histiocytoid cardiomyopathy

Epidemiology

• rare
• usually affects children younger than 2 years of age, with a clear predominance of females over males

Pathology

• presence of characteristic pale granular foamy histiocyte- like cells within the myocardium

Genetics

• associated with defects in MT-CYB

Clinical manifestations

• infants present with dysrhythmia or cardiac arrest,
• clinical course is usually fulminant, sometimes simulating sudden infant death syndrome

More general terms

• cardiomyopathy

Database

• OMIM: https://mirror.omim.org/entry/500000