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.
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.
Study Type
OBSERVATIONAL
Enrollment
80
serum
CHU Angers
Angers, France
RECRUITINGCHU Brest
Brest, France
RECRUITINGCHU de Nantes
Nantes, France
RECRUITINGAP-HP La Salpêtrière
Paris, France
RECRUITINGCHU de Rennes
Rennes, France
RECRUITINGPET-RMI patients profile
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).
Time frame: one hour
PET-RMI patterns in the right ventricle
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)
Time frame: one hour
Prognosis according to PET-RMI patterns
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
Time frame: 15-32 months
Cardiac auto-antibodies according to PET-RMI patterns
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
Time frame: one hour
Spatial concordance
Rate of spatial concordance of segments with late enhancement and PET fixation on bulls-eye representations according to AHA segmentation
Time frame: one hour
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