Arrhythmogenic Cardiomyopathies
Conditions
Keywords
Arrhythmogenic cardiomyopathies, PET-MR imaging, Myocardial inflammation, Prognosis, Cardiac auto-antibodies
Brief summary
Status of the research project: The main complications of arrhythmogenic cardiomyopathies (AC) are sudden death, more rarely heart failure. Recently, data are emerging in favor of an associated role of myocardial inflammation and myocarditis in this pathology, but the impact of inflammation on the presentation and prognosis of the cardiomyopathy, as well as its mechanisms, are not clearly elucidated. To date, endomyocardial biopsy is the gold standard for documenting myocardial inflammation. Aim of the research: To evaluate the interest of a new hybrid PET-MR imaging tool for tissue and metabolic characterization of AC associating MRI and 18F-FDG PET, already used in inflammatory pathologies (cardiac sarcoidosis). Project description: Multicentric observational study of 80 patients with genetic AC undergoing PET-MR. Description of the observed profiles and their impact on the phenotype of the cardiomyopathy and its evolution, study of associated immunological mechanisms, correlation with available anatomopathological data. Expected results and perspectives: first non-invasive description of tissue and metabolic phenotype of AC by PET-MR imaging and its prognostic role, basis for pathophysiological and therapeutic research in case of confirmation of the performances of this imaging for the detection of myocardial inflammation.
Detailed description
Status of the research project: The main complications of arrhythmogenic cardiomyopathies (AC) are sudden death, more rarely heart failure. Recently, data are emerging in favor of an associated role of myocardial inflammation and myocarditis in this pathology, but the impact of inflammation on the presentation and prognosis of the cardiomyopathy, as well as its mechanisms, are not clearly elucidated. To date, endomyocardial biopsy is the gold standard for documenting myocardial inflammation. Aim of the research: To evaluate the interest of a new hybrid PET-MR imaging tool for tissue and metabolic characterization of AC associating MRI and 18F-FDG PET, already used in inflammatory pathologies (cardiac sarcoidosis). Project description: Multicentric observational study of 80 patients with genetic AC undergoing PET-MR. Description of the observed profiles and their impact on the phenotype of the cardiomyopathy and its evolution, study of associated immunological mechanisms, correlation with available anatomopathological data. Expected results and perspectives: first non-invasive description of tissue and metabolic phenotype of AC by PET-MR imaging and its prognostic role, basis for pathophysiological and therapeutic research in case of confirmation of the performances of this imaging for the detection of myocardial inflammation.
Interventions
OTHERBiocollection
serum
Sponsors
University Hospital, Brest
University Hospital, Paris
University Hospital, Angers
Nantes University Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
16 Years to 99 Years
Healthy volunteers
No
Inclusion criteria
* Male and female over 16 years old
Exclusion criteria
* Patients and their relatives with left ventricular or biventricular AC and carrier of a pathogenic or likely pathogenic variant in one of the following genes : PKP2, DSG2, DSC2, JUP, DSP, DES, FLNC, PLN, LMNA, TMEM43, CDH2, BAG3, RYR2, RBM20 Consent form
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| PET-RMI patients profile | one hour | Rate of patients observed in the different expected PET-MRI patterns in the left ventricle (no late enhancement and no PET fixation, late enhancement and no PET fixation, concordant late enhancement and PET fixation, discordant late enhancement and PET fixation). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| PET-RMI patterns in the right ventricle | one hour | Rate of patients with different expected PET-MRI patterns in the right ventricle (no late enhancement and no PET fixation, late enhancement and no PET fixation, concordant late enhancement and PET fixation, discordant late enhancement and PET fixation) |
| Prognosis according to PET-RMI patterns | 15-32 months | Comparison of event rates (death, heart transplantation, resuscitated sudden death, hemodynamically unstable VT, syncopal VT, hospitalization for heart failure, myocarditis) observed during follow-up according to the different expected LV and RV PET-MRI profiles observed at inclusion |
| Cardiac auto-antibodies according to PET-RMI patterns | one hour | Comparison of the rates of patients with circulating cardiac autoantibodies detected by indirect immunofluorescence technique according to the different expected LV and RV PET-MRI profiles at inclusion |
| Spatial concordance | one hour | Rate of spatial concordance of segments with late enhancement and PET fixation on bulls-eye representations according to AHA segmentation |
Countries
France
Contacts
Primary ContactNicolas Piriou, PH
Backup ContactAurélie Thollet
Outcome results
None listed