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HepQuant is an assay to measure liver function in other forms of liver disease such as hepatitis C. It measures the ability of the liver to process an naturally occurring substance called cholate and has been shown to predict clinical outcomes in some patient populations.
The Fontan survivor is a patient who has undergone surgery or a series of surgeries that result in a functional single ventricle. The liver is particularly vulnerable to disease including congestion, fibrosis, and even cirrhosis. Currently liver function in the Fontan is not well-characterized and the HepQuant assay could help answer this question.
A novel test called HepQuant® is an assay that has been used to measure liver function in other types of liver disease. In these populations, it is hoped that the assay may show more subtle or sub-clinical liver abnormalities that blood tests alone cannot detect. HepQuant® is a test that examines the ability of the liver to process a substance called cholate, which is a naturally occurring substance in the body. Cholate gets to the liver 2 ways: through the gut (by mouth) or through the blood (IV or intravenous). The test involves administering oral and IV cholate which is labeled. This label is NOT radioactive, but can act as an indicator/marker for evaluation purposes.
The purpose of this study is to develop a reliable means of measuring liver function in the Fontan survivor by examining cholate clearance. The investigators aim to 1) explore any association between level of cholate clearance and measurement of heart and liver function and 2) determine whether cholate clearance can predict clinical outcomes such as heart failure, ascites (development of fluid in the abdomen which can result from heart failure or liver failure), and need for heart transplant.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Fontan patient | Cholate assay will be administered once to Fontan patients and blood specimens will be collected to analyze cholate clearance. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Cholate assay | Device | Labeled oral and IV cholate will be administered once to the Fontan cohort and blood specimens will be collected at baseline and timepoints thereafter. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Hepatic cholate clearance | D4-cholate and 13C-cholate clearance | Through study completion, an average of 1 year |
| Measure | Description | Time Frame |
|---|---|---|
| Incidence of adverse clinical outcomes | combined outcome of occurrence of any of the following: heart failure admission, clinically significant ascites, protein losing enteropathy, referral for cardiac transplantation, death | 5 years after cholate assay |
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Inclusion Criteria:
Exclusion Criteria:
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Adult Fontan survivors at least 18 years of age
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| Name | Affiliation | Role |
|---|---|---|
| Yuli Kim, MD | University of Pennsylvania | Principal Investigator |
| Maarouf Hoteit, MD | Hospital of the University of Pennslyvania | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital of the University of Pennsylvania | Philadelphia | Pennsylvania | 19104 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40118791 | Derived | Kim YY, Nyman A, Huang YS, Tomlinson AZ, McRae MP, Everson GT, Vaikunth S, Rosenthal B, Rychik J, Hoteit MA. Alterations in Liver Perfusion in Adults With Fontan Circulation as Assessed by Dual Cholate Clearance. J Am Heart Assoc. 2025 Apr;14(7):e039479. doi: 10.1161/JAHA.124.039479. Epub 2025 Mar 21. |
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IPD will not be shared with other researchers
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| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| ID | Term |
|---|---|
| D004066 | Digestive System Diseases |
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