Fontan-associated liver disease and hepatocellular carcinoma in adults

Abstract

The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.

Assessing liver stiffness with conventional cut‐off values overestimates liver fibrosis staging in patients who received the Fontan procedure

“…However, there was no Accumulated study have suggested the development of liver fibrosis according to the time since surgery. 38 A previous study has histologically examined 13 patients and disclosed all of cases had at least some degree of sinusoidal liver fibrosis at mean time of 16.9 years (range 6.9-25) after Fontan procedure. 6 Another histological study found that the time since Fontan procedure >18 years was associated with a higher degree of liver fibrosis as compared with ≤18 years.…”

The first reported case of Noonan syndrome complicated with hepatocellular carcinoma

“…Chronic congestion due to heart failure is one of the pathogeneses of HCC. 13 The present case was complicated by hypertrophic cardiomyopathy and mitral regurgitation. Furthermore, the patient had past history of surgery for an atrial septal defect.…”

“…It is known that increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. 13 Finally, this may have caused liver cirrhosis and the development of HCC. Chronic congestion and/or vessel abnormality may be a reason for the development of HCC in this case.…”

Platelet count and abdominal dynamic CT are useful in predicting and screening for gastroesophageal varices after Fontan surgery

“…The underlying pathophysiology of these complications may reflect the elevated central venous pressure and decreased cardiac output that follow the Fontan procedure. After surgery, the hepatic venous flow empties directly into the Fontan circuit, such that the liver is susceptible to the effects of central venous hypertension [ 16 , 17 ]. High central venous pressure also leads to chronic passive congestion subsequent to sinusoidal dilatation, with increased intrahepatic resistance and reduced portal flow [ 16 , 17 ].…”

“…After surgery, the hepatic venous flow empties directly into the Fontan circuit, such that the liver is susceptible to the effects of central venous hypertension [ 16 , 17 ]. High central venous pressure also leads to chronic passive congestion subsequent to sinusoidal dilatation, with increased intrahepatic resistance and reduced portal flow [ 16 , 17 ]. Decreased cardiac output can further contribute to the reduction of portal flow.…”