Fernando Wangüemert Pérez1, Julio Salvador Hernández Afonso2, María Del Val Groba Marco3, Eduardo Caballero Dorta4, Luis Álvarez Acosta2, Oscar Campuzano Larrea5, Guillermo Pérez5, Josep Brugada Terradellas6, Ramón Brugada Terradellas5. 1. Departamento de Cardiología, Cardiavant Centro Médico Cardiológico, Las Palmas de Gran Canaria, Las Palmas, Spain. Electronic address: fwanguemert@mac.com. 2. Departamento de Cardiología, Hospital Universitario Nuestra Señora de Candelaria, Santa Cruz de Tenerife, Spain. 3. Departamento de Cardiología, Cardiavant Centro Médico Cardiológico, Las Palmas de Gran Canaria, Las Palmas, Spain; Departamento de Cardiología, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Las Palmas, Spain. 4. Departamento de Cardiología, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Las Palmas, Spain. 5. Departamento de Genética, Cardiovascular Genetics Center, Gencardio, Institut d'Investigacions Biomèdiques de Girona (IDIBGI), Girona, Spain. 6. Departamento de Cardiología, Institut Clínic del Tòrax, Hospital Universitario Clínic de Barcelona, Universidad de Barcelona, Barcelona, Spain.
Abstract
INTRODUCTION AND OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: In CPVT-patients flecainide reduces clinical events, exercise-induced VA, the arrhythmic window, and ICD shocks, with good tolerance.
INTRODUCTION AND OBJECTIVES: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: In CPVT-patientsflecainide reduces clinical events, exercise-induced VA, the arrhythmic window, and ICD shocks, with good tolerance.