Autopsy and Endomyocardial Biopsy Findings

摘要: According to the 1995 WHO classification, arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is defined as a heart muscle disorder characterized by progressive fibrofatty replacement of myocardium, initially with typical regional and later global some left involvement, relative sparing septum [1]. The residual myocytes interspersed among adipocytes fibrous tissue provides ideal substrate for re-entrant life-threatening arrhythmias [2, 3, 4, 5, 6, 7, 8]. In 1982, Marcus et al. [2] reported first clinical series in adults, pointing out peculiar fatty “dysplasia” free wall absence an ischemic milieu. their original series, morphologic findings were obtained from 13 adult patients, 12 who had surgery one died because pulmonary embolism postmortem investigation. Aneurysms described socalled triangle dysplasia, i.e., outflow tract, apex, inferior wall. Furthermore, marked decrease myocardial fibers myocardium usually replaced fibrosis fat was evident. areas “dysplasia,” there thickened whitish sclerotic endocardium. At higher magnification, hypertrophy or degeneration few remaining dysmorphic picnotic nuclei detected, well variable amount histiocytic lymphocytic inflammatory infiltrates.