Key result
Ventricular noncompaction links to histiocytoid cardiomyopathy marked by strong MyoD1 expression in case report.
Why the study?
The association between ventricular noncompaction and histiocytoid cardiomyopathy is rare and has been reported only twice previously in the literature.
Case Report (n=1)
Reports a rare association between ventricular noncompaction and histiocytoid cardiomyopathy, noting strong MyoD1 expression in histiocytoid cells.
Single-case report warrants diagnostic caution; hypothesis-generating for MyoD1 utility in pediatric cardiomyopathies.
We report an association between ventricular noncompaction and histiocytoid cardiomyopathy. Both entities are rare, and only 2 cases of their association have been reported previously in the medical literature. Ventricular noncompaction is believed to be caused by an arrest of the normal endomyocardial development, resulting in a thin and compacted epicardial layer and a thickened noncompacted endocardial layer. Histiocytoid cardiomyopathy is a rare arrhythmogenic disorder characterized by aggregates of oncocytic cells involving predominantly the subendocardium. These cells are thought to be abnormal Purkinje cells. In our case, the histiocytoid cells showed strong cytoplasmic expression for the skeletal muscle transcription factor MyoD1, which could be attributed to cross reactivity with an undetermined cytoplasmic antigen.
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Planas et al. (2012) conducted a case report in Ventricular noncompaction and histiocytoid cardiomyopathy (n=1). Ventricular noncompaction and histiocytoid cardiomyopathy was evaluated. A rare case demonstrated the association between ventricular noncompaction and histiocytoid cardiomyopathy, with histiocytoid cells showing strong cytoplasmic expression for MyoD1.
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