Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes

Possner, Mathias; Gordon‐Walker, Timothy T.; Egbe, Alexander C.; Poterucha, Joseph T.; Warnes, Carole A.; Connolly, Heidi M.; Ginde, Salil; Clift, Paul; Kogon, Brian; Book, Wendy; Walker, Niki L.; Wagenaar, Lodewijk J.; Moe, Tabitha G.; Oechslin, Erwin; Kay, W. Aaron; Norris, Mark D.; Dillman, Jonathan R.; Trout, Andrew T.; Anwar, Nadeem; Hoskoppal, Arvind; Bröering, Dieter C.; Bzeizi, Khalid; Veldtman, Gruschen

doi:10.1016/j.ijcard.2020.08.057

Cited by 49 publications

(43 citation statements)

References 38 publications

(40 reference statements)

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“…Serum α-fetoprotein (AFP) is a valuable complementary tool for the diagnosis of HCC because this biomarker is above the upper limit of normal in nearly 80% of Fontan patients diagnosed with HCC. 78 In contrast, in a large prospective series with a high prevalence of nonneoplastic nodules, no patient with Fontan surgery without HCC showed elevated AFP. 40 Accordingly, elevated AFP levels in patients with FALD should be always suspicious of HCC.…”

Section: Hepatic Nodules and Hepatocellular Carcinomamentioning

confidence: 88%

“…The American Heart Association has recently recommended HCC screening using abdominal ultrasound and AFP determination every 6 months after the diagnosis of cardiac cirrhosis. 91 However, 30 to 50% of cases of HCC in FALD have been diagnosed in noncirrhotic patients 76,78 and this general recommendation cannot thus be applied to the Fontan population. The optimal intervals and imaging modality remain unclear; however, it is reasonable to offer HCC surveillance to those patients who underwent surgery more than 10 years earlier.…”

Section: Screening and Diagnostic Programsmentioning

confidence: 99%

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Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

2021

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Fontan-associated liver disease is the term used to encompass the disorders arising from abnormal hemodynamic alterations and systemic venous congestion after the Fontan procedure. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease. While the natural history of this form of liver disease is poorly established, many Fontan patients ultimately develop portal hypertension-related complications such as ascites, esophageal varices, malnutrition, and encephalopathy. Fontan survivors also show an elevated risk of hepatocellular carcinoma. Adequate staging of the liver damage is essential to anticipate screening strategies and improve global management.

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Section: Hepatic Nodules and Hepatocellular Carcinomamentioning

confidence: 88%

Section: Screening and Diagnostic Programsmentioning

confidence: 99%

Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management

2021

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“…The incidence of HCC was of 0.8%, 2.9%, and 13.3% at 10, 20, and 30 years after the surgical procedure (82). HCC seemed to occur late after the Fontan surgery (after 10 years) with a mean age around 30 years and, due to the absence of screening strategy, patients are often diagnosed at an advanced stage (82)(83)(84)(85). In a recent study prospectively conducted on 152 Fontan patients, liver nodules were frequent.…”

Section: Surveillance and Hepatocellular Carcinoma Occurrencementioning

confidence: 99%

“…The best approach of HCC has not been defined yet and the combined liver-heart transplantation still has to be discussed. Currently, the prognosis of HCC related to Fontan procedure is poor (1year survival around 50%) and the management and surveillance of the patients has to be improved (85,94). Recently, the American Heart Association published new guidelines for the surveillance of the patients and a bi annual screening for HCC should be proposed once the cardiac cirrhosis is established (21) (Table 2).…”

Section: Surveillance and Hepatocellular Carcinoma Occurrencementioning

confidence: 99%

From congestive hepatopathy to hepatocellular carcinoma, how can we improve patient management?

et al. 2021

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Summary Heart failure and liver disease often coexist because of systemic disorders and diseases that affect both organs as well as complex cardio-hepatic interactions. Heart failure can cause acute or chronic liver injury due to ischaemia and passive venous congestion, respectively. Congestive hepatopathy is frequently observed in patients with congenital heart disease and after the Fontan procedure, but also in older patients with chronic heart failure. As congestive hepatopathy can evolve into cirrhosis and hepatocellular carcinoma, screening for liver injury should be performed in patients with chronic cardiac diseases and after Fontan surgery. Fibrosis starts in the centro-lobular zone and will extend progressively to the portal area. Chronic liver injury can be reversible if heart function improves. However, in the case of terminal heart failure, uncontrolled by medical resources or by assistive device support, the combination of heart and liver transplants must be discussed in patients with chronic advanced liver fibrosis. In this review of the literature, we will focus on congestive hepatopathy and its complications, such as liver fibrosis and hepatocellular carcinoma, with the aim of improving the management and surveillance of patients experiencing these complications.

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“…However, the magnitude of fibrosis increases with advancing age, 3 and adults with a Fontan circulation are more likely to have cirrhosis than are children. Alarming as well is the increased risk of hepatocellular carcinoma in young adults, 4 potentiated by a progressively fibrotic liver that is under sustained, continuous physiological duress. Liver health is at risk even in the most well-functioning Fontan circulation.…”

mentioning

confidence: 99%