About Tempest

Why are we doing this research?

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.

Why have I been asked to take part?

You have been invited to take part in this study because you have hypertrophic cardiomyopathy.

Taking part is voluntary. It is up to you to decide whether or not you want to take part. If you decide not to take part then you will still receive the usual treatment your hospital offers. The decision you make on whether to take part or not will not affect the standard of care you receive now or in the future.

It is okay if you agree to take part in the study but later change your mind. You do not need to give a reason. The study doctor may also choose to withdraw you if it is necessary for your health. If you do decide to stop taking part we will ask you if you would like to: 1. Continue to complete follow up visits for the study; or 2. Stop taking part with no more study visits.

If you decide that you would like to withdraw from the study we will keep information about you that we already have. If you choose to stop taking part in the study, we would like to continue collecting information about your health from central NHS records, your hospital and your GP. If you do not want this to happen, tell us and we will stop.

What will happen if I agree to take part?

If you agree to take part, you will be asked to sign a consent form. You will be given a copy of the consent form and participant information sheet to keep.

Once you have signed the consent form, we will check and confirm that this study is suitable for you. If it is, you will be entered into the study.

You will be in the study for 1 year. During this time, you will be asked to attend the hospital for 6 visits. Study tests include blood tests, urine test, heart trace (ECG), heart magnetic resonance imaging (MRI scan of the heart), 24-hour heart monitor, exercise test and pregnancy test if female and of childbearing age. A subgroup of participants will undergo an extra MRI scan.

What is Trientine?

Trientine is a medicine currently used in patients with a condition called Wilson’s disease. In Wilson’s disease there is an abnormal build-up of copper within the body that leads to heart muscle thickening similar to HCM. Trientine makes the body clear more copper in the urine and therefore reduces copper levels in the body. Studies have shown that patients with hypertrophic cardiomyopathy also have an excess of copper. In patients with diabetes, trientine reduces heart muscle thickness.

We therefore wish to study whether trientine can improve heart muscle thickening and quality of life in patients with HCM.

How will I know which treatment I'm going to have?

Whether you receive trientine or placebo will be decided at random by computer. This process is called ‘randomisation’ or ‘random allocation’. You and the research team will not be able to choose which treatment you receive.

The trientine and the placebo capsules are identical and therefore you and the research team will not know which treatment you will take during the study. This is called a ‘blinded trial’.

The pharmacy team in your hospital will know which treatment you are receiving so they can prepare the correct one. Treatment details can be made available if needed for your clinical care.


NIHR - National Institute for Health Research logo
Manchester University Hospital
Liverpool Hearth and Chest Hospital
Oxford University Hospital
Royal Brompton and Harefield NHS Trust
University of Liverpool
LCTC - Liverpool Clinical Trials Centre
University of Manchester

This project (project reference NIHR127575) is funded by the Efficacy and Mechanism Evaluation (EME) Programme, an MRC and NIHR partnership. The views expressed in this publication are those of the author(s) and not necessarily those of the MRC, NIHR or the Department of Health and Social Care.

© 2021 LCTC, a division of the University of Liverpool