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Note: This record shows only 22 elements of the WHO Trial Registration Data Set. To view changes that have been made to the source record, or for additional information about this trial, click on the URL below to go to the source record in the primary register.
Register: EUCTR
Last refreshed on: 3 August 2020
Main ID:  EUCTR2020-002242-17-GB
Date of registration: 03/06/2020
Prospective Registration: Yes
Primary sponsor: Manchester University NHS Foundation Trust
Public title: A trial of trientine in patients with hypertrophic cardiomyopathy
Scientific title: A randomised, double-blind, placebo-controlled, phase 2 evaluation of the efficacy and mechanism of trientine in patients with hypertrophic cardiomyopathy. - TEMPEST
Date of first enrolment: 30/07/2020
Target sample size: 172
Recruitment status: Authorised-recruitment may be ongoing or finished
URL:  https://www.clinicaltrialsregister.eu/ctr-search/search?query=eudract_number:2020-002242-17
Study type:  Interventional clinical trial of medicinal product
Study design:  Controlled: yes
Randomised: yes
Open: no
Single blind: no
Double blind: yes
Parallel group: no
Cross over: no
Other: no
If controlled, specify comparator, Other Medicinial Product: no
Placebo: yes
Other: no
Number of treatment arms in the trial: 2
 
Phase:  Human pharmacology (Phase I): no Therapeutic exploratory (Phase II): yes Therapeutic confirmatory - (Phase III): no Therapeutic use (Phase IV): no
Countries of recruitment
United Kingdom
Contacts
Name:    
Address:  University of Liverpool, Institute in the Park, Alder Hey Children’s NHS Foundation Trust L12 2AP Liverpool United Kingdom
Telephone: 0151 794 9763
Email: tempest@liverpool.ac.uk
Affiliation:  Liverpool Clinical Trials Centre
Name:    
Address:  University of Liverpool, Institute in the Park, Alder Hey Children’s NHS Foundation Trust L12 2AP Liverpool United Kingdom
Telephone: 0151 794 9763
Email: tempest@liverpool.ac.uk
Affiliation:  Liverpool Clinical Trials Centre
Key inclusion & exclusion criteria
Inclusion criteria:
1. Written informed consent.
2. Age 18-70 inclusive.
3. Hypertrophic cardiomyopathy (HCM), as defined by the European Society of Cardiology HCM guidelines as: “a wall thickness =15 mm in one or more LV myocardial segments that is not explained solely by loading conditions”. The same definition is applied to first-degree relatives of patients with HCM i.e. all participants are required to have a LV wall thickness =15 mm. Wall thickness is as measured on the most recent cardiovascular magnetic resonance (CMR) scan performed prior to the baseline visit. If CMR has not been performed previously, wall thickness measurement should be taken from the most recent echocardiogram performed prior to the baseline visit .
(It is recognised that in the European Society of Cardiology guidelines a clinical diagnosis of HCM in first-degree relatives requires a wall thickness that is less than this value, however =15 mm is applied here in order to ensure that all participants have an unequivocal phenotype).
4. New York Heart Association class I, II or III at the most recent clinical assessment performed prior to the baseline visit.
Are the trial subjects under 18? no
Number of subjects for this age range: 0
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 157
F.1.3 Elderly (>=65 years) yes
F.1.3.1 Number of subjects for this age range 15

Exclusion criteria:
1. Previous or planned septal reduction therapy.
2. Previously documented myocardial infarction or severe coronary artery disease.
3. Uncontrolled hypertension, defined as a systolic blood pressure of >180mmHg or a diastolic blood pressure of > 100mmHg at Visit 1.
4. Known LV EF < 50%, as measured on the most recent CMR scan performed prior to the baseline visit. If CMR has not been performed previously, the most recent echocardiogram performed prior to the baseline visit should be used.
5. Previously documented persistent atrial fibrillation.
6. Anaemia, defined as haemoglobin being below the local site normal reference range, at Visit 1.
7. Iron deficiency, defined as serum iron being below the local site normal reference range, at Visit 1.
8. Copper deficiency, defined as serum copper being below the normal reference range, at Visit 1.
9. Pacemaker or implantable cardioverter defibrillator.
10. Known severe valvular heart disease, as demonstrated on the most recent heart imaging performed prior to the baseline visit.
11. Previously documented other cardiomyopathic cause of myocardial hypertrophy (e.g. amyloidosis, Fabry disease, mitochondrial disease).
12. History of hypersensitivity to any of the components of the investigational medicinal product (IMP).
13. Known contraindication to MRI scanning.
14. Pregnancy, lactation or planning pregnancy. Women of childbearing capacity are required to have a negative serum pregnancy test before treatment , must agree to pregnancy tests at study visits as defined in the Section 8 and must agree to maintain highly effective contraception as defined in Section 8 during the study.
15. Any medical condition, which in the opinion of the Investigator, may place the patient at higher risk from his/her participation in the study, or is likely to prevent the patient from complying with the requirements of the study or completing the study.


Age minimum:
Age maximum:
Gender:
Female: yes
Male: yes
Health Condition(s) or Problem(s) studied
Hypertrophic Cardiomyopathy (HCM)
MedDRA version: 20.0 Level: PT Classification code 10020871 Term: Hypertrophic cardiomyopathy System Organ Class: 10010331 - Congenital, familial and genetic disorders
Therapeutic area: Diseases [C] - Cardiovascular Diseases [C14]
Intervention(s)

Trade Name: Cufence
Product Name: Cufence
Pharmaceutical Form: Capsule, hard
INN or Proposed INN: Trientine
CAS Number: 38260-01-4
Other descriptive name: Triethylinetetramine dihydrochloride
Concentration unit: mg milligram(s)
Concentration type: equal
Concentration number: 200-
Pharmaceutical form of the placebo: Capsule, hard
Route of administration of the placebo: Oral use

Product Name: Placebo
Pharmaceutical Form: Capsule, hard

Primary Outcome(s)
Main Objective: To find out if trientine reduces heart muscle thickening in patients with hypertrophic cardiomyopathy.
Primary end point(s): Change in LVMi (g/m2), measured using CMR.
Secondary Objective: To find out if trientine improves exercise capacity, improves heart function and reduces abnormal heart rhythms in patients with hypertrophic cardiomyopathy. The study will also assess how trientine works in hypertrophic cardiomyopathy.
Timepoint(s) of evaluation of this end point: Measured from baseline to week 52.
Secondary Outcome(s)
Secondary end point(s): 1. Cumulative urine copper excretion, measured using urinary copper.
2. Change in exercise capacity, measured using cardiopulmonary exercise testing (CPET).
3. Change in number of non-sinus supraventricular heart beats, presence and amount of atrial fibrillation, number of ventricular-origin beats and presence and amount of non-sustained ventricular tachycardia, in 24 hours, measured using ambulatory heart monitoring.
4. Change in circulating high sensitivity troponin.
5. Change in LV global longitudinal strain and strain rate, wall thickness, mass, volumes and ejection fraction (EF) measured using CMR.
6. Change in peak left ventricular outflow tract gradient, measured using CMR.
7. Change in atrial volume and function, measured using CMR.
Timepoint(s) of evaluation of this end point: All assessed from baseline to week 52, except urine copper excretion, which is assessed from baseline to weeks 13, 26, 39 and 52.
Secondary ID(s)
B00844
Source(s) of Monetary Support
National Institute for Health Research (NIHR)
Univar Solutions
Secondary Sponsor(s)
Ethics review
Status: Approved
Approval date: 21/07/2020
Contact:
Results
Results available:
Date Posted:
Date Completed:
URL:
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