Liver cirrhosis (LC), which may result in hepatic failure or cancer, has been reported in patients after Fontan procedure. The purpose of this study was to clarify the frequency and histological characteristics of LC, and to evaluate the risk factors and serological markers of LC with Fontan circulation. Retrospective review of contrast-enhanced CT scans (CT) of the liver was carried out in 57 patients after Fontan procedure. Patients were divided into two groups: LC group (n = 31) and no LC group (n = 26). Age at Fontan procedure, duration after Fontan procedure, catheterization data, and history of failing Fontan circulation were compared between groups. Serological data including γ-GTP and hyaluronic acid were compared. Histology of autopsy specimens was assessed when available. Duration after Fontan procedure was significantly longer in LC group than no LC group. History of failing Fontan circulation was more frequent in LC group than in no LC group. There was no correlation between type of procedure (APC/Bjork/lateral tunnel/TCPC) and LC in this series. Serum hyaluronic acid, γ-GTP, and Forns index were significantly higher in LC group. Significant risk factors for LC were duration after Fontan procedure (>20 years). In autopsy specimens, histopathological changes of LC were observed predominantly in the central venous area. LC diagnosed with CT is frequent in patients long after Fontan procedure, especially after 20 years. Hyaluronic acid and γ-GTP could be useful markers to monitor the progression of liver fibrosis in Fontan patients.
Keywords: CT scan; Failing Fontan circulation; Fontan procedure; Liver cirrhosis; Serological marker.