NCT06324396PHASE1Recruiting
IMProving DRug Dosing and Outcomes for Single VEntricle Patients With Fontan Associated Liver Disease
Children's Mercy Hospital Kansas City
Start Date
3/1/2024
Completion Date
12/31/2026
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 Criteria
Age Range: 8 years to No maximum
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)
* Pharmacotherapy that interacts with Sildenafil (cytochrome P450 3A4 and P450 3A5 (CYP3A4/5) inducers/inhibitors) and/or Pravastatin (organic anion transporting polypeptide (OATP1B1) inducers/inhibitors)
* Inability to swallow a tablet
* \>5X the age-specific upper limit of normal for aspartate aminotransferase (AST) and alanine aminotransferase (ALT), total and conjugated bilirubin
* Diarrhea in the last 24 hours \*History of solid organ transplantation
Interventions
DRUG
Sildenafil 10mg oral tablet (participants <20kg), or 20mg oral tablet (participants ≥20kg)
DRUG
Pravastatin 20 mg oral tablet (ages <13 years), or 40 mg oral tablet (≥14 years)
Conditions
Fontan Circulation
Locations
Children's Mercy Hospital
Kansas City, Missouri 64108
United States