Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force

Giulia Perucca,Maria Beatrice Damasio,Maria Beatrice Damasio,Damjana Ključevšek,Philippe Petit,Kassa Darge,Stéphanie Franchi-Abella,Maria Beatrice Damasio,Kassa Darge,Samuel Stafrace,Philippe Petit,Costanza Bruno,Samuel Stafrace,Kassa Darge,Kassa Darge,Kassa Darge,Kassa Darge,Luisa Lobo,Charlotte De Lange,Lil-Sofie Ording Müller,Kassa Darge,Donald Ibe,Maria Beatrice Damasio,Michael Riccabona,Marcello Napolitano,Anne M Smets,Anne M Smets,Magdalena Maria Woźniak,Kassa Darge,Kassa Darge,Jochen Herrmann,Seema Toso,Stéphanie Franchi-Abella

doi:10.1007/s00247-021-05173-x

Abstract

Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

Highlights

The Fontan procedure was initially performed in 1968 for children affected by tricuspid atresia [1]
Venous congestion caused by this new circulation, as well as the pre, peri- and postoperative cardiac conditions, can cause hepatic fibrosis, often leading to the development of liver cirrhosis
We highlight the need for a consensus on imaging follow-up in Fontan patients

Summary

Introduction

The Fontan procedure was initially performed in 1968 for children affected by tricuspid atresia [1]. According to a recent publication by Dillman et al [4], US should be avoided as unsuitable to assess manifestations of Fontan-associated liver disease such as portal hypertension and hepatic neoplasm; rather, the authors suggested that contrast-enhanced MRI and MR elastography be performed, beginning at age 13, every other year (with CT as an alternative if MRI is contraindicated, and US shear-wave elastography in the off years in this case) as the basic follow-up protocol. Studies are needed on the correlation among liver stiffness, portal hypertension and the occurrence of HCC, an annual surveillance should allow for detection of significant pathology and provide further data on Fontan-associated liver disease development. Additional cross-sectional imaging is unanimously considered the investigational tool in cases of new liver nodules on US; MRI is preferred over CT, and the use of hepatobiliary contrast agent offers some advantages in the diagnostic accuracy. In cases of suspected malignant nodules, biopsy is recommended when possible, alongside assessment for alphafetoprotein (Fig. 2)

Conclusion

Conflicts of interest None