About this trial
Phase 3b/4, randomized, double-blind, parallel-group, placebo-controlled clinical trial to evaluate the efficacy, safety, and pharmacokinetics of a once daily SC injection of elamipretide in subjects with genetically confirmed BTHS for 72 weeks. The primary trial objective is to confirm the efficacy of elamipretide which is approved in the United States(FORZINITY™) under the accelerated approval based on an improvement in knee extensor muscle strength, an intermediate clinical endpoint.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Willing and able to provide signed informed consent form (ICF) prior to participation in any trial-related procedures. If applicable, informed consent in writing from parent(s) or legally-acceptable representative(s) and, informed assent from subject (if age appropriate according to local requirements) should be provided.
Agrees to adhere to the trial requirements for the length of the trial.
Must have genetically confirmed Barth Syndrome (pathogenic variant in the TAZ gene)
Male aged ≥ 5 years at time of the Screening Visit
Disqualifiers
Unable to perform the required functional tests or undergo echocardiography.
History of solid organ transplant, except successful cardiac transplantation > 12 months prior to screening, if, in the opinion of the Investigator, there is no evidence of organ rejection and post-transplant pharmacotherapy, is stable, and does not pose additional safety risk to participant.
Patients with an implantable cardioverter defibrillator (ICD) and with a known occurrence of ICD discharge in the 3 months prior to the Screening Visit.
Current placement on the waiting list for heart transplantation.
Trial design
Parallel
Treatments tested in this trial
Elamipretide
Drugsub cutaneous injection
Placebo
Drugsub cutaneous injection
Treatment groups
Trial outcomes
Primary outcomes
Primary Efficacy End Point
-change in the composite normalized score of the three functional tests: Six-minute walk test (6MWT), the triple-timed up and go test (3TUG), and the Five times sit-to-stand test (5XSST) from baseline to 72 weeks of treatment
Secondary outcomes
Secondary Efficacy End Point 1
\- Change in 6Minute Walk Test from Baseline to Week 72
Secondary Efficacy End Point 2
-Change in 3Timed Up and Go Test from Baseline to Week 72
Secondary Efficacy End Point 3
-Change in 5XSit to Stand Test from Baseline to Week 72
Secondary Efficacy End Point 4
-Change in Patient Global Impression of Severity Scale (PGI-S) score from Baseline to Week 72
Other outcomes
Safety End Point Adverse Event Reporting
-Report the # of subjects with adverse events and incidence and severity of such adverse events (AEs)
Sponsors and contacts
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