Key result
Epinephrine infusion induced delayed afterdepolarizations in 26.7% of CPVT patients with RyR2 mutations compared to 0% of control subjects.
Population
15 CPVT patients with a RyR2 mutation and 12 control subjects; HEK 293 cells expressing mutant RyR2 or…
Comparison
Epinephrine infusion in humans; Membrane… vs Control subjects; wild-type RyR2; baseline…
Design
Case-control
Authors
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Supports RyR2 gain-of-function in CPVT arrhythmogenesis; hypothesis-generating and should not yet alter clinical practice.
Case-Control (n=27)
Absolute Event Rate: 26.7% vs 0%
Mutant RyR2s in CPVT patients exhibit an increased sensitivity to cAMP, leading to delayed afterdepolarizations and spontaneous Ca2+ waves that may explain exercise-induced arrhythmias.
Paavola et al. (2007) conducted a case-control in Catecholaminergic polymorphic ventricular tachycardia (CPVT) (n=27). Epinephrine infusion vs. Control subjects was evaluated on Delayed afterdepolarizations (DADs). Epinephrine infusion induced delayed afterdepolarizations in 26.7% of CPVT patients with RyR2 mutations compared to 0% of control subjects.
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