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Copper chelation in patients with hypertrophic cardiomyopathy

Anna Reid1, Christopher Miller1,2, John Peter Farrant3,2

  • 1Cardiovascular Division, Northwest Heart Centre, Manchester University NHS Foundation Trust, Manchester, UK.

Open Heart
|February 16, 2022
PubMed

Insights

Trientine dihydrochloride safely improved copper homeostasis in hypertrophic cardiomyopathy (HCM) patients. This copper chelation therapy showed potential in reducing left ventricular mass and improving cardiac strain, warranting further investigation.

Area of Science:

  • Cardiology
  • Medical Biochemistry
  • Pharmacology

Background:

  • Copper (Cu) homeostasis disturbances are linked to hypertrophic cardiac phenotypes like Wilson's disease.
  • Abnormal Cu homeostasis was previously identified in hypertrophic cardiomyopathy (HCM) patients.
  • A hypothesis suggested Cu2+-selective chelation with trientine dihydrochloride could slow or reverse HCM progression.

Purpose of the Study:

  • To explore the clinical efficacy of trientine in HCM patients.
  • To assess the safety and tolerability of trientine treatment in HCM.
  • To investigate the potential of trientine as a therapeutic target for HCM.

Main Methods:

  • An open-label pilot study involving 20 HCM patients treated with trientine for 6 months.
  • Comprehensive assessments included cardiac magnetic resonance imaging (CMR) and CMR 31P-spectroscopy at baseline and end of therapy.
  • Ten matched HCM patients served as controls, with predefined endpoints including changes in left ventricular mass (LVM) and myocardial energetics.

Main Results:

  • Trientine treatment was safe and well-tolerated, increasing urinary copper excretion without affecting serum copper.
  • Significant improvements were observed in total atrial strain and global longitudinal LV strain.
  • Trientine treatment led to a significant decrease in LVM compared to controls and a reduction in extracellular matrix volume.

Conclusions:

  • Cu2+-selective chelation with trientine is safe in a controlled environment for HCM patients.
  • Trientine demonstrates potential as a future therapeutic target for managing HCM.
  • A phase 2b trial is currently underway to further evaluate trientine's efficacy in HCM.
Abstract

Keywords:
cardiomyopathiescardiomyopathyhypertrophicmagnetic resonance imaging

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