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The image of the month: Histiocytoid cardiomyopathy: diagnosis by light and electron microscopy.

Myocardial Disease

Histiocytoid cardiomyopathy in a female baby who died at the age of 7 months due to left ventricular fibrillation and heart failure. Clinically, the patient presented with suspected dilated cardiomyopathy and cardiac arrhythmias, however the etiology was unclear. Investigations by light and electron microscopy of ventricular endomyocardial biopsies revealed histiocytoid cardiomyopathy which is a cardiac manifestation of a mitochondrial disorder. The upper picture shows a Giemsa stain with many enlarged histiocyte-like cells with foamy and granular cytoplasm and reduced amounts of myofibrils. Only in the upper part of the picture a few normal myocytes are observed. In electron microscopy we found a severe rarefication of disrupted myofibrils and numerous swollen mitochondria with abnormal structures, vacuolization and disorganized cristae (lower picture). Clinically, patients may present with palpitations, dyspnea, cardiac murmurs or edema. The diagnosis of this rare, genetic, cardiac disorder of childhood, is established on the basis of the clinical findings, electrocardiography (ECG), echocardiography, endomyocardial biopsy, or autopsy. Currently, no specific treatment is available for histiocytoid cardiomyopathy.

Notes to editor

Presented by: Karin Klingel, MD
Department of Molecular Pathology, University Hospital Tübingen, Germany
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.