Actively Recruiting

Phase 1
Age: 8Years +
All Genders
ID06324396

IMProving DRug Dosing and Outcomes for Single VEntricle Patients With Fontan Associated Liver Disease

Led by Children's Mercy Hospital Kansas City · Updated on 2026-01-02

15

Participants Needed

1

Research Sites

N/A

Total Duration

On this page

Sponsors

C

Children's Mercy Hospital Kansas City

Lead Sponsor

I

Indiana Clinical and Translational Sciences Institute

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are studying how liver congestion and fibrosis affect the way children, adolescents, and young adults with Fontan circulation process certain drugs. This investigation focuses on measuring the impact of these liver conditions on the metabolism and transport of medications in this specific group. The study is a Phase 1, open-label, single-center trial designed to gather important information on drug dosing for patients with Fontan-associated liver disease. Participants in the study will receive a single oral dose of two drugs: sildenafil and pravastatin. The dose of sildenafil varies by weight, with 10 mg given to those under 20 kg and 20 mg for those 20 kg or above. Pravastatin dosing is age-based, with 20 mg for children under 13 years and 40 mg for those 14 years or older. This dosing occurs during the assessment phase to evaluate drug concentrations and how the liver condition influences drug processing. Participants will be involved in fasting overnight before receiving the medications. Researchers will measure the concentration of sildenafil and pravastatin over a two-year period, focusing on the area under the curve (AUC) to understand drug metabolism. The study includes assessments to monitor liver function and ensure participant safety, with the total involvement spanning up to two years to capture the long-term effects on drug disposition in this population.

CONDITIONS

Brief Title

IMProving DRug Dosing and Outcomes for Single VEntricle Patients With Fontan Associated Liver Disease

Who Can Participate

Age: 8Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Older than 8 years
  • Completed Fontan procedure
  • Able to provide informed permission-assent if under 18, or consent if 18 or older
  • Fasting overnight for about 8 hours before study participation
Not Eligible

You will not qualify if you...

  • Pregnancy
  • Not fasting before the study
  • Having non-removable metal in the body or any condition making MRI unsafe
  • Use of sildenafil or pravastatin therapy within the past 2 months
  • History or laboratory evidence of intestinal, metabolic, autoimmune, or renal disease that may affect drug processing
  • Taking medications that interact with sildenafil or pravastatin
  • Unable to swallow tablets
  • Liver enzyme levels (AST, ALT) or bilirubin above five times the normal age-specific limits
  • Diarrhea within the last 24 hours
  • History of solid organ transplantation

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Treatment

Duration - Single day

Participants receive a single oral dose of sildenafil and pravastatin.

1 treatment visit (in-person)

Follow-up

Duration - 2 years

Participants are monitored for drug concentration and outcomes over 2 years after dosing.

Multiple visits over 2 years

Trial Site Locations

Total: 1 location

1

Children's Mercy Hospital

Kansas City, Missouri, United States, 64108

Actively Recruiting

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Research Team

J

Jonthan Wagner, DO

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NA

Model

SINGLE_GROUP

Primary Purpose

TREATMENT

Number of Arms

1

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Frequently Asked Questions

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