Semiquantitative characterization of dynamic magnetic resonance perfusion of the liver in pediatric Fontan patients

Pediatr Radiol. 2022 Mar;52(3):483-492. doi: 10.1007/s00247-021-05221-6. Epub 2021 Dec 2.


Background: Dynamic contrast-enhanced magnetic resonance imaging (MRI) of the liver in pediatric Fontan patients often shows peripheral reticular areas of hypoenhancement, which has not been studied in detail.

Objective: To semiquantitatively score the hepatic MR perfusion abnormality seen in pediatric Fontan patients, and to correlate the perfusion abnormality with functional clinical and hemodynamic parameters.

Materials and methods: All children (< 18 years old) after Fontan palliation with combined clinical cardiac and liver MRI performed between May 2017 and April 2019 were considered for inclusion. A semiquantitative perfusion score was used to assess the severity of the hepatic reticular pattern seen on dynamic contrast-enhanced liver imaging. The liver was divided into four sections: right posterior, right anterior, left medial and left lateral. Each liver section was assigned a score from 0 to 4 depending on the amount of abnormal reticular hypoenhancement. Scoring was assigned for each section of the liver across eight successive dynamic contrast-enhanced modified spoiled gradient echo runs. Scores were correlated with clinical and hemodynamic parameters.

Results: All Fontan children showed hepatic reticular hypoenhancement by MRI, most severe in the early portal venous phase with a median maximum total perfusion abnormality score of 12 (range: 9-14). All perfusion abnormalities progressively resolved during the hepatic venous phase. Perfusion abnormality scores were greatest in the right compared to left hepatic lobes (7 range: [6-8] vs. 5 [range: 3-6], P < 0.01). The maximum left hepatic lobe perfusion abnormality scores were greatest in children with versus without imaging signs of portal hypertension (8 [range: 7-8] vs. 4 [range: 3-5], P < 0.01). High unconjugated bilirubin and low platelets correlated with greater perfusion abnormality (R = 0.450, P = 0.024, and R = - 0.458, P < 0.01, respectively). Age at MRI, time from Fontan, focal liver lesions and cardiac MRI hemodynamic parameters did not show significant correlations with the severity of the liver perfusion abnormality.

Conclusion: All Fontan children have hepatic reticular hypoenhancement abnormalities seen with MRI that are most severe in the right hepatic lobe and universally show gradual resolution through the hepatic venous phase. Perfusion abnormality in the left hepatic lobe is worse in children with portal hypertension.

Keywords: Children; Fontan surgery; Fontan-associated liver disease; Liver; Magnetic resonance imaging; Perfusion.

MeSH terms

  • Adolescent
  • Child
  • Fontan Procedure*
  • Humans
  • Hypertension, Portal*
  • Liver / diagnostic imaging
  • Liver / pathology
  • Liver Cirrhosis / pathology
  • Magnetic Resonance Imaging / methods
  • Magnetic Resonance Spectroscopy
  • Perfusion