Tempest Trial
A randomised, double-blind, placebo-controlled, phase 2 evaluation of the efficacy and mechanism of trientine in patients with hypertrophic cardiomyopathy
Lay Summary of Results
Hypertrophic cardiomyopathy is the most common inherited heart condition. It causes thickening and scarring of the heart muscle, and reduces the heart's ability to produce energy efficiently.
This study investigated whether trientine, a drug that affects how the body handles copper, could help people with hypertrophic cardiomyopathy. Copper is an essential mineral that plays an important role in energy production within the heart and helps protect heart cells from damage and scarring. Low copper levels inside heart cells can impair energy production and contribute to thickening of the heart muscle. Excess copper outside heart cells may promote the formation of scar tissue. Trientine is thought to improve these processes by restoring a healthier balance of copper in the heart.
Over the course of a year, people with hypertrophic cardiomyopathy who received trientine experienced a greater reduction in heart muscle thickness than those who received a placebo. Trientine was generally well tolerated, with few side effects.
Overall, the findings suggest that trientine may help reverse some of the abnormal heart muscle growth seen in hypertrophic cardiomyopathy. While the results are encouraging, the study was not designed to determine whether the drug improves symptoms, daily functioning, or long-term clinical outcomes. Further research is needed to see whether these improvements translate into meaningful benefits for patients.
Background
Hypertrophic cardiomyopathy (HCM) is the most common inherited heart condition. Current treatments only aim to alleviate symptoms. There are no treatments that slow or reverse the underlying damage to the heart.
What is the TEMPEST study?
TEMPEST is a research study that has been designed to test whether a drug called trientine dihydrochloride (also called Cufence) reduces heart muscle thickening, improves exercise capacity, improves heart function and reduces abnormal heart rhythms in patients with HCM. The study is also assessing how trientine works in HCM.
Participants in the study will be prescribed either trientine or placebo. A placebo is a medication with no active ingredients and is used to work out how effective trientine is.
Information Sheets
Why are we doing this research?
Several studies in other diseases have shown that copper imbalance is associated with heart thickening similar to HCM. In these diseases, treatment with trientine dihydrochloride, an oral medicine that increases copper elimination in urine, can reverse this thickening.
We want to find out if trientine reduces heart muscle thickening in patients with HCM. We would also like to see if it improves exercise capacity, improves heart function and reduces abnormal heart rhythms. The study will also assess how trientine works in HCM. The results from this study will be used to help us to understand the effects of trientine on the heart in patients with HCM.
Where is TEMPEST up to?
TEMPEST has received all necessary initial approvals (Research Ethics Committee (REC) Medicines and Healthcare products Regulatory Agency (MHRA) Health Research Authority (HRA)) for the study to go ahead.
Hospital departments taking part
The study will take part in Cardiac Departments across the country. To see if your hospital is taking part in this study please go to recruiting sites.
Study funder
This study has been funded by the National Institute for Health Research Efficacy and Mechanism Evaluation (EME) programme (ref: NIHR127575)
Study organiser
This study is sponsored by Manchester University NHS Foundation Trust and coordinated by the Liverpool Clinical Trials Centre at the University of Liverpool.
Study review and approval
The study has been reviewed by a research ethics committee, who have agreed the study is being conducted in a correct and appropriate manner. The study has also been approved by the Medicines & Healthcare products Regulatory Agency (MHRA) and the Health Research Authority (HRA).
The patient recruitment figures and sites currently open.
Patients
Target
152
Randomised
...
Sites
Target
4
Open
6
Study Timeline
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Visit 1
- Consent
- Medical history
- BP, heart rate, height, weight
- Blood and urine test
- ECG, 24 hour heart monitor
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Visit 2
- BP, heart rate
- Blood and urine test
- ECG
- Prescribed trientine or placebo
-
Visit 3
- BP, heart rate
- Blood and urine test
- ECG
- Prescribed trientine or placebo
-
Visit 4
- BP, heart rate
- Blood and urine test
- ECG
- Prescribed trientine or placebo
-
Visit 5
- BP, heart rate
- Blood and urine test
- ECG
- Prescribed trientine or placebo
-
Visit 6 (End of treatment)
- BP, heart rate, height, weight
- Blood and urine test
- ECG, 24 hour heart monitor, exercise test, MRI scan
- Spectroscopy MRI scan (subgroup)
Collaborators
