Brugada Syndrome is a condition in which abnormal sodium channel proteins can result in ventricular arrhythmias and sudden cardiac death in a patient with a structurally normal heart.
It is associated with 3 ECG patterns, the classic being partial/RBBB and ST elevation in the right precordial leads.
Prevalance is estimated at 5 per 10000.
It is commonest in Asians.
Men are 8 to 10 times more affected than women phenotypically, although the genotype prevalence is the same for both genders.
Men are 8 to 10 times more affected than women phenotypically, although the genotype prevalence is the same for both genders.
Around 30% of cases are associated with a SCN5A mutation. This is autosomal dominant.
Sudden cardiac death from Brugada is commonest in the 4th decade
of life. Around 1 in 5 patients with sudden cardiac death from Brugada
would have previously had a syncopal episode. Interestingly arrhythmias
associated with Brugada tend to occur during periods of vagal dominance,
ie at rest or asleep.
Ajmaline is the ‘best’ drug to unmask Brugada syndrome. Other drugs in use include flecainide and procainamide.
Treatment is:
a) to avoid precipitants of arrhythmias
a) to avoid precipitants of arrhythmias
- fever
- drugs such as flecainide, ajmaline, procainamide, propafenone, lithium, amitriptyline
b) ICD
Small print gem:
It is a relatively new condition, first described in 1992, and has
other names including lai tai in Thailand and pokkuri in
Japan
References
Postema, P. et al. Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date website. Heart Rhythm. 2009; 6(9): 1335-41
