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No abstract

Symptomatic BS in the young age is a rare but malignant condition that can manifest with a spectrum of electrical abnormalities (i.e. SND, atrial tachycardias, AV block, and infra-nodal conduction delay) and result in the extreme cases in lethal arrhythmic events and SCD.

Risk stratification in Brugada syndrome in young patients remains challenging. We investigated the clinical characteristics, prognosis, and risk in young patients with the Brugada syndrome. We studied 95 patients with the Brugada syndrome aged ≤19 years. The median age at diagnosis was 12.9 years. The clinical presentation was sudden cardiac death in 7% and syncope in 21%. The remaining 72% were asymptomatic at diagnosis. Electrical abnormalities were present in 36%, including spontaneous type I electrocardiogram (12%), sinus node dysfunction (9%), atrioventricular block (17%), intraventricular conduction delay (16%), and atrial arrhythmias (8%). An electrophysiologic study was performed in 75%; ventricular arrhythmias were induced in 3%. An implantable cardioverter-defibrillator was placed in 25%. During a mean follow-up of 59 months, 9 patients presented with arrhythmic events (event rate: 1.9% per year). Variables significantly associated with events were: presentation with sudden cardiac death or syncope, spontaneous type I electrocardiogram, sinus node dysfunction and/or atrial tachycardia, conduction abnormality, and induction of ventricular arrhythmias during programmed ventricular stimulation. A model including the previous 4 main clinical variables (1, sudden cardiac death or syncope; 2, spontaneous type I electrocardiogram; 3, sinus node dysfunction and/or atrial tachycardia; and 4, conduction abnormality) had a high predictive power (C: 0.93) for the risk of lethal events. A score of ≥4 conferred a 5-year event probability of 30% that increased to 53% if the score was ≥6. In conclusion, our study validated a model to predict risk in young patients with the Brugada syndrome, which takes into account 4 clinical measures.