CE-452775-1 PREDICTORS FOR SUDDEN CARDIAC DEATH AND SUSTAINED VENTRICULAR ARRHYTHMIAS AMONG HIGH-RISK PATIENTS WITH ARRHYTHMOGENIC MITRAL VALVE PROLAPSE SYNDROME

Although mitral valve prolapse (MVP) is a benign entity overall, there small subset of patients with MVP, particularly bileaflet who may experience life-threatening ventricular arrhythmias (VAs) secondary to arrhythmogenic MVP syndrome. Studies characterizing this group have been limited by sample sizes and single center design. To assemble worldwide case series examining the clinical phenotype syndrome including clinical, imaging, treatment characteristics predictors sudden cardiac death (SCD). We retrospectively identified subjects VAs across 17 international centers. stratified our into two groups: 1) MVP-SCD death, sustained tachycardia (VT), or fibrillation (VF); 2) MVP-PVC significant (symptomatic, >5% burden, bigeminy, pleomorphic, requiring therapy) premature complexes (PVCs) only. Deidentified data was abstracted locally combined central registry for analysis. A total 217 were included; 148 (68%) had history SCD VT/VF (MVP-SCD group) 69 (32%) PVCs (MVP-PVC group). Phenotypically, in both groups similar (average age 44 years, 66% female, 72% 58% annular disjunction, 56% inferolateral T-wave inversions, 44% delayed enhancement on magnetic resonance imaging). Syncope more common than (47% vs. 22%, p=0.001) as anterolateral inversions (22% vs 7%, p=0.011). Subjects less likely prescribed beta blockers (35.7% 51.5%, p=0.029) significantly prior surgery (5.5% 19.7%, p=0.002). When completed, an electrophysiology (EP) study positive 42/72 (58%). However, 15/45 (33.3%) negative EP study. In large series, young, disjunction. syncope associated VT/VF. Prior blocker prescription PVC patients. predictive value high-risk