Fontan-associated liver disease (FALD) is a well-recognized complication of single ventricle palliation with the Fontan operation. The exact etiology is unclear but is related to chronically elevated central venous pressures and low cardiac output resulting in liver fibrosis and subsequent cirrhosis. Hepatocellular carcinoma (HCC) is a rare but serious complication of FALD. The reported prevalence of HCC after a Fontan operation is between 1-3% in different series of published studies. In a recent multicenter case series of 54 patients with a Fontan circulation and diagnosed with HCC, the mean age at diagnosis was 30+/- 9.4 years with the youngest patient being 12 years of age. Additionally, the mean duration from Fontan surgery to HCC diagnosis was 21.6 +/- 7.4 years and the 1-year survival was 50%. Survival was further decreased if the tumor was symptomatic, more than 4 cm in size, or had metastasized.


Due to the numerous complications associated with the Fontan circulation, these patients require life-long follow up and regular screening for FALD. Recommendations for surveillance have recently been published which include a clinical assessment, liver function tests, serum biomarkers such as FibroSure and alpha-fetoprotein, imaging with an abdominal ultrasound, abdominal computed tomography scan or abdominal magnetic resonance imaging, and a liver biopsy. The surveillance interval should shorten with longer time elapsed from the Fontan completion.


References


1) Possner M, Gordon-Walker T, Egbe AC, et al. Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes. Int J Cardiol. 2021; 322: 142-148.


2) Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with a Fontan circulation: A scientific statement from the American Heart Association. Circulation. 2019; 140(6): 234-284.


3) Gorden-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: A review. J Cardiol. 2019; 74: 223-232.