Status:
RECRUITING
Fontan Associated Liver Disease and the Evaluation of Biomarkers for Disease Severity Assessment
Lead Sponsor:
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Conditions:
Congenital Heart Disease
Eligibility:
All Genders
18-100 years
Brief Summary
Background: In Fontan Associated Liver Disease (FALD), congestion of blood in the liver causes cirrhosis. This condition can cause death. Researchers want to understand what triggers this process and...
Detailed Description
Study Description: Up to 100 subjects who completed the Fontan procedure for severe Congenital Heart Disease (CHD) and are at risk for congestive hepatopathy or Fontan Associated Liver Disease (FALD)...
Eligibility Criteria
Inclusion
- INCLUSION CRITERIA:
- In order to be eligible to participate in this study, an individual must meet all of the following criteria:
- Male and female subjects \>= 18 years of age.
- Past surgical history of Fontan procedure.
- Prior enrollment in the Liver Diseases Branch protocol 91DK0214
- Underwent cardiac catheterization or transjugular liver biopsy within ten years prior to the date of screening
- Approved to proceed by the NIH Cardiology Consult
- Approved to proceed by the NIH Cardiac Pre-anesthesia Consult
- EXCLUSION CRITERIA:
- An individual who meets any of the following criteria will be excluded from participation in this study:
- Evidence of other forms of liver disease that typically result in cirrhosis.
- Evidence of active Chronic Hepatitis B infection as defined by the presence of hepatitis B surface antigen (HBsAg) in serum and elevated HBV DNA (\>10,000 IU/mL).
- Hepatitis C as defined by the presence of hepatitis C RNA in serum.
- Evidence of other liver disease such as primary sclerosing cholangitis, primary biliary cirrhosis, Wilson s disease, autoimmune hepatitis as defined by either liver histology or laboratory abnormalities.
- Hemochromatosis as defined by presence of 3+ or 4+ stainable iron on liver biopsy or homozygosity for C282Y. Patients with iron saturation indices of \>45% and serum ferritin levels of \>300 ng/ml for men and \>250 ng/ml for women will undergo genetic testing for hemochromatosis.
- Bile duct obstruction as suggested by imaging studies done within the previous six months.
- Active substance abuse, such as alcohol, inhaled or injection drugs within the previous one year (assessed during patient interviews or by patient self-report).
- Evidence of hepatocellular carcinoma; either alpha-fetoprotein (AFP) levels greater than 50 ng/ml (normal \<6.6 ng/ml) and/or ultrasound (or other imaging study) demonstrating a mass suggestive of liver cancer.
- Evidence of Cholangiocarcinoma.
- A documented or otherwise stated severe allergic reaction to contrast.
- Any other severe condition, which in the opinion of the investigators would impede the patient s participation or compliance in the study.
- Radiation exposure exceeds 5 rems during the past year.
- Inability to comply or give written informed consent as there is no direct benefit from participation in this study.
- Female subjects who are currently pregnant will be excluded due to radiation exposure necessary for study completion. In addition, altered hemodynamics may confound the study s results. Following pregnancy, patients may be reconsidered for the study.
Exclusion
Key Trial Info
Start Date :
September 7 2022
Trial Type :
OBSERVATIONAL
Allocation :
ESTIMATED
End Date :
June 30 2051
Estimated Enrollment :
100 Patients enrolled
Trial Details
Trial ID
NCT05213598
Start Date
September 7 2022
End Date
June 30 2051
Last Update
October 23 2025
Active Locations (1)
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1
National Institutes of Health Clinical Center
Bethesda, Maryland, United States, 20892