Brugada Syndrome Management

Diagnosis and Clinical Context

Brugada syndrome is a primary electrical disorder characterized by coved ST-segment elevation in right precordial leads with increased risk of sudden cardiac death in patients with structurally normal hearts, involving ion channel dysfunction, predominantly SCN5A mutations affecting cardiac sodium channels 1, 2, 3.
The American College of Cardiology recommends that patients with Brugada syndrome avoid specific drugs, excessive alcohol, and cocaine, which can trigger life-threatening arrhythmias 1, 3.

The American Heart Association suggests that spontaneous type 1 ECG pattern plus recent syncope presumed due to ventricular arrhythmia indicates the need for an ICD 4.
Quinidine is recommended for patients who decline or are not candidates for ICD, with a Class I recommendation 4.
Annual arrhythmic event rates are 13.5% in cardiac arrest survivors, 3.2% in syncope patients, and 1% in asymptomatic patients 4.

Fever is a critical trigger for ventricular fibrillation in Brugada syndrome and warrants early, aggressive antipyretic treatment, according to the American College of Cardiology 1, 3, 4.
The American College of Cardiology advises that psychotropic medications, anesthetic agents, and sodium channel blockers can precipitate arrhythmias and should be discontinued if present 1, 3.

SCN5A mutations account for 20-30% of phenotype-positive Brugada cases, and negative genetic testing does not exclude Brugada syndrome, as diagnosis is primarily clinical and electrocardiographic 3, 4.
Family screening with ECG and consideration for genetic counseling is recommended for patients with Brugada syndrome 4.