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

Phase 1RecruitingNCT06324396

Children's Mercy Hospital Kansas City15 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Enrollment

Conditions

Fontan Circulation

Interventions

Sildenafil 10mg oral tablet (participants <20kg), or 20mg oral tablet (participants ≥20kg)DRUG

A 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)DRUG

A single oral dose of pravastatin will be administered to all study subjects.

Eligibility

Sex: ALLMin age: 8 Years

Medical Language ↔ Plain English

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

Locations (1)

Children's Mercy Hospital

Kansas City, Missouri, United States

RECRUITING

Outcomes

Primary Outcomes

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.

Time frame: 2 years

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.

Time frame: 2 years

Central Contacts

Jonthan Wagner, DO

CONTACT

816-731-7240 jbwagner@cmh.edu

Data from ClinicalTrials.gov

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