Risk of Liver Cirrhosis and Hepatocellular Carcinoma after Fontan Operation: A Need for Surveillance

Abstract

<jats:p>Liver cirrhosis and hepatocellular carcinoma (HCC) are serious late complications that can occur after the Fontan procedure. This study aimed to investigate the cumulative incidence of cirrhosis and HCC and to identify specific features distinguishing HCC from benign arterial-phase hyperenhancing (APHE) nodules that developed after the Fontan operation. We retrospectively enrolled 313 post-Fontan patients who had been followed for more than 5 years and had undergone ultrasound or computed tomography (CT) of the liver between January 2000 and August 2018. Cirrhosis was diagnosed radiologically. The estimated cumulative incidence rates of cirrhosis at 5, 10, 20, and 30 years after the Fontan operation were 1.3%, 9.2%, 56.6%, and 97.9%, respectively. Multiphasic CT revealed that 18 patients had APHE nodules that were ≥1 cm in size and showed washout in the portal venous phase (PVP)/delayed phase, which met current noninvasive HCC diagnosis criteria. Among them, only seven patients (38.9%, 7/18) were diagnosed with HCC. After cirrhosis developed, the annual incidence of HCC was 1.04%. The appearance of washout in the PVP (p = 0.006), long time elapsed since the initial Fontan operation (p = 0.04), large nodule size (p = 0.03), and elevated serum α-fetoprotein (AFP) level (p &lt; 0.001) were significantly associated with HCC. In conclusion, cirrhosis is a frequent late complication after Fontan operation, especially after 10 years, and HCC is not a rare complication after cirrhosis development. Diagnosis of HCC should not be based solely on the current imaging criteria, and washout on PVP and clinical features might be helpful to differentiate HCC nodules from benign APHE nodules.</jats:p>

Robust Effect of Hepatic Arterial Infusion Chemotherapy and Radiation Therapy on Hepatocellular Carcinoma Arising from Fontan-associated Liver Disease

“…A liver biopsy, the gold standard for diagnosing LC, is difficult to perform in patients with FALD due to hepatic congestion and prophylactic anticoagulation. Several non-invasive diagnostic tools, such as ultrasound elastography and magnetic resonance elastography, are useful for diagnosing LC (10,11). Consistent with previous reports, the imaging findings in this patient showed an LC pattern.…”

Pulmonary Arteriovenous Fistulae After Fontan Operation: Incidence, Clinical Characteristics, and Impact on All-Cause Mortality

“…In this study, we reconfirmed this phenomenon in our large cohort through visual evaluation, and liver cirrhosis was associated with bilateral diffuse-PAVF in two of our patients. Diffuse-PAVF may be one of the clinical phenotypes of Fontan-associated liver disease because of the high incidence of liver cirrhosis long after Fontan operation ( 9 ). Interestingly, diffused-PAVF disappeared with specific therapy immediately after diagnosis in three patients (T-type).…”

Laparoscopic partial liver resection for hepatocellular carcinoma arising from Fontan-associated liver disease: a case report

“…The stage of FALD depends on the duration after FP and hepatic venous pressure [ 6 ]. FALD can develop to liver cirrhosis as early as 11–15 years after FP, and the cumulative incidences of cirrhosis at 20 and 30 years after FP have been reported as 56.6% and 97.9%, respectively [ 7 ]. The incidence of HCC in FALD has been estimated as 1.5–5.0% per year [ 8 ].…”