Management of Symptomatic Syncope in Brugada Syndrome

Diagnosis and Risk Stratification

An implantable cardioverter-defibrillator (ICD) is recommended for patients with Brugada syndrome who have a spontaneous type 1 ECG pattern and recent syncope presumed due to ventricular arrhythmia, provided meaningful survival greater than 1 year is expected, as recommended by the American College of Cardiology and American Heart Association 1, 2, 3, 4, 5
The American College of Cardiology recommends ICD implantation as the Class I recommendation for patients with spontaneous type 1 pattern and syncope presumed due to ventricular arrhythmia 1, 2, 3, 4, 5
Quinidine is recommended as an alternative (Class I recommendation for those who decline or are not candidates for ICD) by the American College of Cardiology 1, 2, 3, 4, 5, 6

Confirm spontaneous type 1 Brugada pattern: Coved ST-segment elevation ≥2 mm in V1 and/or V2 with negative T-waves, as recommended by the American College of Cardiology 7
Use high electrode positioning in second and third intercostal spaces to improve detection, as recommended by the American College of Cardiology 8, 7

Aggressive fever management with antipyretics is recommended to prevent life-threatening events in young patients, as recommended by the American College of Cardiology 6, 7
Avoid QT-prolonging and sodium channel blocking drugs, as recommended by the American College of Cardiology 8, 6, 7
Avoid excessive alcohol intake, as recommended by the American College of Cardiology 8, 7
Avoid cocaine and certain psychotropic/anesthetic agents, as recommended by the American College of Cardiology 8, 6, 7

Genetic counseling and testing may be useful to facilitate cascade screening of relatives (Class IIb recommendation), as recommended by the American College of Cardiology and American Heart Association 1, 2, 3, 4, 5
SCN5A mutations account for 20-30% of cases, though negative genetic testing does not exclude diagnosis, as reported by the American College of Cardiology 8, 7

Annual arrhythmic event rates: 13.5% in cardiac arrest survivors, 3.2% in syncope patients, 1% in asymptomatic patients, as reported by the American College of Cardiology 6
Spontaneous type 1 pattern and symptoms at diagnosis are the strongest predictors of life-threatening arrhythmias in young patients, as reported by the American College of Cardiology 6
If ICD implanted: Monitor for appropriate and inappropriate shocks; consider quinidine or ablation for recurrent shocks, as recommended by the American College of Cardiology and American Heart Association 1, 2, 3, 4, 5