Progression of liver pathology in patients undergoing the Fontan procedure: Chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma.

Abstract

Staged palliative surgical procedures have been an effective treatment of complex congenital heart defects. The Fontan procedure has been of particular benefit to infants with functional single-ventricle complexes but with the consequence of a sustained increase of right-sided venous pressure. We reviewed the clinical and pathologic features of 9 autopsied patients having undergone the Fontan procedure, with special attention given to their liver pathology. The 9 patients died from a few hours to 18 years after the Fontan operation. Chronic passive congestion was seen in 7 patients, and 4 patients surviving 4 to 18 years also had cardiac cirrhosis. Hepatic adenoma in the setting of cardiac cirrhosis was found in a patient surviving for 9 years. One patient surviving for 18 years had hepatocellular carcinoma superimposed on cardiac cirrhosis. Rupture of the hepatoma in this case led to fatal hemorrhage. The study shows that chronically increased hepatic venous pressure from the Fontan procedure might lead to chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma.