Hepatopathy associated with the total cavo-pulmonary connection (Fontan intervention)

Total cavo-pulmonary connection or Fontan procedure has revolutionized the prognosis of patients with single-ventricle congenital heart disease. Nevertheless, the increase of the central venous pressure (CVP) that it induces seems to lead to hepatic fibrosis or even cirrhosis [1] . Several cases of hepatocarcinoma (HCC) have been described. There is so far no way to assess the fibrosis with non-invasive method [2] . The prevalence and contributing factors of this life-threatening complication are not well defined. The objective of our study was to evaluate the prevalence and associated factors with cirrhosis in a cohort of patients undergoing Fontan surgery. Between October 2016 and March 2019, we prospectively evaluated the clinical, biological, radiological and histological hepatologic characteristics of the patients followed at the University Hospital of Tours, and collected the haemodynamic data evaluated in the early postoperative period. Different parameters were compared between groups with and without cirrhosis (METAVIR F4). Thirty-one patients were included. The DCPT was performed on average at 9 years old and the hepatological assessment 15 years later. All patients had sinusoidal dilatation and fibrosis and cirrhosis was present in 10 patients (38%). A 25-year-old woman was diagnosed with multifocal HCC. No significant difference was found between the two groups regarding functional ventricle type, time since surgery, age at surgery, early CVP or CVP measured at the time of biopsy. In conclusion, one-third of patients had histologically proven cirrhosis and two-thirds had extensive fibrosis. There was no evidence of any factor associated with more severe fibrotic changes. Large cohorts are needed for the study of this rare but decisive disease in the prognosis of patients.