Liver Cirrhosis and Hepatocellular Carcinoma After the Fontan Operation: Reaching Clarity in the Face of Uncertainty

Fontan-associated liver disease (FALD) encompasses a spectrum of hepatic abnormalities that are a consequence of the Fontan circulation (Figure). Endstage complications of FALD can include cirrhosis, with or without portal hypertension, and potential development of hepatocellular carcinoma (HCC). In this issue of Circulation, Nii et al1 add additional multicenter data to support growing evidence that HCC in FALD can develop in patients without obvious clinical or imaging evidence of liver cirrhosis. Such important research efforts highlight the challenges in accurate diagnosis and surveillance of liver disease in the Fontan population. Although our understanding of these important clinical manifestations of liver disease continue to evolve, considerable uncertainty persists. In this brief review, we describe the unique features of FALD and consequent implications for management.