in mitochondrial cardiomyopathy following treatment with idebenone.
Lerman-Sagie T, Rustin P, Lev D, Yanoov M,
Sagie A, Ben-Gal T
Mitochondrial Disease Clinic,
Metabolic Neurogenetic Service,
Center, Holon, Israel.
J Inherit Metab Dis 2001 Feb;24(1):28-34
Idebenone, a synthetic analogue of
coenzyme Q10, has been shown to improve cardiac function in patients with
Friedreich ataxia and a deficiency of respiratory chain complexes I-III. We
describe a woman with severe combined right and left heart failure due to a
mitochondrial cardiomyopathy. The patient underwent an endomyocardial biopsy
as part of an evaluation for cardiac transplantation. It showed severely
decreased respiratory complex activities dependent on CoQ, pointing to CoQ
depletion. Following idebenone treatment there was a dramatic improvement in
her clinical status with resolution of the heart failure