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

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.

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