Defects in long chain fatty acid oxidation presenting as severe cardiomyopathy and cardiogenic shock in infancy.
Inborn errors of fatty acid metabolism are important causes of reversible cardiomyopathy in infancy. Disorders in long chain fatty acid oxidation can lead to cardiomyopathy, as fatty acid beta oxidation is the major source of myocardial energy after birth. We present 2 cases of such disorders with cardiac manifestations during infancy, which responded well to a diet low in long chain fatty acids.
Dereddy
Narendra R
NR
Department of Pediatrics, Maria Fareri Children's Hospital, Westchester Medical Center, Valhalla, NY, USA.
Kronn
David
D
Krishnan
Usha
U
D009202:Cardiomyopathies / Q000209:etiology*; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D007223:Infant; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications*; D008297:Male; D010084:Oxidation-Reduction; D012720:Severity of Illness Index; D012770:Shock, Cardiogenic / Q000209:etiology*
D005227:Fatty Acids