Emergent Care / Stabilization:
Review current and previous ECG. Brugada changes are transient; they may be pronounced with fever or medication that causes sodium channel blockade. It is vital to give antipyretics, monitor cardiac rhythm, and avoid sodium channel blockers to deter conversion into potentially life-threatening cardiac rhythms.

Patients with symptomatic type 1 Brugada should immediately be hospitalized for further evaluation and stabilization.

Diagnosis Overview:
Brugada syndrome is characterized by ventricular arrhythmias in the presence of characteristic ECG abnormalities and a structurally normal heart, resulting in recurrent syncope and/or sudden cardiac death. It is usually genetically inherited in an autosomal dominant manner with incomplete penetrance. The underlying genetic defects generally cause reduced sodium inflow to cardiomyocytes during phase 0 of the cardiac action potential, thus shortening the duration of the action potential.

The initial presentation may be syncope or resuscitated sudden cardiac arrest due to polymorphic ventricular tachycardia (VT) or ventricular fibrillation (VF). Onset is generally after adolescence, peaking in the third or fourth decade of life. Arrhythmias occur most commonly at night or at rest. The diagnosis may also be made on a routine ECG or family screening.

Brugada syndrome is reported to be responsible for 4% of all sudden deaths and 20% of sudden deaths in those without structural heart disease. A family history is present in about 20%-30% of patients. Prevalence in the general population is estimated at approximately 0.05%. It is particularly common among individuals of Southeast Asian descent.

The "Brugada pattern" ECG consists of an atypical right bundle branch block with cove-shaped ST elevation in leads V1 to V3 in the absence of obvious structural heart disease, electrolyte disturbances, or ischemia. The ECG findings may be present at baseline or may only become apparent in the setting of a fever or with use of medications having sodium channel blocking activity, such as class 1 antiarrhythmics, tricyclic antidepressants, alcohol, or cocaine. Intermittent presence of the characteristic ECG pattern makes the diagnosis difficult in some patients.

In the pediatric age group, in ages 12 years and younger, there is an increase in presentation of Brugada syndrome and an arrhythmic event if the child had an associated fever. Recurrence of an arrhythmic event was highly dependent on fever occurrence. One-fifth of the pediatric patients did not display ECG with type 1 wave form. Adolescents (aged 13-20 years) with a genetic variation in the SCN5A gene controlling sodium influx, have an increase in reoccurrence of cardiac events.

Pediatric and adult management is similar. If symptomatic, the patient may receive an implanted cardioverter defibrillator (ICD) or quinidine. If asymptomatic, the patient will likely have close follow-up with cardiology, with the pediatric population having repeat ECGs and cardiac MRIs to evaluate the right ventricular outflow tract (RVOT).