Diagnostic Contribution, Prognosis and Physiopathological Aspects in Arrhythmogenic Cardiomyopathy. (ACORE)

Study Overview
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Brief Summary
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This study aims to identify novel inflammatory biomarkers in AC, whether in circulating blood, in situ or as imaging biomarkers to better understand the pathophysiology of the disease and then to determine contribution to the clinical management of patients.

Detailed Description
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The treatment of AC remains based on palliative measures aimed at treating the consequences of the disease: antiarrhythmic treatments, defibrillator, treatments for heart failure. The identification of new biomarkers, in particular circulating ones, would make it possible to open pathophysiology avenues which, in the long term, could lead to therapies targeted to autoimmunity or inflammation. Many scientific and medical questions remain unanswered and require precise databases on the diagnostic and prognostic evaluation of this pathology. The objective of this study is to identify novel inflammatory biomarkers in patients with AC by studying the autoimmunity, inflammatory, and immunological profiles through blood samples and myocardial biopsies.

Official Title
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Biomarkers of Inflammation and Autoimmunity: Diagnostic Contribution, Prognosis and Physiopathological Aspects in Arrhythmogenic Cardiomyopathy.

Conditions
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Arrhythmogenic Cardiomyopathy

Intervention / Treatment
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* Biological: Prospective
* Other: Retrospective


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Adult patient (age ≥ 18 years old) Patient with a probable or confirmed diagnosis of cardiomyopathy according to the diagnostic criteria of the international task force Patient carrying a pathogenic mutation responsible for cardiomyopathy Patient informed individually of the research Exclusion Criteria: Patients under curatorship/guardianship Pregnant women Patients who expressed their opposition to participate in the study

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 100 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Arrhythmogenic Right Ventricular Cardiomyopathy (Prospective)<br> | Biological: Prospective<br>* -Additional blood samples -Myocarditis biopsy sample (routine care) -Additional pericardial fluid sample (routine care)<br>|
| Arrhythmogenic Right Ventricular Cardiomyopathy (Retrospective)<br> | Other: Retrospective<br>* None (only data collection)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measure the correlation between the autoimmunity, inflammatory, and immunological profiles through biomarkers found in blood samples, myocardial biopsies, mass imaging cytometry, and cardiac imaging in patients with Arrhythmogenic Cardiomyopathy (AC) |  | 10 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measure the correlation between circulating biomarkers and the severity of the phenotype determined by the severity of AC and multi-modality imaging |  | 10 years | 
| Measure the correlation between imaging biomarkers and electrocardiogram (ECG) parameters (presence of repolarization and depolarization abnormalities) |  | 10 years | 
| Measure the changes in inflammatory biomarkers in serum and cardiac imaging over time |  | 10 years | 
| Demonstrate a correlation between circulating and imaging biomarkers and the link between the extent of fibro-adipose infiltrates and the ventricular strain |  | 10 years | 
| Measure the correlation between circulating and imaging biomarker values and electro-anatomical mapping data |  | 10 years | 
| Screening for cardiotropic virus in pericardial fluid and circulating blood sampled in routine care during epicardial ablation by PCR |  | 10 years | 
| Measure the correlation between the presence of a pathogenic or common genetic variant and serum/imaging biomarkers. |  | 10 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Arrhythmogenic Cardiomyopathy, Inflammatory biomarkers, Autoantibodies