Model for End-stage Liver Disease (MELD-XI) excluding
international normalised ratio (INR) (p>0.05 for all).
Conclusions
Ideal FALD monitoring remains unclear. ARFI has utility as a binary non-invasive indicator
of cirrhosis, highlighting individuals who may need more frequent ongoing monitoring
for hepatocellular carcinoma. However, no definite advantage to serial ARFI, once
cirrhotic range ARFI results are present, has been identified.
Keywords
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Article info
Publication history
Published online: January 13, 2023
Accepted:
December 8,
2022
Received in revised form:
November 24,
2022
Received:
September 3,
2022
Publication stage
In Press Journal Pre-ProofIdentification
Copyright
© 2022 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.
