More than 30 years of Brugada syndrome: a critical appraisal of achievements and open issues.

Abstract

Over the last three decades, what is referred to as Brugada syndrome (BrS) has developed from a clinical observation of initially a few cases of sudden cardiac death (SCD) in the absence of structural heart disease with ECG signs of "atypical right bundle brunch block" to a predominantly electrocardiographic, and to a lesser extent genetic, diagnosis. Today, BrS is diagnosed in patients without overt structural heart disease and a spontaneous Brugada type 1 ECG pattern regardless of symptoms. The diagnosis of BrS is less clear in those with an only transient or drug-induced type 1 Brugada pattern, but should be considered in the presence of an arrhythmic syncope, family history of BrS, or family history of sudden death. In addition to survived cardiac arrest, syncope is probably the single most decisive risk marker for future arrhythmias. For asymptomatic BrS, risk stratification remains challenging. General recommendations to lower the risk in BrS include avoidance of drugs/agents known to induce and/or increase right precordial ST-segment elevation, including treatment of fever with antipyretic drugs. Several ECG markers that have been associated with an increased risk of SCD have been incorporated into a recently published risk score for BrS. The aim of this article is to provide an overview of the status of risk stratification and to illustrate open issues und gaps in evidence in BrS.