Conventional treatment strategies for catecholaminergic polymorphic ventricular tachycardia (CPVT) include avoidance of strenuous exercise and competitive sports, drugs such as ß-blockers and flecainide and, cervical sympathectomy. An implantable cardioverter-defibrillator (ICD) has been utilized if the response to these strategies is inadequate; however, ICD use in CPVT patients, in addition to usual complications, is associated with an increased risk of life-threatening electrical storm. Ivabradine is a selective inhibitor of hyperpolarization-activated cyclic nucleotide-gated potassium channel 4 generated funny current (If ), which has been shown to be efficacious in suppression of inappropriate sinus tachycardia, junctional tachycardia, atrial tachycardia, and ventricular ectopy in humans. We report an 18-year-old male with a severe CPVT phenotype refractory to flecainide, nadolol, and sympathectomy who exhibited suppression of ventricular arrhythmias after initiation of ivabradine. These findings are of importance as ivabradine could be an important add-on therapy in CPVT patients who are drug refractory or are unable to continue conventional therapies at the recommended doses.

中文翻译：

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儿茶酚胺能多形性室性心动过速对纳多洛尔，氟卡尼特和交感神经切除均难治的患者，使用伊伐布雷定抑制室性心律失常。

儿茶酚胺能性多形性室性心动过速（CPVT）的常规治疗策略包括避免剧烈运动和竞技运动，避免使用ß-blocker和flecainide等药物以及进行宫颈交感神经切除术。如果对这些策略的反应不充分，则可以使用植入式心脏复律除颤器（ICD）。然而，除了通常的并发症外，CPVT患者使用ICD还增加了危及生命的电风暴的风险。伊伐布雷定是超极化激活的环状核苷酸门控钾通道4产生的滑稽电流（If）的选择性抑制剂，已被证明可有效抑制人类不适当的窦性心动过速，结节性心动过速，心房性心动过速和心室异位。我们报道了一名18岁的男性，患有严重的CPVT表型，对氟卡尼，纳多洛尔和交感神经切除术具有难治性，他们在伊伐布雷定启动后表现出对室性心律失常的抑制作用。这些发现非常重要，因为伊伐布雷定可能是CPVT难治性或无法以推荐剂量继续常规治疗的CPVT患者的重要附加疗法。