80 GENETIC MYOCARDITIS: ADVANCED WORKUP AND TREATMENT

Abstract Background Preclinical data support the rationale for targeting myocardial inflammation in genetic nonischemic cardiomyopathies. However, no consistent clinical reports have been provided so far. Methods We describe a series of patients (n=25) with cardiomyopathy and active (AMI) proven by multimodal diagnostic workup. Patient-tailored immunosuppressive therapy was empirically started to target whenever feasible. Multiple outcomes were retrospectively assessed dedicated multidisciplinary disease unit. Results Patients carrying desmosomal (DGV), cytoskeletal (CGV) membrane gene variants (MGV), 12 (48%), 10 (40%), 3 (12%), respectively. DGV carriers uniformly presented myocarditis-like chest pain ventricular arrhythmias, whereas heart failure symptoms found only CGV carriers. Dilated phenotype ring-like pattern on cardiac magnetic resonance allowed best discrimination among genotypes. AMI endomyocardial biopsy all cases, noninvasive imaging 21 (83%). IST 17 (71%) safety issues. By end follow-up (median 69 months, range 21-182), signs documented 6/18 receivers (33%) 4/7 untreated cases (57%). For most genotypes, paucity adverse events noted while immunosuppression as compared off-treatment period. Conclusion Our preliminary provide research spectrum