Fontan Associated Liver Disease and the Evaluation of Biomarkers for Disease Severity Assessment

Launched by NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES (NIDDK) · Jan 27, 2022

Keywords

ClinConnect Summary

This clinical trial is focused on understanding a condition called Fontan Associated Liver Disease (FALD), which can occur in adults who have undergone a specific heart surgery known as the Fontan procedure. The study aims to figure out how long-term issues with blood flow in the liver can lead to serious liver damage and cirrhosis, a severe liver disease that can be life-threatening. Researchers hope to find new ways to identify and treat this condition.

To participate in the study, you need to be at least 18 years old and have a history of the Fontan procedure. You won’t be eligible if you have certain other liver diseases or conditions that could interfere with the study. If you join, you can expect to go through various tests, including blood tests, liver ultrasounds, and a hospital stay for further evaluations. These tests will help researchers gather important information about your liver health and the effects of FALD. It's important to note that while there is no direct benefit to you from participating, your involvement could help advance knowledge and treatment options for this condition in the future.

Gender

ALL

Eligibility criteria

• * INCLUSION CRITERIA:
• In order to be eligible to participate in this study, an individual must meet all of the following criteria:
• • 1. Male and female subjects \>= 18 years of age.
• • 2. Past surgical history of Fontan procedure.
• • 3. Prior enrollment in the Liver Diseases Branch protocol 91DK0214
• • 4. Underwent cardiac catheterization or transjugular liver biopsy within ten years prior to the date of screening
• • 5. Approved to proceed by the NIH Cardiology Consult
• • 6. 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:
• • 1. Evidence of other forms of liver disease that typically result in cirrhosis.
• • 2. 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).
• • 3. Hepatitis C as defined by the presence of hepatitis C RNA in serum.
• • 4. 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.
• • 5. 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.
• • 6. Bile duct obstruction as suggested by imaging studies done within the previous six months.
• • 7. Active substance abuse, such as alcohol, inhaled or injection drugs within the previous one year (assessed during patient interviews or by patient self-report).
• • 8. 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.
• • 9. Evidence of Cholangiocarcinoma.
• • 10. A documented or otherwise stated severe allergic reaction to contrast.
• • 11. Any other severe condition, which in the opinion of the investigators would impede the patient s participation or compliance in the study.
• • 12. Radiation exposure exceeds 5 rems during the past year.
• • 13. Inability to comply or give written informed consent as there is no direct benefit from participation in this study.
• • 14. 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.