Abstract
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a cardiomyopathy described in adults as a risk factor in sudden unexpected cardiac death. The disease is of unknown etiology and is characterized by progressive fatty or fibrofatty replacement of the subendocardial layer of the heart muscle, predominantly of the right ventricle. The disease is not associated with primary pulmonary disease or mitral insufficiency. The aim of the present paper is to show that ARVC in children coexists with chronic myocarditis, as was shown with the aid of immunocytochemical staining for markers for T-lymphocytes (CD3, CD4, CD8) B lymphocytes (CD22) and macrophages (CD68), and for adhesion molecules (ICAM-1, VCAM, ELAM) and an endothelial marker (CD31/PECAM-1). Out of 55 endomyocardial biopsy specimens in children with a clinical diagnosis of dilated cardiomyopathy, myocarditis or arrhythmias, ARVD was recognized histologically in 12 patients (21.8%). Nine of these patients had shown immunocytochemical equivalents of persistent myocarditis.
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