Clinical approach to the patient with Brugada Syndrome: risk stratification and optimal management

Abstract

The Brugada Syndrome (BrS) is an inherited cardiac ion channel disorder associated with increased risk of ventricular arrhythmias and mortality. Diagnosis is based on a characteristic electrocardiographic (ECG) pattern of coved type ST-segment elevation >2 mm followed by a negative T-wave in ≥1 of the right precordial leads V1 to V3. Since the first description of BrS, the definition of disease and underlying pathophysiological mechanisms have been significantly improved in recent years. Also, significant progress has been made in the field of genetic testing in these patients. Still, there are several open questions regarding the management and outcome of these patients. There is more information about patients who would need an implantable cardiac defibrillator for the primary prevention of sudden cardiac death (that is, those with spontaneous Type I Brugada ECG pattern and arrhythmia-related syncope), but currently published data concerning asymptomatic patients with Brugada ECG pattern and other less-well defined presentations are conflicting. Whereas the role of cardiac defibrillator in patients with Brugada Syndrome is clear, optimal use of catheter ablation and antiarrhythmic drug therapy needs to be further investigated. In this review, we summarize current evidence and contemporary management of patients with BrS.