Elsevier

Heart, Lung and Circulation

Volume 24, Issue 12, December 2015, Pages 1141-1148
Heart, Lung and Circulation

Position Statement
Update on the Diagnosis and Management of Brugada Syndrome

https://doi.org/10.1016/j.hlc.2015.07.020Get rights and content
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Brugada Syndrome (BrS) is an autosomal dominant channelopathy with variable penetrance affecting the sodium channel. It reduces the transport of sodium ions essential for proper generation of the cardiac action potential. The resulting inhomogeneous repolarisation in areas of the RV epicardium causes malignant ventricular arrhythmias.

BrS is diagnosed by typical cove shaped ST elevation of > 2 mm in ≥1 RV precordial lead V1, V2 occurring spontaneously or after provocative drug test with IV administration of Class 1 antiarrhythmic drug such as flecainide or ajmaline.

The incidence of BrS is variable being higher in South East Asians and is generally quoted as 1:2000. It is responsible for up to 20% of sudden arrhythmic deaths in those without structural heart disease. Typical presentation is syncope or resuscitated sudden death and symptoms usually occur at night or at rest especially after a large meal. Fever is a common trigger, particularly in children.

Genetic testing for BrS is a Class 2A indication and the yield has increased recently to nearly 40%. Genetic testing assists with family screening.

Keywords

Brugada Syndrome
Atrial fibrillation
Genetic testing
Sudden death
Syncope
Heart disease

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