About this trial
Characteristics and clinical course of disease In participants with cardiomyopathy associated with Friedreich Ataxia (CLARITY-FA)
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Male or female, ages ≥6 years at the time of signing the informed consent (and assent, if applicable).
Diagnosis of FA, based on clinical phenotype and genotype (GAA expansion on both alleles or compound heterozygous), with onset of FA occurring at ≤25 years of age
Confirmed left ventricular hypertrophy (LVH)
Left ventricular ejection fraction ≥30%
Disqualifiers
Presence of other form(s) of CM contributing to heart failure (HF), clinically significant cardiac anatomic abnormality or congenital cardiac malformation, clinically significant coronary artery, uncorrected, hemodynamically significant primary structural valvular disease not due to CM
Currently receiving intermittent or continuous intravenous (IV) inotrope infusion, presence of a ventricular assist device, or history of prior heart transplantation
Contraindication to cMRI, participants <12 years of age who cannot complete the cMRI without sedation will instead undergo ECHOs and are exempt from this criterion.
Prior organ transplantation
Trial population
Up to 65 participants with genetically confirmed FA-CM will be enrolled in the study with allocation by age categories and cohorts
Trial design
Cohort
Prospective
Treatments tested in this trial
Not listed
Trial groups
Trial outcomes
Primary outcomes
Characterize cardiac disease presentation and progression among participants
Change from baseline in left ventricular mass index (LVMi)
Secondary outcomes
Describe progression of maximal wall thickness (MWT) among this population
Change from baseline
Describe progression of high sensitivity troponin I among this population
Change from baseline
Describe participant perception and clinician assessment of illness in Kansas City Cardiomyopathy Questionnaire-12 (KCCQ-12)
Change from baseline
Describe clinician assessment of illness in modified Friedreich Ataxia Rating Scale (mFARS)
Change from baseline
Sponsors and contacts
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