Development of Novel MRI Methods for Detecting, Discriminating, and Measuring Liver Fibrosis and Congestion in Fontan Patients

Study Overview
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Brief Summary
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In this research study, the investigators will conduct a prospective cross-sectional study of pediatric and adult Fontan patients that will correlate a variety of quantitative MRI biomarkers with histopathologic data.

Detailed Description
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The purpose of this study is to develop noninvasive MRI methods for detecting, discriminating, and measuring liver fibrosis and congestion in the adolescent and adult Fontan population by correlating quantitative imaging measurements with histopathologic data, using a cross-sectional approach. Over 5 years, approximately 40 pediatric and adult subjects (approximately 8 subjects per year) undergoing clinically-indicated liver biopsy for the evaluation of either 1) focal liver lesions/masses, or 2) suspected liver fibrosis/parenchymal disease will be recruited to participate in this study. Subjects will undergo research MR imaging within ±2 weeks of the liver biopsy procedure. A variety of MRI biomarkers will be correlated with histopathologic data obtained from the clinically indicated liver biopsy.

Official Title
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Development of Novel MRI Methods for Detecting, Discriminating, and Measuring Liver Fibrosis and Congestion in Fontan Patients

Conditions
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Liver Fibrosis

Intervention / Treatment
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* Device: Non-Contrast Magnetic Resonance Imaging


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Single ventricle physiology congenital heart disease status post Fontan palliation Undergoing clinically-indicated liver biopsy Ability to tolerate 60 minutes in an MRI scanner without sedation Exclusion Criteria: Known contraindication to MR imaging (e.g., implanted non-MRI compatible device) Known or suspected pregnancy. Female subjects of child bearing potential will undergo urine pregnancy testing prior to imaging

Ages Eligible for Study
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Minimum Age: 8 Years
Maximum Age: 99 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Fontan patients<br>Pediatric and adult Fontan patients will undergo MRI (Magnetic Resonance Imaging) of the liver using novel, non-contrast MRI methods. These MR methods will be used to detect, discriminate and measure liver fibrosis and congestion. The resulting quantitative imaging measurements will be correlated with histopathologic data obtained from a clinically-indicated liver biopsy. | Device: Non-Contrast Magnetic Resonance Imaging<br>* The investigators plan to prospectively assess the correlation of liver fibrosis and congestion measurements obtained from novel, non-contrast MR imaging methods to histopathologic data obtained from a clinically-indicated liver biopsy in Fontan patients.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| MR elastography data correlation to histologic data | MR elastography of the liver data will be correlated with histopathologic data obtained from non-lesional liver tissue. Histologic fibrosis and congestion will be scored by a pathologist using semi-quantitative grading systems (e.g., METAVIR scoring system for fibrosis | 60 minutes | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| MR T1 mapping data (corrected and uncorrected) correlation to histologic data | MR T1 mapping liver data (corrected and uncorrected) will be correlated with histopathologic data obtained from non-lesional liver tissue. Histologic fibrosis and congestion will be scored by a pathologist using semi-quantitative grading systems (e.g., METAVIR scoring system for fibrosis) | 60 minutes | 
| MR T1rho data correlation to histologic data | MR T1rho liver data will be correlated with histopathologic data obtained from non-lesional liver tissue. Histologic fibrosis and congestion will be scored by a pathologist using semi-quantitative grading systems (e.g., METAVIR scoring system for fibrosis) | 60 minutes | 
| MR T2 mapping data correlation to histologic data | MR T2 mapping liver data will be correlated with histopathologic data obtained from non-lesional liver tissue. Histologic fibrosis and congestion will be scored by a pathologist using semi-quantitative grading systems (e.g., METAVIR scoring system for fibrosis) | 60 minutes | 
| MR Diffusion weighted data correlation to histologic data | MR Diffusion weighted liver data will be correlated with histopathologic data obtained from non-lesional liver tissue. Histologic fibrosis and congestion will be scored by a pathologist using semi-quantitative grading systems (e.g., METAVIR scoring system for fibrosis) | 60 minutes | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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MRI, Liver fibrosis, Fontan surgery, Novel Methods