LAUSR

Hepatic problems related to a Fontan circulation have been highlighted and elastography using ultrasound is a non-invasive tool that can measure the severity of hepatic stiffness. We investigated the hepatic stiffness using shear wave elastography (SWE) and related factors in patients with a Fontan circulation. This study enrolled 64 patients with a Fontan circulation who underwent cardiac catheterization and abdominal ultrasound from 2011 to 2015. The correlation between the laboratory tests, hemodynamic factors by cardiac catheterization, and SWE was evaluated. The patients were classified into non-cirrhotic level (≥ 2.0 m/s) and cirrhotic level (< 2.0 m/s) groups by the SWE value. The mean age was 17.6 years and the mean duration after the Fontan operation was 12.1 years. The mean value of SWE in patients (1.95 m/s) was higher than the normal (< 1.3 m/s). The SWE was higher in patients without than those with a fenestration (2.03 vs. 1.75 m/s, P = 0.003). In a multiple regression analysis between SWE and other factors, the CVP, fenestration, and lipoprotein Apo B had a significant correlation. In a multivariate analysis of cirrhotic level group, the CVP was the only significant factor. The hepatic stiffness had significantly progressed in most patients with a Fontan circulation. A low CVP and Fontan circulation with a fenestration might reduce the progression of the hepatic stiffness.