TY - JOUR
T1 - Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone
AU - Lerman-Sagie, T.
AU - Rustin, P.
AU - Lev, D.
AU - Yanoov, M.
AU - Leshinsky-Silver, E.
AU - Sagie, A.
AU - Ben-Gal, T.
AU - Munnich, A.
PY - 2001
Y1 - 2001
N2 - Idebenone, a synthetic analogue of coenzyme Q 10, 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.
AB - Idebenone, a synthetic analogue of coenzyme Q 10, 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.
UR - http://www.scopus.com/inward/record.url?scp=0035100542&partnerID=8YFLogxK
U2 - 10.1023/A:1005642302316
DO - 10.1023/A:1005642302316
M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???
C2 - 11286379
AN - SCOPUS:0035100542
SN - 0141-8955
VL - 24
SP - 28
EP - 34
JO - Journal of Inherited Metabolic Disease
JF - Journal of Inherited Metabolic Disease
IS - 1
ER -
