Figure 3.

Cellular electrical abnormalities and their relation to changes in the ECG. (Left) At fast pacing (heart) rates, mutation-induced changes in epicardial AP morphologies (thick line) cause dispersion of plateau potentials and a voltage gradient (∇V_m, arrow) (WT, thin line). This gradient will manifest on the ECG as ST segment elevation, indicative of BrS. (Middle) Mutations may prolong APD in M cells (thick line) compared to WT (thin line). The delay in repolarization (ΔAPD ∼ 60ms) is reflected as QT prolongation on the ECG, a hallmark of LQTs. (Right) A rightward shift in the Na⁺ channel activation curve is sufficient to reduce AP upstroke and slow cardiac impulse conduction (wide QRS segment) as observed in ICCD. WT, wild-type.