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.
> 8 years
Status Post Fontan Completion
Ability to provide informed permission-assent (<18 years) or consent (≥18 years)
Fasting overnight (~8 hours)
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
Single group
Sildenafil 10mg oral tablet (participants <20kg), or 20mg oral tablet (participants ≥20kg)
DrugA single oral dose of sildenafil will be administered to all study subjects.
Pravastatin 20 mg oral tablet (ages <13 years), or 40 mg oral tablet (≥14 years)
DrugA single oral dose of pravastatin will be administered to all study subjects.
Sildenafil concentration as measured by area under the curve (AUC)
Area under the time-exposure curve (AUC 0-n) for sildenafil as determinants of the dose-exposure relationship.
Pravastatin concentration as measured by area under the curve (AUC)
Area under the time-exposure curve (AUC 0-n) for pravastatin as determinants of the dose-exposure relationship.
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Children's Mercy Hospital Kansas City
Lead sponsor
Indiana Clinical and Translational Sciences Institute
Collaborator
Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD)
Collaborator
