Hepatic Perfusion and Liver Health as Assessed by Dual Cholate Clearance Assay in Fontan-associated Liver Disease (FALD)
The goal of this study is to use the HepQuant dual cholate clearance assay, which has been shown to measure liver function in people with known chronic liver conditions, to assess severity of Fontan-associated liver disease (FALD). This study aims to understand the role impaired blood flow to the liver plays in liver function in Fontan patients compared to patients with right heart failure and healthy controls. The study will also determine whether cholate clearance is a good measure to use in this population, and whether it will be able to predict clinical outcomes. Participants will undergo a HepQuant dual cholate clearance assay and a cardiac magnetic resonance imaging (MRI) at the beginning of the study, and then data on health status will be collected for 5 years.
• Fontan: Adult Fontan patients ≥ 18 years of age who have undergone right heart catheterization within the past 1 year.
• RHF Controls: non-Fontan adults with two ventricle anatomy with systemic left ventricle, CVP estimate ≥ 8 mmHg and left ventricle (LV) function ≥ 50% by echocardiogram performed within the past 1 year. Can include those with congenital heart disease- repaired or unrepaired.
• Normal Controls: non-Fontan adults with normal cardiac anatomy, normal biventricular function, \< moderate tricuspid regurgitation and CVP estimate \< 5 mmHg by echocardiogram performed within the past 1 year.