Hepatic pathology after Fontan palliation: spectrum of imaging findings

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

To define the hepatic imaging characteristics associated with Fontan circulation.

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.

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.

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.