Rationale for trientine in HCM
Wilson disease, LV hypertrophy, HCM, diabetes
WILSON DISEASE, LV HYPERTROPHY, GENETIC DISORDER, DIABETES
Trientine dihydrochloride is a highly selective copper II chelator that is licensed in Wilson disease, a genetic disorder of copper excretion. Patients with Wilson disease can exhibit a cardiac phenotype that mimics HCM.Type II diabetes is associated with LV hypertrophy and abnormal copper homeostasis.HCM is associated with altered copper homeostasis, specifically, elevated serum copper and caeruloplasmin, in comparison to matched healthy volunteers.