Imaging of Fontan-associated liver disease

Jonathan R. Dillman*, Andrew T. Trout, Tarek Alsaied, Anita Gupta, Adam M. Lubert

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

20 Scopus citations

Abstract

The Fontan operation has dramatically altered the natural history of functionally single ventricle congenital heart disease. Patients who have undergone the Fontan operation are living longer and, thus, noncardiac morbidity resulting from the Fontan operation is increasingly being recognized. Fontan-associated liver disease (FALD), one of the chief morbidities following the Fontan operation, is believed to be a multifactorial process that manifests as hepatic congestion and fibrosis, portal hypertension, and development of focal liver lesions, including malignant tumors. This article reviews the imaging findings of FALD in the pediatric and young adult population, reviews the literature related to the imaging of FALD and discusses possible screening algorithms for this population. The need for further research to better understand the causes of FALD, to establish if early liver stiffness measurements (or their change over time) predict long-term outcomes and complications, and to define optimal liver screening procedures is highlighted.

Original languageEnglish (US)
Pages (from-to)1528-1541
Number of pages14
JournalPediatric radiology
Volume50
Issue number11
DOIs
StatePublished - Oct 1 2020

Keywords

  • Children
  • Congestive hepatopathy
  • Fontan circulation
  • Fontan-associated liver disease
  • Liver
  • Magnetic resonance imaging
  • Single ventricle congenital heart disease
  • Ultrasound
  • Young adults

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Pediatrics, Perinatology, and Child Health

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