The recovery from dilated cardiomyopathy followed by hypertrophic cardiomyopathy, in the course of deficiency of long-chain 3-hydroxyacyl- coenzyme A dehydrogenase in infant

Medium-Chain Acyl-CoA Dehydrogenase Deficiency in an Infant with Dilated Cardiomyopathy

We report about an infant affected by dilated cardiomyopathy (CMP) in whom metabolic investigations evidenced medium-chain-acyl-CoA dehydrogenase deficiency (MCADD), that is one of three types of inherited disorders of mitochondrial fatty-acid beta-oxidation. Long-chain and very long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficits are recognized as responsible of hypertrophic or, less fre...

[Long-chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency and cardiomyopathy].

Review: Metabolic cardiomyopathy and conduction system defects in children.

Metabolic cardiomyopathies include amino acid, lipid and mitochondrial disorders, as well as storage diseases. A number of metabolic disorders are associated with both myopathy and cardiomyopathy. These include the glycogen storage diseases, ie, acid maltase deficiency (infantile, childhood, and adult onset), McArdle disease, and debrancher and brancher deficiencies. Disorders of lipid metaboli...

The value of myocardial perfusion imaging in differentiating between idiopathic dilated cardiomyopathy from the ischemic form [Persian]

Introduction: Differentiating between ischemic cardiomyopathy (ICM) and idiopathic dilated cardiomyopathy (IDCM) is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction (EF) introducing a noninvasive tool to diagnose those who will benefit from angiography seems...

Investigation of Polymorphisms in Non-Coding Region of Human Mitochondrial DNA in 31 Iranian Hypertrophic Cardiomyopathy (HCM) Patients

The D-loop region is a hot spot for mitochondrial DNA (mtDNA) alterations, containing two hypervariable segments, HVS-I and HVS-II. In order to identify polymorphic sites and potential genetic background accounting for Hypertrophic CardioMyopathy (HCM) disease, the complete non-coding region of mtDNA from 31 unrelated HCM patients and 45 normal controls were sequenced. The sequences were aligne...