Family history of SCD or syncope (risk ratio [RR] 4.97, 95% CI 3.96-6.23, P < 0.001), inducible ventricular arrhythmia on EPS (RR 3.56, 95% CI 1.30-9.74, P = 0.01), and spontaneous type 1 Brugada ECG (RR 4.07, 95% CI 2.23-7.41, P < 0.001) were associated with an increased risk of future cardiac events.
Desmosomal and LMNA gene variants identify the subset of DCM patients who are at greatest risk for SCD and life-threatening ventricular arrhythmias, regardless of the left ventricular ejection fraction.