Brugada Syndrome

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

1. See Also
   1. Arrhythmias in Athletes
   2. Sudden Death in Athletes
   3. Sudden Arrhythmia Death Syndrome (Brugada Syndrome)
2. Definition
   1. Sudden unexpected nocturnal death
   2. Includes only idiopathic cases of Sudden Cardiac Death
3. 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
4. Pathophysiology
   1. J Wave Syndrome
   2. Inherited disorder of myocardial Sodium channels
   3. No structural abnormalities
5. Symptoms
   1. Often asymptomatic until terminal event (40%)
   2. Exertional Syncope history may be present
   3. Agonal Respirations may be present during sleep
6. 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
7. Diagnosis
   1. Shanghai Scoring System
8. Labs
   1. Genetic Test is available
9. Management
   1. Symptomatic, type I Brugada morphology is most concerning
   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
10. 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)
11. Prognosis
    1. Mortality: 10% (without ICD placement)
12. 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]