II. Definition

  1. Sudden unexpected nocturnal death
  2. Includes only idiopathic cases of Sudden Cardiac Death

III. Epidemiology

  1. Incidence: Uncommon
    1. Accounts for <5% of Sudden Cardiac Death cases
  2. Family History of sudden death events often present
  3. Most common in men
  4. Greater Incidence in Southeast asia
    1. Northeastern Thailand (40/year per 100,000)
    2. Japan
    3. Philipines

IV. Pathophysiology

  1. Inherited disorder of myocardial Sodium channels
  2. No structural abnormalities

V. Diagnostics: EKG findings

  1. Prolonged QT Interval
  2. Right Bundle Branch Block
  3. Brugada Sign
    1. Seen best in the right precordial leads (V1-V3)
    2. End of QRS marked by significant upward deflection (J Point elevation)
    3. ST segment elevation (leads v1-v3)
  4. Types
    1. Type 1 (coved-type)
      1. J-wave amplitude >2 mm (or mV) at peak AND
      2. Followed by negative T Wave (minimal or no isoelectric component)
    2. Type 2 (saddleback pattern)
      1. J-wave amplitude >2 mm (or mV) at peak AND
      2. ST Elevation >1 mm above baseline, gradually down-sloping AND
      3. Positive or biphasic T Wave
    3. Type 3
      1. Right precordial ST Elevation

VI. Symptoms

  1. Often asymptomatic until terminal event (40%)
  2. Exertional Syncope history may be present
  3. Agonal Respirations may be present during sleep

VII. Labs

  1. Genetic test is available

VIII. Management

IX. Complications

  1. Ventricular Tachycardia
  2. Cardiac Arrest with Ventricular Fibrillation
  3. Sudden Cardiac Death
    1. Mean age of onset 41 years old (but range of onset varies from newborns to elderly)

X. Prognosis

  1. Mortality: 10% (without ICD placement)

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Ontology: Brugada Syndrome (disorder) (C1142166)

Definition (MSH) An autosomal dominant defect of cardiac conduction that is characterized by an abnormal ST-segment in leads V1-V3 on the ELECTROCARDIOGRAM resembling a right BUNDLE-BRANCH BLOCK; high risk of VENTRICULAR TACHYCARDIA; or VENTRICULAR FIBRILLATION; SYNCOPAL EPISODE; and possible sudden death. This syndrome is linked to mutations of gene encoding the cardiac SODIUM CHANNEL alpha subunit.
Definition (SNOMEDCT_US) Clinical manifestations of cardiac syncope, ventricular tachycardia, ventricular fibrillation, or sudden death in conjunction with a genetic mutation associated with Brugada Syndrome and/or a Brugada pattern ECG (spontaneous or provoked).
Definition (SCTSPA) Manifestaciones clínicas de síncope cardíaco, taquicardia ventricular, fibrilación ventricular o muerte súbita junto con una mutación genética asociada con el síndrome de Brugada y/o un patrón electrocardiográfico semejante a éste.
Definition (NCI_CDISC) Polymorphic ventricular tachycardia in the absence of structural heart disease, associated with a baseline ECG pattern during sinus rhythm showing right bundle branch block with ST segment elevation in leads V1 through V3. It can also be characterized by documentation of ECG patterns associated with Brugada Syndrome, some of which may be unmasked when provoked with drugs. The most common genetic mutations identified for Brugada syndrome are in the sodium channel gene SCN5A.
Definition (NCI) An electrocardiographic finding of a pattern of right bundle branch block and ST-segment elevation within electrocardiogram leads V1-V3. This pattern emerges as a result of a defect in ion channel genes, resulting in atypical electrophysiological activity in the right ventricle and a propensity for malignant tachyarrhythmias.
Concepts Disease or Syndrome (T047)
MSH D053840
SnomedCT 418818005
Spanish síndrome de Brugada (trastorno), síndrome de Brugada, Síndrome de Brugada
English Brugada Syndrome, RIGHT BUNDLE BRANCH BLOCK, ST SEGMENT ELEVATION, AND SUDDEN DEATH SYNDROME, SUNDS, SUDDEN UNEXPLAINED NOCTURNAL DEATH SYNDROME, Brugada's syndrome, Brugada's syndrome (diagnosis), BRUGADA SYNDROME 1, Brugada Syndrome (disorder), Brugada Syndrome [Disease/Finding], brugada syndrome, brugadas syndrome, BRGDA1, Brugada Syndrome 1, Sudden Unexplained Nocturnal Death Syndrome, Right Bundle Branch Block, St Segment Elevation, And Sudden Death Syndrome, brugada syndrome-1 (diagnosis), brugada syndrome-1, Ventricular arrhythmia associated with Brugada syndrome, Brugada Syndrome Ventricular Arrhythmia by ECG Finding, Brugada Syndrome Ventricular Arrhythmia by EKG Finding, BRUGADA SYNDROME, Brugada Syndrome Ventricular Arrhythmia, Brugada syndrome, Brugada syndrome (disorder)
Portuguese Síndrome de Brugada, Síndroma de Brugada
Russian БРУГАДА СИНДРОМ, BRUGADA SINDROM
Italian Sindrome di Brugada
German Brugada-Syndrom
French Fibrillation ventriculaire idiopathique, Syndrome de Brugada, FVI (Fibrillation Ventriculaire Idiopathique), Syndrome de Brugada de type 1, Syndrome des Brugada
Japanese ブルガダ症候群, ブルガダショウコウグン, Brugada症候群, ブルガタ症候群
Swedish Brugadas syndrom
Czech Brugadův syndrom, Brugada syndrom
Finnish Brugadan oireyhtymä
Polish Zespół Brugadów, Choroba Burgadów
Hungarian Brugada-syndroma
Norwegian Brugadas syndrom
Dutch Brugada-syndroom