Editorial Arrhythmogenic Right Ventricular Cardiomyopathies Clinical Forms and Main Differential Diagnoses
نویسنده
چکیده
In this issue of Circulation, Burke et al 1 report the results of a morphological and morphometric analysis of RV myocardium to differentiate fatty infiltration of the RV free wall from fibrofatty infiltration, which is the histological marker of ARVC. A similar analysis was previously presented by Thiene et al, classifying their patients as having "lipomatous" as opposed to "fibrolipomatous" replacement of lac myocardium and putting the two entities in the same group. Unless small areas of fibrosis are overlooked, the two subgroups of Burke et al and Thiene et al have the histological picture of myocardium intermingled with and/or replaced by fat without associated fibrosis. In this population of patients who died suddenly, we are faced with the observation that strands of myocardial fibers within fat without fibrosis may lead to sudden death. This possibility has rather important consequences, because a large proportion of seemingly normal hearts have fatty infiltration of the RV myocardium. It may be that the fatty pattern in these patients represents the early stage of the disease before the appearance of fibrotic tissue. Therefore, a large proportion of normal individuals may in fact have the prerequisite for the development of RV cardiomyopathies.
منابع مشابه
Arrhythmogenic right ventricular cardiomyopathies: clinical forms and main differential diagnoses.
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