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

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.

Liver Biopsy Leads to Serendipitous Diagnosis of Glycogen Storage Disease Type IX in a Patient With Fontan-Associated Liver Disease

“…This case exemplifies the overlapping signs and symptoms between FALD and GSDs and shows the utility of liver biopsy particularly in atypical presentation of patients with FALD. There are conflicting guidelines in the literature for FALD imaging and liver biopsy surveillance (5,(7)(8)(9)(10). The American Heart Association and European Society of Pediatric Radiology both recommend noninvasive surveillance with ultrasound, elastography, and if indicated cross sectional imaging starting in childhood.…”

Liver cirrhosis in children – the role of imaging in the diagnostic pathway

“…Quantitative longitudinal measurement of liver stiffness is used to control disease activity in a variety of disorders. The change in liver stiffness over time (delta) has been suggested to direct management of patients with Fontan circulation [ 93 , 94 ]. Liver stiffness has been established as a quantitative, noninvasive method to monitor disease progression in patients with autoimmune disorders and especially autoimmune hepatitis [ 95 ].…”

Multiple Liver Nodules in Fontan-Associated Liver Disease

“…The Fontan procedure is a palliative surgery for patients with an anatomic or functional single-ventricular congenital heart disease, consisting of a total extracardiac cavopulmonary connection created by anastomosing the superior vena cava to the right pulmonary artery (PA) and insertion of an extracardiac conduit between the inferior vena cava and the PA [ 1 , 2 ]. The consequent chronic liver congestion and ischemia result in FALD, leading to cirrhosis in 1–5% of patients per year and hepatocellular carcinoma (HCC) in 1.3% [ 1 , 2 , 3 ]. Indeed, annual and early liver surveillance is mandatory [ 1 ].…”

“…The consequent chronic liver congestion and ischemia result in FALD, leading to cirrhosis in 1–5% of patients per year and hepatocellular carcinoma (HCC) in 1.3% [ 1 , 2 , 3 ]. Indeed, annual and early liver surveillance is mandatory [ 1 ].…”

“…This clinical case highlights an association between multiple FNH and FALD. Given the inherent risk of HCC in FALD and similar MRI findings between FNH and HCC in this background, their differential diagnosis becomes challenging [ 1 , 2 ]. In this context, AFP and MRI hepatobiliary contrast are key [ 2 , 3 ].…”