Department of Cardiology and Angiology, University Hospital Münster, Albert-Schweitzer Strasse 33, 48149 Münster, Germany.
Europace. 2011 Jun;13(6):897-901. doi: 10.1093/europace/euq517. Epub 2011 Feb 2.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease that can cause sudden cardiac death due to ventricular fibrillation (VF). While pharmacological therapy with beta-blockers and/or Ca(2)(+) antagonists is often unreliable, a recent study has demonstrated that flecainide can effectively suppress arrhythmia in a murine model of CPVT as well as clinically in two human subjects suffering from CPVT. We here present the case of an 11-year-old boy suffering from CPVT-1 as well as a review of the current relevant literature. After resuscitation due to VF at age 9, an automated implantable cardioverter-defibrillator (ICD) was implanted in 2007. Under beta-blocker therapy, repeated shocks were delivered due to either fast ventricular tachycardia (VT) or VF. This persisted under additional therapy with verapamil. Implantable cardioverter-defibrillator routine interrogations showed frequent non-sustained VT with an average of 8.8 per day. Additionally, the patient suffered from impaired physical performance due to decreased chronotropic competence. In July 2009, flecainide was added to the beta-blocker/verapamil regimen, resulting in a plasma level of 0.20 mg/L. No ICD shock or sustained VT occurred until December 2010. Genetic testing revealed an RyR2 receptor mutation. The case demonstrates the challenge of diagnosis and management of CPVT. It furthermore supports recent experimental evidence that the class 1 antiarrhythmic drug flecainide can suppress CPVT. The presented case supports a novel strategy in treating CPVT with the class I antiarrhythmic agent flecainide.
儿茶酚胺多形性室性心动过速（CPVT）是一种遗传性心律失常疾病，可导致室颤（VF）引起的心脏性猝死。虽然β受体阻滞剂和/或钙通道拮抗剂的药物治疗通常不可靠，但最近的一项研究表明，氟卡尼可有效抑制 CPVT 小鼠模型以及 2 例 CPVT 患者的心律失常。我们在此介绍了 11 岁患有 CPVT-1 的男孩的病例，并对当前相关文献进行了综述。9 岁时因 VF 复苏后，于 2007 年植入自动植入式心脏除颤器（ICD）。在β受体阻滞剂治疗下，由于快速室性心动过速（VT）或 VF 反复发作。在维拉帕米的额外治疗下仍然如此。植入式心脏复律除颤器常规检查显示频繁的非持续性 VT，平均每天 8.8 次。此外，由于变时功能不全，患者的身体机能受损。2009 年 7 月，在β受体阻滞剂/维拉帕米治疗方案中添加氟卡尼，导致血药浓度为 0.20mg/L。直到 2010 年 12 月，均未发生 ICD 电击或持续性 VT。基因检测显示 RyR2 受体突变。该病例说明了 CPVT 的诊断和管理的挑战。它进一步支持了最近的实验证据，即 I 类抗心律失常药物氟卡尼可抑制 CPVT。所介绍的病例支持用 I 类抗心律失常药物氟卡尼治疗 CPVT 的新策略。
