Severity of hepatic fibrosis after Fontan operation is associated with early postoperative inferior vena cava pressure and time from surgery

Abstract

Hepatic fibrosis increasing over time after a Fontan procedure is well documented, but the others potential risk factors of the severity of hepatopathy are still lacking. Since 2016, we started a systematic hepatic evaluation of patients undergoing a total cavo-pulmonary connection (TCPC) for at least 10 years, including clinical features, blood sample, abdominal ultrasound, liver elastography and liver biopsy. We look for clinical, biological and hemodynamic factors potentially associated with greater degree of fibrosis at biopsy. Fourteen patients had a complete evaluation with liver biopsy. Median time from TCPC to hepatic assessment was 15.9 years (range 3.0–25.9). Seventy one percent had biological cholestasis, and only 14% had cytolysis. Abdominal ultrasound revealed hepatomegaly in 4 patients and splenomegaly in 3, but no liver nodularity. METAVIR score of liver fibrosis at biopsies showed pre-cirrhotic or cirrhotic lesions (F3–4) in 9 patients out the 14 (F1 = 3; F2 = 2; F3 = 5; F4 = 4). The degree of fibrosis was significantly correlated to inferior vena cava pressure (IVCP) obtained by cardiac catheterization 1.4 years (range 0–3.9) after the Fontan procedure ( r = 0.57, P = 0.05), and to IVCP*time from Fontan operation ( r = 0.60, P = 0.03), and tended to be correlated with intrahepatic pressure recorded during the liver biopsy, but without statistical significance ( r = 0.44, P = 0.11). There was no association between fibrosis and gender, age, age at the Fontan operation, type of systemic ventricle, functional status, liver stiffness at elastography, and blood markers of hepatic function. In this study, two-thirds of patients undergoing TCPC experienced severe liver fibrosis, correlated with early measurement of IVCP and time from Fontan after the Fontan completion. Reduction of pulmonary pressure in this population should be considered to decrease the risk of severe fibrosis or cirrhosis.