Cholate Clearance in Fontan and Heart Failure

HepQuant

Status: Enrolling

Conditions: Right Heart Failure; Fontan

Treatments: Radiation: MRI; Device: Dual cholate clearance assay

Study type: Observational

Funder types: Other; Industry

Identifiers

NCT07009132

Fontan Cholate 2.0

Details and patient eligibility

About

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.

Enrollment: 75 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: Accepts Healthy Volunteers

Inclusion criteria

• 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.

Exclusion criteria

• Pregnant or breastfeeding
• Unable to comprehend and/or give informed consent
• Sensitivity to human serum albumin, or its preparations
• Participants with extensive resection of large segments of the small intestine (short gut) or severe gastroparesis
• Participants on non-selective beta blockers, angiotensin converting-enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), ursodeoxycholic acid, cholic acid, or other bile acids who are unwilling or unable to delay taking their normal dose the morning of their dual cholate clearance testing.
• Participants on dietary supplements, bile acid sequestrants, GLP-1 agonists, narcotics, and other medications affecting gastric emptying or intestinal absorption who are unwilling or unable to delay taking or withhold as outlined in the instructions on deviating from the SHUNT test.
• Contraindication to cardiac MRI
• For RHF and normal controls: diabetes, chronic liver disease, >moderate alcohol use, BMI >30, oxygen-dependent, pulmonary hypertension22 on therapy, known porto-pulmonary hypertension

Trial design

75 participants in 3 patient groups

Fontan

Description:

Patients with a Fontan diagnosis who have undergone a right heart catheterization in the past year

Treatment:

Device: Dual cholate clearance assay

Radiation: MRI

Right Heart Failure (RHF) Controls

Description:

Non-Fontan patients with RHF (patients with normal 2-ventricle anatomy who have intermediate or elevated right-sided filling pressures (CVP) by echocardiogram)

Treatment:

Device: Dual cholate clearance assay

Radiation: MRI

Normal Controls

Description:

Patients with normal cardiac anatomy and normal CVP (right-sided filling pressure) by echocardiogram.

Treatment:

Device: Dual cholate clearance assay

Radiation: MRI