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Effects of flecainide on exercise-induced ventricular arrhythmias and recurrences in genotype-negative patients with catecholaminergic polymorphic ventricular tachycardia.

机译:氟卡尼对患有儿茶酚胺能性多形性室性心动过速的基因型阴性患者运动性心律失常和复发的影响。

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摘要

BACKGROUND: Conventional therapy with beta-blockers is incompletely effective in preventing arrhythmic events in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). We have previously discovered that flecainide in addition to conventional drug therapy prevents ventricular arrhythmias in patients with genotype-positive CPVT.OBJECTIVE: To study the efficacy of flecainide in patients with genotype-negative CPVT.METHODS: We studied the efficacy of flecainide for reducing ventricular arrhythmias during exercise testing and preventing arrhythmia events during long-term follow-up.RESULTS: Twelve patients with genotype-negative CPVT were treated with flecainide. Conventional therapy failed to control ventricular arrhythmias in all patients. Flecainide was initiated because of significant ventricular arrhythmias (n = 8), syncope (n = 3), or cardiac arrest (n = 1). At the baseline exercise test before flecainide, 6 patients had ventricular tachycardia and 5 patients had bigeminal or frequent ventricular premature beats. Flecainide reduced ventricular arrhythmias at the exercise test in 8 patients compared to conventional therapy, similar to that in patients with genotype-positive CPVT in our previous report. Notably, flecainide completely prevented ventricular arrhythmias in 7 patients. Flecainide was continued in all patients except for one who had ventricular tachycardia at the exercise test on flecainide. During a follow-up of 48±94 months, arrhythmia events (sudden cardiac death and aborted cardiac arrest) associated with noncompliance occurred in 2 patients. Flecainide was not discontinued owing to side effects in any of the patients.CONCLUSIONS: Flecainide was effective in patients with genotype-negative CPVT, suggesting that spontaneous Ca(2+) release from ryanodine channels plays a role in arrhythmia susceptibility, similar to that in patients with genotype-positive CPVT.
机译:背景:使用β-受体阻滞剂的常规疗法在预防儿茶酚胺能性多形性室性心动过速(CPVT)患者的心律失常事件方面并不完全有效。我们以前已经发现氟卡尼除常规药物治疗外还可以预防基因型阳性CPVT患者的室性心律失常。目的:研究氟卡尼在基因型阴性CPVT患者中的疗效。方法:我们研究了氟卡尼对减轻心室功能的疗效。运动测试中出现心律不齐,并在长期随访中预防心律失常。结果:氟卡尼胺治疗了12例基因型阴性CPVT患者。常规疗法未能控制所有患者的室性心律失常。因严重的室性心律不齐(n = 8),晕厥(n = 3)或心脏骤停(n = 1)而开始使用氟卡尼特。在氟卡尼治疗之前进行的基线运动测试中,有6例患者出现室性心动过速,有5例患者出现双胎性或频繁性室性早搏。与常规疗法相比,氟卡尼胺在运动试验中减少了8例患者的室性心律失常,与我们先前报告中基因型阳性CPVT的患者相似。值得注意的是,氟卡尼可完全预防7例患者的室性心律失常。除一名患者在使用氟卡尼的运动试验中出现室性心动过速外,所有患者均继续使用氟卡尼。在48±94个月的随访中,有2例患者发生了与不依从相关的心律失常事件(心脏猝死和心脏骤停中止)。结论:Flecainide在基因型阴性CPVT患者中有效,提示从ryanodine通道自发释放Ca(2+)在心律失常易感性中发挥作用,类似于在任何患者中均未停用氟卡尼德。基因型阳性CPVT患者。

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