Infantile histiocytoid cardiomyopathy

Common Name(s)

Infantile histiocytoid cardiomyopathy

Histiocytoid cardiomyopathy, which was initially described by {8:Voth (1962)}, goes by various names, including infantile xanthomatous cardiomyopathy ({4:MacMahon, 1971}), focal lipid cardiomyopathy ({2:Bove and Schwartz, 1973}), oncocytic cardiomyopathy ({7:Silver et al., 1980}), infantile cardiomyopathy with histiocytoid change ({3:Ferrans et al., 1976}), and foamy myocardial transformation of infancy ({9:Yatani et al., 1988}). The disorder is a rare but distinctive entity of infancy and childhood characterized by the presence of characteristic pale granular foamy histiocyte-like cells within the myocardium. It usually affects children younger than 2 years of age, with a clear predominance of females over males. Infants present with dysrhythmia or cardiac arrest, and the clinical course is usually fulminant, sometimes simulating sudden infant death syndrome ({1:Andreu et al., 2000}).
 

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Scientific Literature

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Symptoms, Diagnosis, and Treatment

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