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Autopsy and Endomyocardial Biopsy Findings

  • Cristina Basso
  • Gaetano Thiene

Abstract

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

Keywords

Fatty Infiltration Cardiac Sarcoidosis Ventricular Free Wall Ventricular Cardiomyopathy Arrhythmogenic Right Ventricular Dysplasia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Italia 2007

Authors and Affiliations

  • Cristina Basso
    • 1
  • Gaetano Thiene
    • 1
  1. 1.Department of Medical Diagnostic SciencesUniversity of PaduaItaly

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