Patients with a DPP6 risk- haplotype for familial idiopathic ventricular fibrillation have normal left systolic function but abnormal deformation

Abstract Funding Acknowledgements Type of funding sources: None. Introduction An aberrant DPP6 mutation haplotype on chromosome 7 is associated with familial idiopathic ventricular fibrillation in severely affected Dutch families numerous cases sudden cardiac death (SCD) So far, no clinical parameters could be linked to predict SCD risk other than this genetic predisposition. In various disease, global longitudinal strain (GLS) and mechanical dispersion (MD), both markers left (LV) dysfunction, are predictive tools for patients at arrhythmias (VA) SCD. The goal the current study was investigate whether LV detected by GLS MD, present haplotype. Method risk-haplotype carriers sufficient echocardiographic images were included as (n = 31, 15 males mean age 41 ± 11 years) individuals evaluated during cascade screening who tested but appeared not controls 14, males, 39 12 years). Echocardiographic bi-plane ejection fraction (LVEF), MD determined. Results LVEF similar between (57.3%) (60.5%, p 0.07). contrast, (-18.0%) significant lower compared (-21.1%, < 0.0001). (43.4ms) significantly higher (26.7ms, Conclusion risk- have LVEF, LV-GLS controls. These findings can used discrimination. Whether these prediction events has determined after a longer follow up. characteristics p-value (%) 60.5 57.3 0.07 -21.2 -18.0 0.0001 ( ms) 26.7 43.4