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Pediatric Radiology
Published in: Pediatric Radiology 3/2013

01-03-2013 | Original Article

Hepatic pathology after Fontan palliation: spectrum of imaging findings

Authors: Daniel B. Wallihan, Daniel J. Podberesky

Published in: Pediatric Radiology | Issue 3/2013

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Abstract

Background

Patients with congenital heart disease corrected by Fontan palliation have chronic liver congestion that commonly progresses to fibrosis and cirrhosis with resultant complications.

Objective

To define the hepatic imaging characteristics associated with Fontan circulation.

Materials and methods

A retrospective study was performed in patients who underwent Fontan palliation who had CT or MR examinations including the liver. The liver was evaluated for parenchymal morphology, abnormal enhancement, nodules and imaging findings of fibrosis and cirrhosis.

Results

MRI or CT examinations including the liver were evaluated in 42 Fontan patients. The most common imaging finding was abnormal parenchymal enhancement, present in 38 patients. Hypervascular nodules were present in 13 patients (31%). Imaging findings of cirrhosis were seen in eight patients (19%). One patient with cirrhosis had a large liver mass, subsequently diagnosed as fibrolamellar hepatocellular carcinoma.

Conclusion

A high percentage of patients had imaging abnormalities of the liver, chiefly abnormal parenchymal enhancement, which became more apparent as the duration of the Fontan circulation increased. The hypervascular nodules sometimes present had imaging characteristics most closely resembling those of focal nodular hyperplasia. The underlying fibrosis and eventual development of cirrhosis raise the risk of developing hepatocellular carcinoma.
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Metadata
Title
Hepatic pathology after Fontan palliation: spectrum of imaging findings
Authors
Daniel B. Wallihan
Daniel J. Podberesky
Publication date
01-03-2013
Publisher
Springer-Verlag
Published in
Pediatric Radiology / Issue 3/2013
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-012-2531-y

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