Comment: Fontan-associated liver disease (FALD) has become a well-recognized sequela of single ventricle physiology with a Fontan circulation. After creation of the Fontan circuit a progressive macroscopic changes such as liver fibrosis and cirrhosis are observed over time. These are the single most important risk factors for the development of hepatocellular (HCC) carcinoma in the non-Fontan population. Although HCC in the Fontan population is uncommon, mortality is high and no clear risk factors have been identified to date.
This multicenter study includes a population of 54 Fontan patients, comprising all patients diagnosed in the nine participating centers and 20 published cases retrieved from Pubmed.
Mean age at HCC diagnosis was 30+9.4 years with the youngest patient being only 12 years of age. Mean duration from Fontan surgery to HCC diagnosis was 21.6+7.4 years. The most frequent location of HCC was right hepatic lobe. It is important to note that HCC was asymptomatic at the time of diagnosis in 50% of Fontan patients, alpha-fetoprotein (AFP) was normal in 26% of the cases and only 51% patients had liver cirrhosis. Cumulative survival after 12 and 24 months were 50% and 40%, respectively. Once the tumor became symptomatic and/or reached a size of >4 cm, 1-year-survival rates dropped to 30% and even lower in the presence of metastases.
The heterogeneity of presentation suggests that the relation between Fontan hemodynamics and the biochemical scene triggering the development of HCC is complex. Further studies including a comparison cohort of matched Fontan patients without HCC may help to identify variables associated to a higher risk of HCC development. In the meanwhile, the optimal timing of initial assessment, modality and frequency of surveillance remain topics of debate1 but close surveillance of this patients is warranted since development of HCC has been described even after successful cardiac transplant.
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