myocardium.
Fatty acid oxidation disorders(FAODs)can give rise to insufficiency of mitochondrial energy
production and accumulation of metabolic intermediates in
cardiomyocytes,such as
lipid and long-chain acyl
carnitine,leading to myocardial lesions.FAODs include
carnitine-dependent
fatty acid transport disorders and mitochondrial
fatty acid beta-
oxidation disorders,with any
enzyme or transporter defect in
fatty acid oxidation process resulting in this
disease. Although FAODs are one of the rare causes of pediatric
cardiomyopathy,some
patients with FAODs are expected to have
cardiomyopathy alleviated and
quality of life improved by early correction of
fatty acid metabolic disorders.