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

Background: 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. Methods: We reviewed the clinical and pathologic features of 9 autopsied patients having undergone the Fontan procedure, with special attention given to their liver pathology. Results: 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 19 years had hepatocellular carcinoma superimposed on cardiac cirrhosis. Rupture of the hepatoma in this case led to fatal hemorrhage. Conclusion: 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.