IMProving DRug Dosing and Outcomes for Single VEntricle Patients With Fontan Associated Liver Disease
Children's Mercy Hospital Kansas City
Summary
This is a single center, open-label, prospective, investigation to quantify the effects liver congestion and fibrosis has on hepatic drug metabolism and transport in children, adolescents, and young adults with Fontan circulation.
Eligibility
- Age range
- 8+ years
- Sex
- All
- Healthy volunteers
- No
Inclusion Criteria: * \> 8 years * Status Post Fontan Completion * Ability to provide informed permission-assent (\<18 years) or consent (≥18 years) * Fasting overnight (\~8 hours) Exclusion Criteria: * Pregnancy * Non-fasting * Non-removable metal in body or where magnetic resonance imaging (MRI) is considered unsafe * Sildenafil and/or Pravastatin therapy within last 2 months * History of underlying or laboratory evidence of underlying intestinal, metabolic, autoimmune, renal disease that can alter Sildenafil and Pravastatin disposition (absorption, metabolism, distribution, or clearance)…
Interventions
- DrugSildenafil 10mg oral tablet (participants <20kg), or 20mg oral tablet (participants ≥20kg)
A single oral dose of sildenafil will be administered to all study subjects.
- DrugPravastatin 20 mg oral tablet (ages <13 years), or 40 mg oral tablet (≥14 years)
A single oral dose of pravastatin will be administered to all study subjects.
Location
- Children's Mercy HospitalKansas City, Missouri