Hepatotocellular Carcinoma-A Rare Complication Post Fontan Operation

In this case report, we present one of the very rare long-term complications of Fontan circulation, the development of hepatocellular carcinoma in a 29-year-old man with tricuspid atresia, who had undergone a classical Fontan operation at 10 years of age with further conversion to extra-cardiac Fontan at 27 years of age. In patients with classical Fontan connection, the elevated central venous pressure results in hepatic congestion, portal hypertension and together with an inefficient ventricular vascular coupling, may cause chronic hepatic injury and development of cardiac cirrhosis. We recommend regular physical examination, biochemical tests and additional imaging modalities to detect any early liver cirrhosis or suspicious hepatic lesion and propose the inclusion of alpha-foeto protein as part of surveillance testing in Fontan patients with liver cirrhosis.