International audienceBACKGROUNG: Brugada Syndrome (BrS) is an arrhythmogenic disease associated with sudden cardiac death (SCD) which seldom manifests or is recognized in childhood. OBJECTIVE: To describe the clinical presentation of pediatric BrS to identify prognostic factors for risk stratification, and propose a data-based approach management. METHODS: We studied 106 patients, under 19 years of age at diagnosis of BrS from 16 European hospitals. RESULTS: At diagnosis, BrS was spontaneous (n=36) or drug-induced (n=70). Mean age was 11.1±5.7 years and most patients were asymptomatic [family screening (n=67), incidental (n=13)] while 15 had experienced syncope, 6 aborted SCD or symptomatic ventricular tachycardia and 5 others symptoms. During follow-up (median: 54 months), 10 patients had life-threatening arrhythmias (LTA) including 3 deaths. Six experienced syncope and 4 SVT. Fever triggered 27% of LTA events. An ICD was implanted in 22 with major adverse events in 41%. Of the 11 patients treated with hydroquinidine, 8 remained asymptomatic. Genetic testing was performed in 75 patients and SCN5A rare variants were identified in 58; 15 out of 32 (47%) tested probands were genotype positive. Nine out of the 10 patients with LTA underwent genetic testing and all were genotype positive whereas the 17 SCN5A negative patients remained asymptomatic. Spontaneous BrS type 1 ECG (p=0.005) and symptoms at diagnosis (p=0.001) were predictors of LTA. Time to the first LTA event was shorter in patients with both symptoms at diagnosis and spontaneous BrS type 1 ECG pattern (p=0.006). CONCLUSIONS: Spontaneous type 1 ECG and symptoms at diagnosis are predictors of LTA events in the young affected by BrS. The management of BrS should become age-specific and prevention of SCD may involve genetic testing, aggressive use of anti-pyretics and quinidine with risk-specific consideration for the IC
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