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Brugada Syndrome

Aka: Brugada Syndrome, 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. Inherited disorder of myocardial sodium channels
  5. Associated EKG findings
    1. Prolonged QT Interval
    2. Brugada Sign
      1. Seen best in the right precordial leads (V1-V3)
      2. End of QRS marked by significant upward deflection
      3. ST segment elevation
    3. Associated with Right Bundle Branch Block
  6. Symptoms
    1. Often asymptomatic until terminal event (40%)
    2. Exertional Syncope history may be present
  7. References
    1. Antzelevitch (2003) J Am Coll Cardiol 41(10):1665-71
    2. Brugada (1992) J Am Coll Cardiol 20(6):1391-6
    3. Littmann (2003) Am Heart J 145(5):768-78
    4. Meyer (2003) Am Fam Physician 68(3):483-8

Brugada Syndrome (disorder) (C1142166)

Definition (NCI) A condition characterized by a pattern of right bundle branch block (RBBB) and ST-segment elevation within electrocardiogram (ECG) leads V1-V3 . This pattern emerges as a result of a defect in ion channel genes, resulting in abnormal electrophysiological activity in the right ventricle and a propensity to malignant tachyarrhythmias. (NCI)
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 (NCI) A condition characterized by a pattern of right bundle branch block (RBBB) and ST-segment elevation within electrocardiogram (ECG) leads V1-V3. This pattern emerges as a result of a defect in ion channel genes, resulting in abnormal electrophysiological activity in the right ventricle and a propensity to malignant tachyarrhythmias.
Concepts Disease or Syndrome (T047)
MSH D053840
SnomedCT 418818005
Spanish síndrome de Brugada (trastorno), síndrome de Brugada, Síndrome de Brugada, Sindrome 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), VENTRICULAR ARRHYTHMIA ASSOCIATED WITH BRUGADA SYNDROME, Ventricular arrhythmia associated with Brugada syndrome, BRUGADA SYNDROME 1, Brugada Syndrome (disorder), Brugada Syndrome [Disease/Finding], brugada syndrome, brugadas syndrome, 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 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
Hungarian Brugada-syndroma
Dutch Brugada-syndroom
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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