Abstract 2440: Clinical Presentation and Outcomes in Children Affected by a Brugada Syndrome
Introduction. Brugada syndrome (BrS) is an arrhythmogenic disease characterized by ST-segment elevation in the right precordial leads, conduction slowing and a high risk of sudden cardiac death. In its first description, BrS included two young children but little is known about clinical presentation of BrS in the pediatric population.
Methods and results. We identified 29 children younger than 16 presenting a type I EKG. The population included 16 males and 13 females with a mean age at diagnosis of 8±5 years. A family history of BrS was found in 24 out of 29 patients. The diagnosis of BrS was made in 17 cases during a familial screening, in 9 cases after syncope, in 1 case after aborted sudden cardiac death and in 2 cases after symptomatic supraventricular tachycardia’s. Ventricular arrhythmic events were triggered by fever in 5 cases (50%). Among the 16 patients who had exhibited a spontaneous type I EKG, 10 were symptomatic (62%) while only 1 was symptomatic among the 13 patients who had a type I EKG only after ajmaline challenge (8%). A genetic screening for SCN5A mutation was performed in 22 patients and was positive in 16. The PR interval (163±31 ms) and the QRS interval (105±17 ms) were longer than in an age-matched control child population belonging to the same families (PR 144±25 ms; p<0.01 and QRS 86±15 ms; p<0.0001). An electrophysiological study was performed in 6 children and induced sustained ventricular fibrillation in 3 (50%). An ICD was implanted in 4 children (3 symptomatic and 1 asymptomatic), hydroquinidine was given to 3 children and 1 child was implanted with a pacemaker for symptomatic sinus node dysfunction. During a mean follow-up of 38±22 months, 3 children with prior symptoms and a spontaneous type 1 EKG experienced arrhythmic events (2 appropriate shocks and 1 sudden death). One child had an ICD infection requiring the removoal of the device.
Conclusion: In this population of pediatric BrS patients, fever triggered the arrhythmic events in 50% of the cases. Like in the adult population, the risk of arrhythmic event seems to be higher in case of symptoms and spontaneous type I EKG.