Congenital "histiocytoid" Cardiomyopathy: Evidence Suggesting a Developmental Disorder of the Purkinje Cell System of the Heart

The so-called "histiocytoid cardiomyopathy" is an unusual cardiac disorder of infancy and childhood, characterized by the presence of numerous foamy, lipid-containing cells between the endocardium and the striated myocardial cells of the left ventricle and the interventricular septum. The disease usually affects females, the clinical picture being dominated by severe disturbances of conduction. The original designations of the disorder stem from the morphological resemblance of the foamy cells to lipid-laden histiocytes. However, subsequent investigations have shown these cells to contain myofibrils interposed with Z lines. It has, therefore, been suspected that the leading cell population might be related to the myocardium. Using a histochemical method for the demonstration of cholinesterase activity in the foamy cells, we present evidence that "histiocytoid" cardiomyopathy may in fact correspond to a maldevelopment of the Purkinje cell system of the heart.