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. 2016 Aug;5(2):84–89. doi: 10.15420/AER.2016.23.2

Table 1: ECG, Clinical and Genetic Features of Brugada Syndrome and Early Repolarisation Syndrome.

Brugada Syndrome ER Syndrome
ECG Features
Diagnostic pattern Type 1 ECG* ER pattern¥
Additional ECG abnormalities Type 2 ECG**, type 3*** ECG, ER pattern¥, f-QRS§ -
Dynamicity of ECG pattern over time Yes Yes
Induction of ECG pattern by sodium channel blockers Yes No
Vagal-mediated accentuation of ECG pattern Yes Yes
Age-dependent manifestation of ECG pattern Yes Unknown
Clinical Features
Male predominance Yes Yes
Mean age of first arrhythmic event 35–40 35–40
Prognostic value of VA inducibility at EP study Controversial No
Circumstances of arrhythmic episodes Rest/sleep/fever Rest
Genetic Features
Inheritance Autosomal dominant Autosomal dominant
Sporadic cases Yes Yes
Association with extra-cardiac diseases Yes (myopathies) Not established
Gene mutations SCN5A, KNCJ8, CACNA1C, CACNA2D1, CACNB2b, ABCC9, SCN10A, GpD1L, SCN1B, KCNE3, SCN3B, KCND3, RANGFR, SLMAP, SCN2B, PKP2, FGF12, HEY2,SEMA3A SCN5A, KNCJ8, CACNA1C, CACNA2D1, CACNB2b, ABCC9, SCN10A

*Type 1 ECG is characterised by a coved-type ST-segment elevation ≥2 mm in at least one right precordial lead (V1–V3), followed by symmetric negative T waves, with little or no isoelectric separation. **Type 2 ECG displays a ST-segment elevation of >2 mm in right precordial leads followed by positive or biphasic T waves, resulting in a saddleback configuration. ***Type 3 ECG is defined as any of the two previous types if ST-segment elevation is ≤1 mm. ¥ ER pattern is characterised by a notch or slur ≥1 mm, occurring on the final 50 % of the downslope of an R-wave, in two adjacent inferior (II, III and aVF), lateral (I, aVL and V4–V6), or infero-lateral leads. § f-QRS (fragmented QRS) presents as a notch midway on the downslope of an R-wave. BrS = Brugada syndrome; ER syndrome = early repolarisation syndrome.