title: Acute dilated cardiomyopathy in a patient with deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase.
abstract: Deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase (LCHADD) is a rare inborn error of metabolism. It is associated with hypertrophic cardiomyopathy and less frequently with dilated cardiomyopathy. The incidence and pathophysiology of cardiac involvement in LCHADD is poorly understood. This report describes the acute decompensation of a 3-year-old girl who had LCHADD with rapidly developing dilated cardiomyopathy. A review of the literature and possible causes of cardiomyopathy in LCHADD are explored.
authors.affiliation: Department of Pediatrics, Nationwide Children's Hospital Heart Center, Columbus, OH 43205-2696, USA.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Dyke
authors.forename: Laura
authors.initials: L
authors.lastname: Konczal
authors.forename: Dennis
authors.initials: D
authors.lastname: Bartholomew
authors.forename: Kim L
authors.initials: KL
authors.lastname: McBride
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Hoffman
mesh_terms: D000208:Acute Disease; D044942:Acyl-CoA Dehydrogenase, Long-Chain / Q000172:deficiency*; D002311:Cardiomyopathy, Dilated / Q000201:enzymology* / Q000235:genetics*; D002675:Child, Preschool; D005260:Female; D006801:Humans
chemical_list: D044942:Acyl-CoA Dehydrogenase, Long-Chain