Wangüemert Pérez Fernando, Hernández Afonso Julio Salvador, Groba Marco María Del Val, Caballero Dorta Eduardo, Álvarez Acosta Luis, Campuzano Larrea Oscar, Pérez Guillermo, Brugada Terradellas Josep, Brugada Terradellas Ramón
Departamento de Cardiología, Cardiavant Centro Médico Cardiológico, Las Palmas de Gran Canaria, Las Palmas, Spain.
Departamento de Cardiología, Hospital Universitario Nuestra Señora de Candelaria, Santa Cruz de Tenerife, Spain.
Rev Esp Cardiol (Engl Ed). 2018 Mar;71(3):185-191. doi: 10.1016/j.rec.2017.04.032. Epub 2017 Aug 5.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disease characterized by polymorphic or bidirectional ventricular arrhythmias (VA) triggered by physical or emotional stress in young people with a structurally normal heart. Beta-blockers are the cornerstone of treatment, while flecainide has recently been incorporated into the therapeutic arsenal. The aim of this study was to report our experience with this drug.
The cohort included 174 genotype-positive CPVT-patients from 7 families. We collected data from patients who were receiving flecainide and analyzed the indications, adverse effects and dosage, clinical events, VA and arrhythmic window during exercise testing, and implantable cardioverter-defibrillator (ICD) shocks during follow-up.
Eighteen patients (10.4%) received flecainide; 17 patients in combination with beta-blockers, and 1 patient as monotherapy due to beta-blocker intolerance. None of the patients presented side effects. In 13 patients (72.2%) the indication was the persistence of exercise-induced VA and in 5 patients (27.7%) persistent ICD-shocks, despite on beta-blockers. After flecainide initiation, the exercise-induced VA quantitative score was reduced by more than 50% in 66.7% of the members of family 1 (32.76 ± 84.06 vs 74.38 ± 153.86; P = .018). The arrhythmic window was reduced (5.8 ± 11.9 bpm vs 19.69 ± 21.27 bpm; P = .007), and 4 of 5 patients with appropriate ICD shocks experienced no further shocks in the follow-up.
In CPVT-patients flecainide reduces clinical events, exercise-induced VA, the arrhythmic window, and ICD shocks, with good tolerance.
儿茶酚胺能多形性室性心动过速（CPVT）是一种遗传性疾病，其特征为在心脏结构正常的年轻人中，由身体或情绪应激引发多形性或双向性室性心律失常（VA）。β受体阻滞剂是治疗的基石，而氟卡尼最近也被纳入了治疗手段。本研究的目的是报告我们使用这种药物的经验。
该队列包括来自7个家族的174例基因阳性的CPVT患者。我们收集了接受氟卡尼治疗的患者的数据，并分析了其适应证、不良反应及剂量、临床事件、运动试验期间的VA和心律失常窗口，以及随访期间植入式心律转复除颤器（ICD）电击情况。
18例患者（10.4%）接受了氟卡尼治疗；17例患者与β受体阻滞剂联合使用，1例患者因不耐受β受体阻滞剂而作为单一疗法使用。所有患者均未出现副作用。13例患者（72.2%）的适应证为运动诱发的VA持续存在，5例患者（27.7%）尽管使用了β受体阻滞剂仍有持续性ICD电击。开始使用氟卡尼后，家族1中66.7%的成员运动诱发的VA定量评分降低了50%以上（32.76±84.06对74.38±153.86；P = 0.018）。心律失常窗口缩小（5.8±11.9次/分对19.69±21.27次/分；P = 0.007），5例接受适当ICD电击的患者中有4例在随访中未再出现电击。
在CPVT患者中，氟卡尼可减少临床事件、运动诱发的VA、心律失常窗口及ICD电击，耐受性良好。
