Journal of cardiovascular electrophysiology · 2026
Long-Term Serial Exercise Stress Testing in Catecholaminergic Polymorphic Ventricular Tachycardia on Beta-Blocker and Flecainide Therapy.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Wangüemert-Perez Fernando, Ostos-Cañero German, Wangüemert-Guerra Marta, Acosta-Materan Carlos, Cárdenes-León Aridane, Caballero-Dorta Eduardo, Pérez-Rodríguez Kevin, Brugada Ramón, Brugada Josep, Martínez-Quintana Efrén
Аннотация
INTRODUCTION: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a potentially life-threatening arrhythmic disorder typically treated with beta-blockers and, occasionally, with flecainide.
METHODS: All patients underwent genetic testing, electrocardiogram, echocardiogram, and exercise testing. Ventricular arrhythmias were assessed using qualitative and quantitative scoring systems. Flecainide dosing was progressively titrated, and follow-up extended from 2007 to 2024.
RESULTS: Among 235 genetically confirmed carriers of the RyR2 (ryanodine receptor 2) p.Gly357Ser mutation, 32 required combination therapy with beta-blockers and flecainide (age at diagnosis 18 [1-55] years; age at flecainide initiation 32 [15-66] years; 50% male). Forty-seven percent had an implantable cardioverter defibrillator (ICD). Flecainide was indicated for exercise-induced ventricular arrhythmias despite beta-blocker therapy, and the median treatment duration was 7.3 years. All patients received propranolol (median dose 65 mg/day). Flecainide (median dose 100 mg/day) was well tolerated, with no syncope or stress-induced symptoms. Before flecainide, five patients (16%) experienced ventricular arrhythmic events recorded by the ICD, including two requiring appropriate shocks; no events occurred after treatment initiation. Both qualitative (2.07 ± 0.77 vs. 1.22 ± 1.08, p < 0.001) and quantitative (69.78 ± 83.17 vs. 15.29 ± 5.53, p < 0.001) arrhythmic scores improved significantly. Additionally, maximum heart rate and the percentage of age-predicted maximum heart rate were significantly reduced, while metabolic equivalents increased significantly (12.3 ± 3.8 vs. 14.7 ± 7.5; p = 0.010).
CONCLUSION: In CPVT patients, the addition of flecainide to beta-blocker therapy was associated with a significant reduction in arrhythmic burden and improvement in exercise-related parameters during long-term follow-up.
METHODS: All patients underwent genetic testing, electrocardiogram, echocardiogram, and exercise testing. Ventricular arrhythmias were assessed using qualitative and quantitative scoring systems. Flecainide dosing was progressively titrated, and follow-up extended from 2007 to 2024.
RESULTS: Among 235 genetically confirmed carriers of the RyR2 (ryanodine receptor 2) p.Gly357Ser mutation, 32 required combination therapy with beta-blockers and flecainide (age at diagnosis 18 [1-55] years; age at flecainide initiation 32 [15-66] years; 50% male). Forty-seven percent had an implantable cardioverter defibrillator (ICD). Flecainide was indicated for exercise-induced ventricular arrhythmias despite beta-blocker therapy, and the median treatment duration was 7.3 years. All patients received propranolol (median dose 65 mg/day). Flecainide (median dose 100 mg/day) was well tolerated, with no syncope or stress-induced symptoms. Before flecainide, five patients (16%) experienced ventricular arrhythmic events recorded by the ICD, including two requiring appropriate shocks; no events occurred after treatment initiation. Both qualitative (2.07 ± 0.77 vs. 1.22 ± 1.08, p < 0.001) and quantitative (69.78 ± 83.17 vs. 15.29 ± 5.53, p < 0.001) arrhythmic scores improved significantly. Additionally, maximum heart rate and the percentage of age-predicted maximum heart rate were significantly reduced, while metabolic equivalents increased significantly (12.3 ± 3.8 vs. 14.7 ± 7.5; p = 0.010).
CONCLUSION: In CPVT patients, the addition of flecainide to beta-blocker therapy was associated with a significant reduction in arrhythmic burden and improvement in exercise-related parameters during long-term follow-up.
Предметные рубрики MeSH
HumansFlecainideTachycardia, VentricularPolymorphic Catecholaminergic Ventricular TachycardiaExercise TestMiddle AgedAdrenergic beta-AntagonistsTreatment OutcomeAnti-Arrhythmia AgentsTime FactorsFemaleAdultMaleAdolescentYoung AdultRyanodine Receptor Calcium Release ChannelChildAgedHeart RateChild, PreschoolPredictive Value of TestsDrug Therapy, CombinationMutationPropranolol