Several years ago, there were 4 cases of hepatocellular carcinoma (HCC) following Fontan procedure reported in the NEJM. (Reviewed in this blog: Hepatocellular carcinoma after Fontan Procedure).
Another recent report describes 3 patients who presented with HCC more than 10 years after Fontan procedure. The age of these patients varied from 20 to 28 years. The authors use the term Fontan-Associated Liver Disease (FALD). They note that FALD is strongly associated with the interval from the procedure, increasing in frequency with more time following surgery. The risk of FALD is 4.4 times greater between years 11-15 years than in the first 10 years.
The authors recommend screening for HCC in patients 10 years after Fontan procedure. They suggest a baseline MRI followed by biannual ultrasounds and alpha-fetoprotein tests.
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