Hepatic fibrosis and cirrhosis in the Fontan circulation: a detailed morphological study

Abstract

The histological features of the liver in patients with a Fontan circulation are similar to those described in cardiac sclerosis. Sinusoidal dilatation and sinusoidal fibrosis are marked in the Fontan series. The presence of a significant amount of orcein negative sinusoidal fibrosis suggests there may be a remediable component, although the dense fibrous bands are predominantly orcein positive, suggesting chronicity and permanence. No inflammation or hepatocellular damage is evident, suggesting that fibrosis may be mediated by a non-inflammatory mechanism.

Liver Cirrhosis and/or Hepatocellular Carcinoma Occurring Late After the Fontan Procedure ― A Nationwide Survey in Japan ―

“…20 However, no inflammation or hepatocellular damage was evident in patients undergoing the Fontan procedure, suggesting that fibrosis may be mediated by a non-inflammatory mechanism. 21 We could not determine the most reliable marker for the detection of liver disease in patients undergoing the Fontan procedure in the present study. However, although the mean ALT level was within the normal range, the mean AST level was higher than the normal range, suggesting that the AST/ALT ratio may be a suitable marker to detect liver disease in patients undergoing the Fontan procedure.…”

Hepatic sinusoids in liver injury, inflammation, and fibrosis: new pathophysiological insights

“…Degree of thrombosis was thought to trigger degree of necrosis and consecutive fibrogenesis [58]. Of particular interest, fibrosis in congestive hepatopathy did not seem to result from inflammation, as no inflammatory infiltrates have been found in liver specimens from patients having undergone the Fontan procedure [60]. So far, these concepts have not been tested experimentally due to the nonexistence of an adequate animal model.…”

Human Heterotaxy Syndrome

“…105,106 Hepatic Dysfunction Hepatic dysfunction, liver fibrosis, and cirrhosis are common Sinusoidal dilatation and sinusoidal fibrosis are marked in Fontan patients. 107, 108 The cause of the hepatic changes may be chronic elevation of central venous and portal venous pressures. 87 Significant portal fibrosis had occurred in most of those who died soon after the Fontan procedure, suggesting that hepatic fibrosis begins before the Fontan operation.…”