Hepatic Perfusion and Liver Health as Assessed by Dual Cholate Clearance Assay in Fontan-associated Liver Disease (FALD)
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 75
- 试验地点
- 2
- 主要终点
- Hepatic cholate clearance
研究概览
简要总结
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •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.
排除标准
- •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
研究组 & 干预措施
Normal Controls
Patients with normal cardiac anatomy and normal CVP (right-sided filling pressure) by echocardiogram.
干预措施: Dual cholate clearance assay (Device)
Normal Controls
Patients with normal cardiac anatomy and normal CVP (right-sided filling pressure) by echocardiogram.
干预措施: MRI (Radiation)
Fontan
Patients with a Fontan diagnosis who have undergone a right heart catheterization in the past year
干预措施: Dual cholate clearance assay (Device)
Fontan
Patients with a Fontan diagnosis who have undergone a right heart catheterization in the past year
干预措施: MRI (Radiation)
Right Heart Failure (RHF) Controls
Non-Fontan patients with RHF (patients with normal 2-ventricle anatomy who have intermediate or elevated right-sided filling pressures (CVP) by echocardiogram)
干预措施: Dual cholate clearance assay (Device)
Right Heart Failure (RHF) Controls
Non-Fontan patients with RHF (patients with normal 2-ventricle anatomy who have intermediate or elevated right-sided filling pressures (CVP) by echocardiogram)
干预措施: MRI (Radiation)
结局指标
主要结局
Hepatic cholate clearance
时间窗: Baseline
13C-cholate and d4-cholate clearance as assessed by the dual cholate clearance test
次要结局
- Occurrence of at Least One Heart or Liver-Related Clinical Outcome(5 years after first cholate assay)