Division of Pediatric Cardiology, Department of Pediatrics, Comer Children's Hospital and the Pritzker School of Medicine of the University of Chicago, Chicago, Illinois.
Center for Arrhythmia Care, Heart and Vascular Center, Pritzker School of Medicine of the University of Chicago, Chicago, Illinois.
Pacing Clin Electrophysiol. 2020 May;43(5):527-533. doi: 10.1111/pace.13913. Epub 2020 May 2.
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 (I ), 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.
儿茶酚胺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性运动、使用β受体阻滞剂和氟卡尼等药物以及颈交感神经切除术。如果对这些策略的反应不足,则会使用植入式心脏复律除颤器 (ICD);然而,除了常见的并发症外,CPVT 患者使用 ICD 还与危及生命的电风暴风险增加相关。伊伐布雷定是一种超极化激活环核苷酸门控钾通道 4 产生有趣电流 (I) 的选择性抑制剂,已被证明可有效抑制不适当的窦性心动过速、结性心动过速、房性心动过速和室性心动过速。我们报告了一例 18 岁男性,其 CPVT 表型严重,对氟卡尼、纳多洛尔和交感神经切除术均无反应,在开始使用伊伐布雷定后,其室性心律失常得到抑制。这些发现很重要,因为伊伐布雷定可能是药物难治或无法按推荐剂量继续常规治疗的 CPVT 患者的重要附加治疗。