Brugada Syndrome is associated wit cardiac conduction abnormalities, a high risk of ventricular arrhythmias and a high risk of sudden death.


Inheritance: usually autosomal dominant, less often de novo


Criteria for the Brugada syndrome:

(1) one of the 3 types of ECG

(2) one or more of the followng

(2a) documented ventricular fibrillation

(2b) self-terminating polymorphic ventricular tachycardia

(2c) a family history of sudden death

(2d) family members with coved-type ECGs

(2e) electrophysiologic inducibility

(2f) syncope

(2g) nocturnal agonal respiration

(2h) mutations in one or more of the 16 genes associated with Brugada syndrome (SCN5A, SCN1B, SCN2B, SCN3B, GPD1L, CACNA1C, CACNB2, CACNA2D1, KCND3, JCNE3, KCNE1L (KCNE5), KCNJ8, HCN4, RANGRF, SLMAP, TRPM4).

(2i) Type 2 or Type 3 ECG with conversion to Type 1 following a challenge with a sodium channel blocker

(3) no other factor can account for the ECG pattern


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