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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a myocardial disease, often familial, that is characterised pathologically by fibrofatty replacement of the right ventricular myocardium, and clinically by ventricular arrhythmias of right ventricular origin which may lead to sudden death, mostly in young people and athletes.1-5 The term “dysplasia” was originally used to describe an entity that was considered to be the result of a developmental defect of the right ventricular myocardium.1 A better understanding of clinical manifestations as well as morphologic findings does not support the theory of a congenital absence of the myocardium, but is in keeping with a non-ischaemic, ongoing atrophy of the right ventricular myocardium, most likely genetically determined, which becomes symptomatic in adolescents and young adults.2-4 On the basis of its nature of progressive heart muscle disease of unknown aetiology, ARVC has been more appropriately included among the cardiomyopathies in the recent classification proposed by the task force of the World Health Organization/International Society and Federation of Cardiology. Although several theories have been advanced, the aetiopathogenesis of ARVC is still unknown.5
The most striking pathologic feature of ARVC is the diffuse or segmental loss of the myocardium of the right ventricular free wall and its replacement by fibrofatty tissue (fig 1); it is frequently transmural and accounts for aneurysmal dilations of the diaphragmatic, apical, and infundibular regions (so called “triangle of dysplasia”) in nearly 50% of the cases in the necropsy series.2-4 A wave front progression of the pathological process occurs from the subepicardium to the endocardium, so that residual myocardium is confined to the inner subendocardial layer and to the trabeculae of the right ventricle, where islands of surviving myocardial cells are scattered throughout the fibrofatty tissue. Patchy acute myocarditis with myocyte death and round cell (mostly T lymphocytes) inflammatory infiltrates …