Local Inflammation in Arrhythmogenic Right Ventricular Cardiomyopathy

Local Inflammation in Arrhythmogenic Right Ventricular Cardiomyopathy

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.

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