LAUSR

BACKGROUND Despite a strong genetic background, Brugada syndrome (BrS) mainly affects middle-aged patients. Data are scarce in the youngest and oldest age groups. OBJECTIVE To describe the clinical characteristics and the variations in rhythmic risk in BrS patients according to age. METHODS Consecutive BrS patients diagnosed in 15 French tertiary centres in France were enrolled from 1993 to 2016 and followed up prospectively. All of the clinical and ECG data were double reviewed. RESULTS Among the 1,613 patients enrolled (45±15 years, 69% male), three groups were defined according to age (52 patients < 17 years, 1,285 between 17 and 59 years and 276 > 60 years). In the youngest patients, we identified more female gender (42%), diagnosis by familial screening (63%), previous SCD (15%), SCN5A mutation (62%) sinus dysfunction (8%) and aVR sign (37%) (P < 0.001). The oldest patients had the same clinical characteristics except for gender (40% women; P < 0.001). During a median follow-up of 5.5 [2.1;10.0]years, 91 patients experienced an arrhythmic event including 7(13%) in the youngest patients, 80 (6%) in middle-aged patients and 4 (1%) n the oldest patients. Annual event rates were respectively 2.1%, 1% and 0.3% (P < 0.01). CONCLUSIONS Age on diagnosis changes the clinical presentation of BrS. Although children are more identified during familial screening, they present the highest risk of SCD which is an argument for early and extensive familial screening. The oldest patients present the lowest risk of SCD.