Trientine is primarily used for the treatment of Wilson’s disease (WD), a genetic condition/disorder that causes copper accumulation in the body. While the medication can help manage copper levels, there`s limited evidence regarding its direct impact on Hypertrophic Cardiomyopathy (HCM). It’s best to consult a medical professional for specific and personalized advice on treating or managing both conditions.

The Efficacy of Trientine in Patients With Hypertrophic Cardiomyopathy (TEMPEST):

TEMPEST is a research study that has been formulated to test whether a therapeutic drug named trientine dihydrochloride (Cufence) alleviates heart muscle thickening, rectifies exercise efficiency, refines heart function, and decreases abnormal heart rhythms in those with hypertrophic cardiomyopathy (HCM). The study is also evaluating how trientine treatment works in patients with HCM.

Recipients in the study will be given either trientine or placebo for 1 year to compare the differences. A placebo is a medicine with no active pharmaceutical ingredients and is used to work out how promising trientine is.

This study aims to recruit 172 participants with HCM aged 18 to 70 years. The study is expected to run till December 2023.

The Efficacy of Trientine in Patients With Wilson’s Disease (WD):

There are 2 trientine salts. Trientine dihydrochloride salt (TETA 2HCL) is not stable at room temperature and needs to be stored at 2 to 8 °C. Trientine tetrahydrochloride, or TETA 4HCL is a comparatively more stable trientine salt that can be stored at room temperature.

A study was conducted by reviewing data from the national WD registry in France. 43 patients with WD who received TETA 2HCL or TETA 4HCL as a single agent for at least 12 months until 2010 were included. The primary endpoints were hepatic and neurological results. Secondary endpoints were the events that led to the discontinuation of the medicine.

Results: Changes in medicine were frequent, leading to the analysis of 57 treatment sequences of TETA 4HCL or TETA 2HCL. The mean duration of the treatment sequence was significantly longer in the group of TETA 4 HCL (12.6 years) than in the group of TETA 2HCL (7.6 years) (p = 0.011). 10 patients experienced both trientine salts: 8 stopped TETA 4 HCL (6 had a hepatologic phenotype, and 2 had a neurological phenotype) because this therapy did not exist anymore (mean duration 7.4 years). 3 of these patients already felt TETA 2 HCL prior to the sequence. 2 patients with a hepatologic phenotype (1 had a previous sequence of TETA 4 HCL before) halted TETA 2 HCL because of cold storage complications (mean duration 42.8 years). The total sequences were 57. All of the patients were found clinically stable. There was no difference in efficacy. Both therapies were well tolerated, except for a scenario of recurrence of lupus erythematosus-like syndrome in the TETA 2HCL group.

Conclusions: The 2 salts of trientine were promising in treating WD patients. However, discontinuation of TETA 2HCL was common, related to the cold storage requirement. As adherence to treatment is a major factor in the rich management of WD, clinicians need to be even more vigilant in detecting adherence complexities in those receiving TETA 2HCL.