Topic: Cardiovascular disease
Conditions
Interventions
Using trientine to assess if it improves fitness and heart function to improve treatment options for HCM. Participants will receive trientine for 6 months.
Sponsors
University Hospital of South Manchester NHS Foundation Trust
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Male or female > 18 years of age 2. Females will be non-pregnant and non-lactating with no intention of pregnancy during study treatment (see point 6) 3. Confirmed diagnosis of HCM in line with 2011 ACCF / AHA consensus document 4. Positive genotype 5. LV ejection fraction = 50% 6. Women of childbearing potential (not >1 year post-menopausal) must agree to use one of the following acceptable birth control methods: 6.1. True complete abstinence when this is in line with the preferred and usual lifestyle of the subject 6.2. Surgical sterilization of either the female subject in study (e.g., bilateral tubal ligation) or of her male partner (vasectomy with documented azoospermia) if he is the sole partner of that subject 6.3. Established progesterone-only hormonal contraception (implantable, patch, oral or intramuscular [IM]) administered for at least one month prior to study medication administration 6.4. Double barrier method: condom and occlusive cap (diaphragm) with spermicidal foam/gel/film/ cream/suppository
Exclusion criteria
Exclusion criteria: 1. History of any other cardiovascular disorder, including aortic stenosis, aortic coarctation, hypertension, renal artery stenosis, atrial fibrillation 2. NYHA Class III/IV heart failure 3. Diabetes mellitus 4. Contraindication to magnetic resonance imaging (MRI) scanning (including claustrophobia) 5. Known hypersensitivity to Trientine or excipients 6. Known hypersensitivity to Gadolinium-based contrast agent 7. eGFR 40kg/m2 9. History of significant malabsorption 10. Copper deficiency at baseline 11. Iron deficiency at baseline 12. Haemoglobin 2 years post- menopausal and/or not surgically sterilised) must have a negative blood serum pregnancy test, performed at visit 1 prior to administration of study medication 19. Any clinically significant or unstable medical or psychiatric condition that would interfere with the patient’s ability to participate in the study 20. Any other condition, which in the opinion of the research team, may put participants at risk during the study, or which may affect the outcome of the study 21. New medication within the preceding month of the study (excluding short term prescriptions) 22. Participation in another study involving an investigational product in the previous 12 weeks
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Myocardial energetics, measured using cardiac MRI prior to starting treatment and at the end of treatment | — |
Countries
United Kingdom
Outcome results
None listed