Fontan-Associated Hepatocellular Carcinoma: Case Report and Management Dilemmas

Fontan-Associated Liver Disease (FALD) is a recognized complication post-Fontan procedure in surviving patients as a result of the long-term pathophysiological effects of elevated central venous pressure (CVP) on a hepatocellular level. While liver cirrhosis can occur, the risk of developing hepatocellular carcinoma (HCC) may develop without any histological or radiological evidence of cirrhosis. We describe the case of a 24-year-old male with a history of hypoplastic left heart syndrome, status post-Fontan procedure 16 years ago, and von Willebrand disease, who presented with mild right upper quadrant abdominal pain and fever. A CT scan was done on presentation and showed multifocal hepatic lesions with radiological features of HCC with no evidence of cirrhosis. Fontan patients are at higher risk of developing HCC even without cirrhosis. Increased awareness among physicians who are routinely involved with patients and initiating a structured surveillance program for this high-risk population is recommended.