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Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone
Author(s) -
LermanSagie T.,
Rustin P.,
Lev D.,
Yanoov M.,
LeshinskySilver E.,
Sagie A.,
BenGal T.,
Munnich A.
Publication year - 2001
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1023/a:1005642302316
Subject(s) - idebenone , coenzyme q10 , cardiomyopathy , medicine , heart failure , cardiology , ataxia , heart transplantation , coenzyme q – cytochrome c reductase , cardiac function curve , mitochondrial disease , mitochondrial respiratory chain , dilated cardiomyopathy , respiratory system , mitochondrion , mitochondrial dna , biology , genetics , cytochrome c , psychiatry , gene
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.