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. 2023 Jun 30;25(6):euad156. doi: 10.1093/europace/euad156

Figure 6.

Figure 6

Ventricular arrhythmia induction in CRDS. (A) Ventricular fibrillation induction in CRDS using programmed ventricular stimulation. Shown are the ECG tracings of a CRDS proband carrying the RYR2-T4196I LOF variant. Ventricular arrhythmia is reproducibly precipitated using the LBLPS stimulation protocol (adapted with permission from Sun et al.83). (B) Spontaneous VF initiation in CRDS. Implantable cardioverter defibrillator tracings from a CRDS proband harbouring a RYR2 LOF variant. Shown is an episode of ventricular fibrillation preceded by sinus tachycardia. Ventricular tachycardia is then initiated by a sequence of two premature ventricular complexes, a long pause, a sinus beat, and a shorter coupled PVC (adapted with permission from Roston et al.82). (C) Effects of quinidine and flecainide on VA in D4646A +/− mutant mice. The tracing in the top row displays typical VF induction using a LBLPS stimulation (mice treated with H2O control). Tracings from the second and third row demonstrate a dose-dependent (10 mg/kg vs. 40 mg/kg per day) suppression of inducible ventricular arrhythmia after pretreatment with quinidine sulphate for 6 days. Similar effects on VA inducibility were also observed in mice after pretreatment with different doses (4 mg/kg vs. 20 mg/kg per day) of flecainide for 6 days. LBLPS, long-burst, long-pause, and short-coupled ventricular extra stimulus; PVC, premature ventricular contraction; VA, ventricular arrhythmia; VF, ventricular fibrillation (adapted with permission from Sun et al.83).