Hepatic Perfusion and Liver Health as Assessed by Dual Cholate Clearance Assay in Fontan-associated Liver Disease (FALD)

Summary

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.

Eligibility

Age range

18+ years

Sex

All

Healthy volunteers

Yes

Interventions

• Device
  Dual cholate clearance assay

  Study participants are administered labeled oral and intravenous cholate, and then two blood samples are taken over 60-minutes. Serum cholate levels are measured by blood draws.

• Radiation
  MRI

  Unsedated non contrast cardiac magnetic resonance imaging (MRI)

Location