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

作者：Campuzano Oscar; Alcalde Mireia; Iglesias Anna; Barahona Dussault Catherine; Sarquella Brugada Georgia; Benito Begona; Arzamendi Dabit; Flores Jose; Leung Tack Ki; Talajic Mario; Oliva Antonio; Brugada Ramon^*

来源：Journal of Clinical Pathology, 2012, 65(12): 1077-1083.

DOI：10.1136/jclinpath-2012-201022

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. %26lt;br%26gt;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. %26lt;br%26gt;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. %26lt;br%26gt;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.

• 出版日期2012-12