Brugada Syndrome

Aka: Brugada Syndrome, Brugada, Sudden Arrhythmia Death Syndrome, SADS, Bangungut, Pokkuri, Lai Tai, Sleep Death

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II. Definitions

  1. Brugada Syndrome
    1. Genetically inherited Sodium channelopathy (Sodium channel gene SCN5A)
    2. Presents with Cardiac Syncope, Ventricular Tachycardia, Ventricular Fibrillation, or sudden death
    3. Divided into 3 types, with the first most recognizable (Right Bundle Branch Block, ST Elevation V1-V3)
    4. Brugada often manifests after triggers: Fever, Electrolyte abnormalities, Sodium Channel Blocker drugs

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. J Wave Syndrome
  2. Inherited disorder of myocardial Sodium channels (esp. Sodium channel gene SCN5A)
  3. No cardiac structural abnormalities

V. Symptoms

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

VI. 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, wide and large J Wave)
      1. Most concerning of the J Wave Patterns
      2. J-Wave amplitude >2 mm (or mV) at peak AND
      3. 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

VII. Diagnosis

  1. Shanghai Scoring System
    1. https://www.sciencedirect.com/science/article/pii/S2405500X18301695#tbl1
    2. Score based on EKG, history, Family History, Genetic Testing
    3. Interpretation (score >3.5 probable Brugada, 2-3 possible Brugada Syndrome)
      1. Brugada Type 1 criteria are typically met without specific testing
      2. Brugada Types 2-3 typically require electrophysiology for diagnosis confirmation

VIII. Differential Diagnosis: RBBB Pattern

  1. See Right Bundle Branch Block
  2. Early Repolarization
    1. Calculate Corrado Index
      1. Ratio = (J Point at ST start)/(J Point at 80 ms later)
      2. Ratio >1 AND a downsloping ST Segment is consistent with Brugada Type 1

IX. Labs

  1. Genetic Test is available

X. Management

  1. Disposition
    1. Symptomatic, type I Brugada morphology is most concerning
      1. Admit patients with Syncope and suspected Brugada Syndrome and consult electrophysiology
    2. Asymptomatic patients with incidental Brugada findings on ekg
      1. May be discharged with electrophysiology follow-up
  2. Treatment Options
    1. Management decisions are based on symptomatic versus asymptomatic Brugada findings
    2. Internal Cardiac Defibrillator (ICD)
    3. Quinidine
  3. Exercise and sports participation are not contraindicated
    1. However avoid significant Hypothermia or hyperthermia
  4. Avoid provocative factors
    1. Treat fever
    2. No Cocaine
    3. Avoid excessive Alcohol

XI. 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)

XII. Prognosis

  1. Mortality: 10% (without ICD placement)

XIII. References

  1. Krishnan (2018) Cardiac Arrhythmias Conference, UMN, Minneapolis, MN
  2. Joshi and Dermark (2016) Crit Dec Emerg Med 30(8):3-12
  3. Antzelevitch (2003) J Am Coll Cardiol 41(10):1665-71 [PubMed]
  4. Brugada (1992) J Am Coll Cardiol 20(6):1391-6 [PubMed]
  5. Littmann (2003) Am Heart J 145(5):768-78 [PubMed]
  6. Meyer (2003) Am Fam Physician 68(3):483-8 [PubMed]

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Related Studies

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, ﾌﾞﾙｶﾞﾀﾞｼｮｳｺｳｸﾞﾝ, 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
Derived from the NIH UMLS (Unified Medical Language System)

Related Topics in Electrocardiogram

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