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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Patients | Experimental | 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 |
|
| Control case | Experimental | 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). |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Peripheral immunological assessment on venous blood | Biological | 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 |
| Measure | Description | Time Frame |
|---|---|---|
| Identify the inflammatory components by C-reactive protein | Rate of C-reactive protein in the blood | 24 months |
| Identify the inflammatory components by interleukine1 | Rate of interleukin 1 beta in the blood | 24 months |
| Identify the inflammatory components by onterleukine6 | Rate of interleukin 6 in the blood | 24 months |
| Identify the inflammatory components by interleukine10 | Rate of interleukin 10 in the blood | 24 months |
| Identify the inflammatory components by Tumor Necrosis Factor | Rate of Tumor Necrosis Factor alpha in the blood | 24 months |
| Identify the inflammatory components by Transforming Growth Factor | Rate of Transforming Growth Factor beta in the blood | 24 months |
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Inclusion Criteria:
For cases:
For controls * Admitted for ablation procedures (accessory pathway, atrial flutter) on otherwise healthy hearts.
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Philippe MAURY, MD | Contact | 5 61 32 34 70 | +33 | maury.p@chu-toulouse.fr |
| Maxime BENEYTO | Contact | beneyto.m@chu-toulouse.fr |
| Name | Affiliation | Role |
|---|---|---|
| Philippe MAURY, MD | University Hospital, Toulouse | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Toulouse University Hospital Center | Recruiting | Toulouse | France |
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| ID | Term |
|---|---|
| D019571 | Arrhythmogenic Right Ventricular Dysplasia |
| D007249 | Inflammation |
| D003966 | Camurati-Engelmann Syndrome |
| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D009202 | Cardiomyopathies |
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| Immunological assessment carried out on intracardiac material | Biological | Immunological assessment carried out as part of the research, on intracardiac material taken during the electrophysiological examination: 1 EDTA tube |
|
| D006331 | Heart Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D010009 | Osteochondrodysplasias |
| D001848 | Bone Diseases, Developmental |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
| D030342 | Genetic Diseases, Inborn |