Surveillance for hepatocellular carcinoma secondary to cardiogenic cirrhosis in patients with congenital heart disease

Liver disease secondary to congenital heart disease in children

“…In the non-cancerous region, fibrous thickening was observed in the central vein. In 2015, HCC was reported to have developed in a 24-year-old woman with tetralogy of Fallot (TOF) [30]. Although she underwent three separate cardiac operations, Fontan palliation was not performed.…”

“…Nonetheless, it is reasonable to speculate that severe hepatic fibrosis is associated with HCC. In fact, the majority of the reported cases of FALD with HCC were accompanied by severe hepatic fibrosis [5,7,10,12,[14][15][16][17]30]. Thus, the quantification of hepatic fibrosis is indispensable for predicting the development of HCC in this population.…”

“…Aside from the literature on FALD, there have been few reports, there have been few reports that liver tumors were associated with congenital heart disease [29,30,77]. In regard to FALD, the collected data from 6 studies showed that, 10 to 15 years after Fontan operation, hypervascular nodules were developed in approximately 20% to 30% of patients (Table 1) [17,68,[78][79][80][81].…”

“…Taking into consideration that 3 patients (TGA, TOF) without Fontan palliation were diagnosed with HCC in their early 20s [29,30], patients without Fontan palliation should be examined in their late teens. We therefore propose that routine examinations for cardiac hepatopathy should be started at 15 years of age in this population (Figure 3).…”

“…Because Fontan palliation has had such a major impact for patients with congenital heart disease, the development of HCC in patients undergoing Fontan palliation has begun to draw attention from both cardiologists and hepatologists. Importantly, it has also been reported that HCC can develop in patients suffering from congenital heart disease without Fontan palliation [29][30][31].…”