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

Pediatr Radiol. 2021 Dec;51(13):2598-2606. doi: 10.1007/s00247-021-05173-x. Epub 2021 Oct 15.

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.

Keywords: Adolescents; Children; Cirrhosis; Fontan procedure; Hepatocellular carcinoma; Liver; Liver fibrosis; Magnetic resonance imaging; Ultrasound.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Child
  • Fontan Procedure* / adverse effects
  • Heart Defects, Congenital* / diagnostic imaging
  • Heart Defects, Congenital* / surgery
  • Humans
  • Liver / pathology
  • Liver Cirrhosis / pathology
  • Liver Diseases* / pathology
  • Postoperative Complications / pathology
  • Radiology*