Local Inflammation in Arrhythmogenic Right Ventricular Cardiomyopathy (LI-ARVC)

The understanding of ARVC pathophysiology remains incomplete. Several clues indicate that disease progression is mediated through inflammation. The present study aim to document the feasibility of detecting the potential presence of intracardiac local inflammatory components in patients with ARVC.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable condition characterized by right ventricular (RV) dilatation/dysfunction and malignant ventricular arrhythmias. The understanding of ARVC pathophysiology remains incomplete. Several clues indicate that disease progression is mediated through inflammation. First, presence of subepicardial late gadolinium enhancement sharing the same characteristics as the ones found in myocarditis is common on cardiac magnetic resonance imaging (CMR). Second, clinical pathology findings of inflammatory infiltrates of mononuclear cells are frequent and correlate to the extent and severity of ARVC. Finally, from a biological standpoint, the exploratory study conducted by Campian et al. has shown an exaggerated humoral inflammatory response in peripheral blood whilst anti-desmoglein-2 antibodies (targeting a component of the desmosome) emerge as a sensitive and specific biomarker for ARVC. As specific treatments for ARVC are currently lacking, a better understanding of the humoral pathophysiology of the disease could unlock new therapeutic targets. We recently demonstrated that collecting local cardiomyocytes was feasible through irrigated ablation catheters in patients with ARVC. These steerable catheters may easily map the whole right ventricle and locate endocardial or epicardial scars. Aspiration of local blood or cellular material through the inner lumen of the catheter once pressed on the parietal wall may be an interesting technique for retrieving local inflammation markers.

Cardiac Electrophysiologic Techniques, Inflammation, Cytokines, Interleukin-1, Interleukin-6, Interleukin-10, Transforming Growth Factor beta, Tumor Necrosis Factor-alpha

Carrier of a definite diagnosis of arrhythmogenic dysplasia of the right ventricle in line with the criteria of the Task Force 2010 (see Appendices), admitted for an electrical mapping of the right ventricle

Without heart disease, admitted for a Kent bundle ablation or endocavity procedure / Wolff-Parkinson-White syndrome or common flutter (in subjects in whom the same irrigated material will be used and for whom echocardiography will have excluded associated heart disease).

Peripheral immunological assessment carried out as part of the research, on venous blood at the puncture point necessary for the electrophysiological examination: 1 heparin tube and 1 EDTA tube

Immunological assessment carried out as part of the research, on intracardiac material taken during the electrophysiological examination: 1 EDTA tube

Inclusion Criteria: For cases: Arrhythmogenic right ventricular dysplasia diagnosed (according to 2010 Task Force Criteria) Admitted for right ventricle electrophysiologic mapping For controls * Admitted for ablation procedures (accessory pathway, atrial flutter) on otherwise healthy hearts. Exclusion Criteria: Diagnostic of systemic chronic inflammatory disease Presence of possible or proven cardiac involvement of an inflammatory disease, an acute or chronic infectious disease. Taking immunosuppressant or immunomodulating medications