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Rationale and design of a randomised trial of trientine in patients with hypertrophic cardiomyopathy
John Farrant1,2, Susanna Dodd3,4, Carly Vaughan3
1BHF Manchester Centre for Heart and Lung Magnetic Resonance Research, Manchester University NHS Foundation Trust, Manchester, UK.
Insights
Trientine, a copper chelator, is being studied for its potential to treat hypertrophic cardiomyopathy (HCM). The TEMPEST trial investigates if trientine can reduce left ventricular mass and improve cardiac function in HCM patients.
Area of Science:
- Cardiology
- Pharmacology
- Biochemistry
Background:
- Hypertrophic cardiomyopathy (HCM) involves left ventricular hypertrophy (LVH), fibrosis, oxidative stress, and energy depletion.
- Copper ions catalyze oxidative stress and inhibit antioxidants; trientine selectively chelates copper.
- Preclinical and open-label studies suggest trientine may reduce LVH, fibrosis, and improve mitochondrial function in diabetes and HCM.
Purpose of the Study:
- To evaluate the efficacy and mechanism of action of trientine in patients with hypertrophic cardiomyopathy (HCM).
Main Methods:
- The TEMPEST trial is a multicenter, double-blind, randomized, placebo-controlled phase II study.
- Patients with HCM (NYHA classes I-III) receive trientine or placebo for 52 weeks.
- Primary outcome: change in left ventricular (LV) mass via cardiovascular magnetic resonance; secondary outcomes assess exercise capacity, arrhythmias, and cardiac function.
Main Results:
- This section is not yet available as the study is ongoing.
Conclusions:
- The TEMPEST trial aims to determine the efficacy and mechanism of trientine in managing HCM.
Aims:
Hypertrophic cardiomyopathy (HCM) is characterised by left ventricular hypertrophy (LVH), myocardial fibrosis, enhanced oxidative stress and energy depletion. Unbound/loosely bound tissue copper II ions are powerful catalysts of oxidative stress and inhibitors of antioxidants. Trientine is a highly selective copper II chelator. In preclinical and clinical studies in diabetes, trientine is associated with reduced LVH and fibrosis, and improved mitochondrial function and energy metabolism. Trientine was associated with improvements in cardiac structure and function in an open-label study in patients with HCM.
Methods:
The Efficacy and Mechanism of Trientine in Patients with Hypertrophic Cardiomyopathy (TEMPEST) trial is a multicentre, double-blind, parallel group, 1:1 randomised, placebo-controlled phase II trial designed to evaluate the efficacy and mechanism of action of trientine in patients with HCM. Patients with a diagnosis of HCM according to the European Society of Cardiology Guidelines and in New York Heart Association classes I-III are randomised to trientine or matching placebo for 52 weeks. Primary outcome is change in left ventricular (LV) mass indexed to body surface area, measured using cardiovascular magnetic resonance. Secondary efficacy objectives will determine whether trientine improves exercise capacity, reduces arrhythmia burden, reduces cardiomyocyte injury, improves LV and atrial function, and reduces LV outflow tract gradient. Mechanistic objectives will determine whether the effects are mediated by cellular or extracellular mass regression and improved myocardial energetics.
Conclusion:
TEMPEST will determine the efficacy and mechanism of action of trientine in patients with HCM.
Trial Registration Numbers:
NCT04706429 and ISRCTN57145331.
