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J Clin Pathol 65:1077-1083 doi:10.1136/jclinpath-2012-201022
  • Original articles

Arrhythmogenic right ventricular cardiomyopathy: severe structural alterations are associated with inflammation

  1. Ramon Brugada1,2
  1. 1Centre de Genètica Cardiovascular Institut Investigació Biomèdica Girona and University of Girona, Girona, Spain
  2. 2Montreal Heart Institute, University of Montreal, Montreal, Canada
  3. 3Secció Cardiologia, Hospital Sant Joan de Déu, Barcelona, Spain
  4. 4Institute of Forensic Medicine, Catholic University, Rome, Italy
  1. Correspondence to Dr Ramon Brugada Terradellas, Director Cardiovascular Genetics Center IDIBGI-UdG, Dean School of Medicine, Universitat de Girona, Girona 17003, Spain; ramon{at}brugada.org
  • Accepted 4 August 2012
  • Published Online First 3 September 2012

Abstract

Aim Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a rare cardiomyopathy associated with sudden cardiac death. It is characterised by a progressive right ventricle (RV) fibrofatty replacement, although biventricular replacement (BV) is also common. Inflammation believed to be a key player in disease progression and outcome. Our study investigates the relationship between the presence of inflammatory infiltrates in myocardium and the severity of structural heart alterations in ARVC.

Methods Our study included eight control and 36 ARVC postmortem human heart samples. We performed macroscopic assessment and microscopic analysis for different inflammatory cell types.

Results Fibrofatty replacement of RV was present in all our cases. Thirteen cases showed sole RV involvement (36.11%). Of these, only one showed inflammatory infiltrates (7.69%). Sixteen cases showed severe ARVC phenotypic forms characterised by BV involvement and right auricular (RA) fatty accumulation plus RV dilation (44.44%); eight of them also showed inflammatory infiltrates (50%). Immunohistochemical studies revealed ventricular multifocal inflammatory infiltrates, showing seven T-lymphocytes as the main infiltrate cell types.

Conclusions The presence of inflammatory infiltrates in ventricular myocardium of ARVC samples is associated with severe structural heart changes, indicating that an inflammatory process may be a modulator of severity in ARVC.