Key result
Combined beta-blocker and flecainide therapy significantly reduced the risk of cardiac events compared to beta-blocker monotherapy (HR 0.08) in children with catecholaminergic polymorphic ventricular tachycardia.
Why the study?
The major CPVT-causative gene has been associated with neuropsychiatric manifestations, prompting an analysis of clinical presentations, neuropsychiatric manifestations, and treatment outcomes in children with CPVT.
Does the addition of flecainide and/or left cardiac sympathetic denervation to beta-blocker therapy reduce cardiac events and ventricular arrhythmia burden in children with catecholaminergic polymorphic ventricular tachycardia?
Cohort (n=23)
No
Does the addition of flecainide and/or left cardiac sympathetic denervation to beta-blocker therapy reduce cardiac events and ventricular arrhythmia burden in children with catecholaminergic polymorphic ventricular tachycardia?
Hazard Ratio: 0.08 (95% CI 0.02–0.38)
p-value: p=0.002
In children with CPVT, the addition of flecainide or left cardiac sympathetic denervation to beta-blocker therapy significantly reduces the risk of cardiac events and ventricular arrhythmia burden.
BACKGROUND AND OBJECTIVES: , the major CPVT-causative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT. METHODS: We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated. RESULTS: variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028). Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02-0.38; p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23-179.95; p<0.001). CONCLUSIONS: Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.
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Lee et al. (2024) conducted a cohort in Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) (n=23). Combined beta-blocker and flecainide therapy vs. Beta-blocker monotherapy was evaluated on Cardiac events (composite of presumed cardiac death, aborted cardiac arrest, appropriate ICD shock, or syncope of cardiac origin) (HR 0.08, 95% CI 0.02-0.38, p=0.002). Combined beta-blocker and flecainide therapy significantly reduced the risk of cardiac events compared to beta-blocker monotherapy (HR 0.08) in children with catecholaminergic polymorphic ventricular tachycardia.
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