Evaluation of Fontan-associated Hepatic Stiffness and the Efficacy of Udenafil in Reducing Liver Stiffness
FALD
Imaging and Biomarker Evaluation of Hepatic Stiffness in Children Enrolled in the Fontan Udenafil Exercise Longitudinal Study
2 other identifiers
interventional
88
2 countries
17
Brief Summary
A study to evaluate the efficacy of MZ101 therapy in reducing liver stiffness.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at below P25 for phase_3
Started Feb 2018
Typical duration for phase_3
17 active sites
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
First Submitted
Initial submission to the registry
January 22, 2018
CompletedFirst Posted
Study publicly available on registry
February 13, 2018
CompletedStudy Start
First participant enrolled
February 20, 2018
CompletedPrimary Completion
Last participant's last visit for primary outcome
September 15, 2020
CompletedStudy Completion
Last participant's last visit for all outcomes
September 16, 2020
CompletedResults Posted
Study results publicly available
September 18, 2025
CompletedOctober 14, 2025
September 1, 2025
2.6 years
January 22, 2018
August 24, 2025
September 24, 2025
Conditions
Keywords
Outcome Measures
Primary Outcomes (2)
Effect of Udenafil 87.5 mg BID Treatment on Liver Stiffness in Udenafil Continuation and Naive Groups After 52 Weeks, as Determined by Ultrasound Shear Wave Elastography
Liver stiffness was measured at baseline and 52 weeks using Ultrasound Shear Wave Elastography (USWE) separately in Udenafil Continuation (U+) and Udenafil Naive (U-) groups. A decrease in value indicates a decrease in liver stiffness.
52 weeks
Effect of Udenafil 87.5 mg BID Treatment on Liver Stiffness in Udenafil Continuation and Naive Groups After 52 Weeks, as Determined by Magnetic Resonance Elastography
Liver stiffness was measured at baseline and 52 weeks using Magnetic Resonance Elastography (MRE) separately in Udenafil Continuation (U+) and Udenafil Naive (U-) groups. A decrease in value indicates a decrease in liver stiffness.
52 weeks
Secondary Outcomes (2)
Effect of Udenafil 87.5 mg BID on Enhanced Liver Fibrosis (ELF) Score After 52 Weeks of Treatment
52 weeks
Effect of Udenafil 87.5 mg BID on Brain Natriuretic Peptide After 52 Weeks of Treatment
52 weeks
Study Arms (2)
Udenafil Continuation (U+)
EXPERIMENTALSubjects who were receiving Udenafil 87.5 mg BID in FUEL study prior to enrolling to this study (FALD).
Udenafil Naive (U-)
EXPERIMENTALSubjects who were receiving Placebo BID in FUEL study prior to enrolling to this study (FALD).
Interventions
Open-label extension with Udenafil 87.5 mg BID treatment for 52 weeks.
Eligibility Criteria
You may qualify if:
- Enrollment in on-going Phase 3 Open-Label Safety Study
- Informed assent from subject, informed consent from parent/legal guardian as appropriate
You may not qualify if:
- Non-enrollment in the on-going Phase 3 Open-Label Study
- Subjects with contra-indications for MRI (these subjects will be excluded from the MRI component of this study)
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (17)
Children's Hospital Colorado
Aurora, Colorado, 80045, United States
Nemours Cardiac Center/Alfred I. duPont Hospital for Children
Wilmington, Delaware, 19803, United States
Children's National Medical Center
Washington D.C., District of Columbia, 20008, United States
Children's Healthcare of Atlanta
Atlanta, Georgia, 30322, United States
Children's Mercy Hospital
Kansas City, Kansas, 64108, United States
Boston Children's Hospital
Boston, Massachusetts, 02115, United States
University of Michigan Congenital Heart Center
Ann Arbor, Michigan, 48109-4204, United States
Children's Mercy Hospital Kansas City
Kansas City, Missouri, 64108, United States
University of Nebraska Children's Hospital and Medical Center
Omaha, Nebraska, 68114, United States
Cincinnati Children's Hospital Medical Center
Cincinnati, Ohio, 45229, United States
Children's Hospital of Philadelphia
Philadelphia, Pennsylvania, 19104, United States
Medical University of South Carolina
Charleston, South Carolina, 29425, United States
Vanderbilt University Medical Center
Nashville, Tennessee, 37232, United States
Texas Children's Hospital
Houston, Texas, 77030, United States
Primary Children's Medical Hospital/Dept. of Pediatric Cardiology
Salt Lake City, Utah, 84113, United States
Children's Hospital of Wisconsin
Milwaukee, Wisconsin, 53226, United States
The Hospital for Sick Children
Toronto, Ontario, ON M5G 1X8, Canada
MeSH Terms
Interventions
Results Point of Contact
- Title
- Dr. Scott Oglesby
- Organization
- Mezzion Pharmaceuticals
Study Officials
- PRINCIPAL INVESTIGATOR
David J Goldberg, MD
Children's Hospital of Philadelphia
- PRINCIPAL INVESTIGATOR
Kurt R Schumacher, MD
University of Michigan Congenital Heart Center/C.S. Mott Children's Hospital
Publication Agreements
- PI is Sponsor Employee
- No
- Restrictive Agreement
- No
Study Design
- Study Type
- interventional
- Phase
- phase 3
- Allocation
- NON RANDOMIZED
- Masking
- NONE
- Purpose
- TREATMENT
- Intervention Model
- SINGLE GROUP
- Sponsor Type
- INDUSTRY
- Responsible Party
- SPONSOR
Study Record Dates
First Submitted
January 22, 2018
First Posted
February 13, 2018
Study Start
February 20, 2018
Primary Completion
September 15, 2020
Study Completion
September 16, 2020
Last Updated
October 14, 2025
Results First Posted
September 18, 2025
Record last verified: 2025-09
Data Sharing
- IPD Sharing
- Will not share