Transplant-Amenable Hepatocellular Carcinoma in a Fontan Patient

Abstract

The Fontan circulation alters a patient’s physiology and imparts long-term risks related to chronically elevated systemic venous pressure. An increasing number of patients with Fontan physiology are surviving into adulthood and are at risk of hepatic sequalae. The ideal timeline and method of hepatic surveillance in the Fontan population remains to be defined. In this case, the patient was diagnosed with hepatocellular carcinoma more than 20 years after undergoing the Fontan procedure and was a candidate for combined heart-liver transplant. That her disease progressed prior to organ availability supports the argument for hepatic surveillance in this population.

Keywords

• Fontan procedure
• Cirrhosis
• Hepatocellular carcinoma
• Liver disease
• Congenital heart disease