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Is the Cause of Arrhythmogenic Right Ventricular Cardiomyopathy Congenital or Acquired?

  • C. Basso
  • A. Rampazzo
  • B. Bauce
  • F. Calabrese
  • G. Danieli
  • A. Nava
  • M. Valente
  • G. Thiene
Conference paper

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a myocardial disease in which the myocardium of the right ventricular free wall is partially or almost entirely replaced by fatty or fibrofatty tissue [1–3]. The process begins at the epicardium and gradually extends through the myocardium towards the subendocardium. The anterior right ventricular outflow tract, ]the apex, and the inferoposterior wall are the most frequently affected sites, constituting the so-called “triangle of dysplasia” [4]. Histological examination of affected myocardial tissue demonstrates residual myocytes interspersed among adipocytes and fibrous tissue, providing a substrate for ventricular arrhythmias that may range from asymptomatic premature ventricular beats to ventricular tachycardia, which is typically of left bundle branch block morphology, merging into ventricular fibrillation. Sudden cardiac death may be the first manifestation of the disease, particularly during effort [2]. The left ventricle may be involved with disease progression accounting for heart failure [3–5].

Keywords

Ventricular Free Wall Ventricular Cardiomyopathy Arrhythmogenic Right Ventricular Dysplasia Catecholaminergic Polymorphic Ventricular Tachycardia Ryr2 Gene 
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 2004

Authors and Affiliations

  • C. Basso
    • 3
  • A. Rampazzo
    • 3
  • B. Bauce
    • 2
  • F. Calabrese
    • 3
  • G. Danieli
    • 1
  • A. Nava
    • 2
  • M. Valente
    • 3
  • G. Thiene
    • 3
  1. 1.Institutes of BiologyUniversity of Padua Medical SchoolItaly
  2. 2.Institutes of CardiologyUniversity of Padua Medical SchoolItaly
  3. 3.Institutes of PathologyUniversity of Padua Medical SchoolItaly

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