Given the fact that chronic liver disease and cirrhosis can develop in patients after the Fontan procedure, it comes as little surprise that cases of hepatocellular carcinoma (HCC) are being reported as well (NEJM 2013; 368: 1756-57).

This letter to editor describes four patients ages 24 to 42 who developed HCC following a classic Fontan or a variation.  Three of the four had very elevated alpha-fetoprotein levels; the lowest of the four patients was 106 ng/mL.  The letter notes that cirrhosis “may develop…approximately 11 to 15 years after a Fontan procedure; an incidence of cancer of 1.5 to 5.0% per year” is estimated after development of cirrhosis based on previous studies.

The letter also describes difficulties with regard to potential screening and treatment.

Related blog entry/references:

• Congenital heart disease and the Liver | gutsandgrowth
• Looking for trouble | gutsandgrowth
• -J Thorac Cardiovasc Surg 2005; 129: 1348-52
• -Transplantation 2009; 88: 219-25
• -J Am Coll Cardiol 2008; 52: e143-e263.