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Autopsy and Endomyocardial Biopsy Findings

  • Chapter
Arrhythmogenic RV Cardiomyopathy/Dysplasia

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 [2–8]. 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.

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Authors and Affiliations

  1. Department of Medical Diagnostic Sciences, University of Padua, Italy

    Cristina Basso MD, PhD & Gaetano Thiene MD

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  1. Cristina Basso MD, PhD
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  2. Gaetano Thiene MD
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Editors and Affiliations

  1. Section of Cardiology, Department of Medicine, Sarver Heart Center, University of Arizona, Tucson, AZ, USA

    Frank I. Markus

  2. Department of Cardiological, Thoracic and Vascular Sciences, University of Padua, Italy

    Andrea Nava

  3. Department of Medical Diagnostic Sciences, University of Padua, Italy

    Gaetano Thiene

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Basso, C., Thiene, G. (2007). Autopsy and Endomyocardial Biopsy Findings. In: Markus, F.I., Nava, A., Thiene, G. (eds) Arrhythmogenic RV Cardiomyopathy/Dysplasia. Springer, Milano. https://doi.org/10.1007/978-88-470-0490-0_5

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