Skip to Main Content

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Life-threatening cardiac arrhythmia (ventricular fibrillation and ventricular tachycardia [VT]) with no demonstrable structural cardiac disease

  • Brugada syndrome is defined by those who have experienced sudden cardiac death or sustained VT in patients with a Brugada pattern on 12-lead ECG

  • Brugada pattern is pseudo-right bundle branch block with persistent ST elevation in V1–V2

  • The disease is secondary to a sodium channel (SCN5A) mutation

  • Flecainide or procainamide (sodium channel blockers) may be used to unmask the ECG changes (ST elevation in V2)

GENERAL CONSIDERATIONS

  • Spontaneous or inducible polymorphic VT

  • Some patients remain asymptomatic and are termed Brugada pattern. If VT or sudden death, then Brugada syndrome

  • QT interval is normal

  • Young males and adolescents are most often affected

  • Suggested to be a cause of "sleep death" among young Asian males

  • The syndrome is familial, and a mutation in chromosome 3 has been identified

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Cardiac arrest

  • Asymptomatic

PHYSICAL EXAM FINDINGS

  • Normal physical exam

DIFFERENTIAL DIAGNOSIS

  • Long QT syndrome

  • Idiopathic ventricular arrhythmia

  • ECG changes may mimic acute myocardial infarction (cardiac enzymes and regional wall motion are usually normal but may be transiently abnormal after a cardiac arrest)

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Genetic testing in specialized labs

ELECTROCARDIOGRAPHY

  • ECG with pseudo-right bundle branch block with ST elevation in V1–V2

  • If the syndrome is suspected but the baseline ECG is normal, challenge with the administration of flecainide or procainamide is recommended

IMAGING STUDIES

  • Echocardiogram to exclude structural heart disease

DIAGNOSTIC PROCEDURES

  • Symptomatic or asymptomatic patients with abnormal ECG at baseline require electrophysiologic studies to determine whether sustained ventricular arrhythmia can be induced

TREATMENT

CARDIOLOGY REFERRAL

  • All patients should be evaluated by a cardiac electrophysiologist and a medical geneticist for genetic testing

HOSPITALIZATION CRITERIA

  • After a cardiac arrest, all patients are hospitalized to the cardiac intensive care unit

  • Asymptomatic patients with abnormal ECG do not require hospitalization

THERAPEUTIC PROCEDURES

  • Implantable cardioverter-defibrillator (ICD) is needed for patients with life-threatening arrhythmia or inducible sustained ventricular arrhythmias on electrophysiologic testing

SURGERY

  • If there is no venous access, then epicardial placement of an ICD may be required

MONITORING

  • ECG monitoring in hospital

DIET AND ACTIVITY

  • General healthy lifestyle

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • Discharge 24 hours after ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.