Abstract 12163: Liver Disease in Right Heart Failure: Not Just Fontan Associated

Abstract

Introduction: Hepatic fibrosis, cirrhosis, and hepatocellular carcinoma caused by the Fontan circulation, known as Fontan Associated Liver Disease (FALD), are due to low cardiac output and high venous pressure within the Fontan circuit. Hypothesis: We hypothesized that patients with congenital heart disease causing right heart dysfunction would have liver findings similar to FALD. Methods: We identified patients with the Fontan circulation or congenital heart disease associated with moderately depressed or dilated RV on echo or MRI, and/or increased right atrial pressure on cardiac cath. We defined hepatic congestion as increased kPA on Fibroscan, liver or IVC abnormalities on US, increased kPA or liver abnormalities on MRI elastography, and/or sinusoidal congestion on liver biopsy. The incidence of hepatic congestion was compared by chi squared analysis. Results: A total of 33 patients were identified for this study, 23 Fontan and 10 non-Fontan. Hepatic congestion was found in 25 patients (76%). Hepatic congestion was most often diagnosed by US in both Fontan (82.6%) and non-Fontan patients (40%). The most common findings on liver biopsy, including one non-Fontan Ebstein, were sinusoidal dilation, portal fibrosis, and perisinusoidal fibrosis. The incidence of hepatic congestion in Fontan was no different from non-Fontan however power was limited. Conclusions: Hepatic congestion was found in 83% of Fontan and 60% of non-Fontan patients. Fontan and non-Fontan patients alike are at risk for hepatic congestion and there is need for further evaluation to understand the clinical relevance of hepatic congestion caused right heart dysfunction.