Arterialised hepatic nodules in the Fontan circulation: Hepatico-cardiac interactions

Congenital Heart Disease in the Older Adult

“…Several studies have shown that these abnormalities on CT scan are common among Fontan patients, 182,183 and they correlate with hepatic vein and Fontan pressure. 182,188 Hypervascular liver lesions are concerning findings in this patient population, because these lesions may be seen in hepatocellular carcinoma. 187 In fact, there have been cases of hepatocellular carcinoma reported in Fontan patients with cardiac hepatopathy.…”

“…187 In fact, there have been cases of hepatocellular carcinoma reported in Fontan patients with cardiac hepatopathy. 188,189 Diffusion-weighted MRI and ultrasonography can both be used to image the liver. Contrast-enhanced ultrasound has been shown to improve the diagnosis and assessment of liver cirrhosis.…”

Fontan procedure: imaging of normal post-surgical anatomy and the spectrum of cardiac and extracardiac complications

“…Chronic increase of the venous pressure results in the development of regenerative nodules and the formation of intra-and extrahepatic VVS. 2,14,17,18 Regenerative nodules have a similar appearance to focal nodular hyperplasia (FNH) on dynamic contrast-enhanced imaging: both are brightly hyperenhancing lesions on the arterial phase and slightly hyperintense or isointense to the background liver on the venous phase, and must be differentiated from hepatocarcinoma, which shows the typical contrast medium washout on delayed images, 2 among other distinctive features. Benign hypervascular hepatic nodules, frequently located in the periphery, are common in the failing Fontan circulation (Fig 11a).…”

“…A recent study showed that postmortem histology suggests FNH is the underlying disease and its presence very likely indicates arterialization of hepatic blood flow and reciprocal portal venous deprivation. 18 …”

Long-term Issues After the Fontan Procedure

“…Liver biopsy and transhepatic gradients often are required to fully assess the liver. 30 All patients with Fontan circulation should be assessed for liver dysfunction, and was only from 5 to 12 years postoperation. Longer follow-up to determine which procedure is superior for arrhythmia prevention is needed.…”