Cause of increased hepatic vascular stiffness in Fontan patients

Determine whether the observed increase in hepatic vascular stiffness in patients with Fontan circulation is primarily attributable to chronic venous congestion leading to elevated intrahepatic pressures or to the development of hepatic fibrosis.

Background

Elastography studies indicate that liver vasculature stiffens over time in patients living with a Fontan circuit. The Fontan physiology produces chronically elevated central venous pressure and altered hemodynamics, both of which could affect the liver. However, the mechanistic origin of the stiffness increase is uncertain: it could reflect pressure-driven congestion or structural changes due to fibrosis.

Resolving this question would clarify the pathophysiological progression of Fontan-associated liver disease (FALD) and inform whether therapeutic focus should be on relieving venous congestion or slowing fibrotic remodeling.