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External validation of risk factors for malignant ventricular arrhythmias in lamin A/C mutation carriers

Background International recommendations state that implantable cardiovertor defibrillator should be considered early in patients with a confirmed disease-causing LMNA mutation with clinical risk factors, especially in the presence of non-sustained ventricular tachycardia, LVEF  Objective To evaluate the performance of the 4 prognosis factors on a separate external cohort of LMNA patients. Methods We identified 101 consecutive LMNA mutation carriers who have been managed and genotyped in our center between 2004 and 2015. We recorded for all patients the values of the 4 previous identified predictive factors and whether they experienced a malignant ventricular aarhythmia (MVA) or not. Results The mean follow-up was 4.7 years. MVA was oberserved in 16 patients. Four risk groups were defined from the four previous predictive factors and log-rank test showed statistical significance (P  Fig. 1 ). Conclusion The 4 risk factors (non-sustained ventricular tachycardia, LVEF