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Results Overall, 30/91 (33%) patients with LQT3 exhibited evidence of presumed PSH [fascicular PVCs 30/30 (100%); short-coupled VAs 17/30 (56%)]. Kaplan-Meier and Cox regression analyses demonstrated an increased risk of VF events in individuals with LQT3 and PSH (log rank p less then 0.03; HR=3.95, 95% CI 1.15-15.7, p=0.03). Interestingly, variants in the voltage-sensing domain regions of Nav1.5 were more frequently observed in LQT3 patients with PSH than those without [19/30 (63%) vs. 9/61 (15%); p less then 0.0001]. Conclusions This
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