Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents

Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents.

Rathgeber SL, Guttman OR, Lee AF, Voss C, Hemphill NM, Schreiber RA, Harris KC.

J Am Heart Assoc. 2020 Jan 7;9(1):e012529. doi: 10.1161/JAHA.119.012529. Epub 2020 Jan 4.

PMID: 31902322 Free PMC Article

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Take Home Points:

  • Fontan-associated liver disease begins in childhood.
  • Liver stiffness measured by transient elastography is associated with splenomegaly and time since Fontan operation.

Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: Fontan-associated liver disease is an increasingly important complication and already starts in childhood. In this study the authors evaluated 76 pediatric Fontan patients with a median age of 11.7 years. In all patients an echocardiogram, laboratory studies, transient elastography and an abdominal ultrasound were performed. Furthermore, the aspartate transaminase (AST) to platelet ratio index (APRI) was calculated. 17 patients underwent additional liver biopsy.

The authors found a significant correlation between the time since Fontan operation as well as splenomegaly and liver stiffness measured by transient elastography (see figure).

In addition, the presence of splenomegaly was found to be associated with low platelets, AST and alkaline phosphatase. Liver biopsy did not demonstrate severe changes in this relatively young cohort, but mild-to-moderate histopathologic liver changes were found in all patients. There was no significant difference regarding liver stiffness between patients with a dominant left and right ventricle.

The results are very interesting, but the study also clearly demonstrates that further research in Fontan-associated liver disease is needed.

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