Brugada syndrome: Difference between revisions

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== Pathophysiology ==
==Background==
*Consider as cause of syncope in patients with family history of sudden death
*80% of Brugada syndrome diagnosed only after a cardiac arrest<ref>Paul  M., Gerss  J., Schulze-Bahr  E.; Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data. Eur Heart J. 28 2007:2126-2133.</ref>
*Autosomal dominant Na-channelopathy which increases the risk of sudden cardiac death (~10%/yr)<ref>Cerrato N, Giustetto C, et al. Prevalence of Type 1 Brugada Electrocardiographic Pattern Evaluated by Twelve-Lead Twenty-Four-Hour Holter Monitoring. The American Journal of Cardiology.115(1). 2015. 52-56.</ref>
*Much more common in men (up to 9x), particularly Southeast Asian males
*[[ECG]] shows incomplete [[RBBB]] with [[ST elevation]] in V1-V3
**In uncomplicated RBBB, usually there is no ST change to slight ST depression
**If presenting symptom is chest pain rather than syncope, consider strongly STEMI


*Genetically-linked Na-channelopathy
==Clinical Features==
**Incomplete RBBB with ST elevation in V1-V3
*Typically asymptomatic
**Increased risk of sudden cardiac death  
*Patients may have [[Vfib]] [[cardiac arrest]] or sudden death
***~10%/yr
*Diagnostic [[ECG]] findings transient and variable, with only 1/3 of serial ECGs diagnostic in confirmed spontaneous coved-type Brugada syndrome patients<ref>Richter  S., Sarkozy  A., Veltmann  C.; Variability of the diagnostic ECG pattern in an ICD patient population with Brugada syndrome. J Cardiovasc Electrophysiol. 20 2009:69-75.</ref>
**Much more common in men (up to 9x)


== ECG Criteria ==
==Differential Diagnosis==
{{ST elevation DDX}}


*Note - ECG findings can be transient
==Evaluation==
===ECG Criteria===
Note - ''ECG findings can be transient''


*Type 1-Elevated ST segment (&gt;2mm) descends w/ upward convexity to a TWI  
*Type 1-Elevated ST segment (>2mm) descends with upward convexity to a TWI  
*Type 2-Elevated ST segment (&gt;1mm) descends toward baseline then rises again (saddleback) to upright T wave  
*Type 2-Elevated ST segment (>1mm) descends toward baseline then rises again (saddleback) to upright T wave  
*Type 3-Elevated ST segment (&lt;1mm) descends toward baseline then rises again to upright T wave  
*Type 3-Elevated ST segment (<1mm) descends toward baseline then rises again to upright T wave  
[[File:Brugada.jpg]]<br>
====Other ECG Pearls in Brugada Syndrome====
*V1-v2 lead placement in 2nd or 3rd IC spaces (rather than conventional 4th IC space) increases chance of recording type I pattern<ref>Shimizu  W., Matsuo  K., Takagi  M.; Body surface distribution and response to drugs of ST segment elevation in Brugada syndrome: clinical implication of eighty-seven-lead body surface potential mapping and its application to twelve-lead electrocardiograms. J Cardiovasc Electrophysiol. 11 2000:396-404.</ref>
*Other factors that increase type I ECG tracings<ref>Ikeda  T., Abe  A., Yusu  S.; The full stomach test as a novel diagnostic technique for identifying patients at risk of Brugada syndrome. J Cardiovasc Electrophysiol. 17 2006:602-607.</ref><ref>Shimeno  K., Takagi  M., Maeda  K., Tatsumi  H., Doi  A., Yoshiyama  M.; Usefulness of multichannel Holter ECG recording in the third intercostal space for detecting type 1 Brugada ECG: comparison with repeated 12-lead ECGs. J Cardiovasc Electrophysiol. 20 2009:1026-1031.</ref><ref>Keller  D.I., Huang  H., Zhao  J.; A novel SCN5A mutation, F1344S, identified in a patient with Brugada syndrome and fever-induced ventricular fibrillation. Cardiovasc Res. 70 2006:521-529.</ref>:
**Fever
**Night time
**After heavy meals
**Recovery phase of exercise<ref>Makimoto  H., Nakagawa  E., Takaki  H.; Augmented ST-segment elevation during recovery from exercise predicts cardiac events in patients with Brugada syndrome. J Am Coll Cardiol. 56 2010:1576-1584.</ref>
*[[ECG]] features placing pt at higher risk for symptomatic Brugada
**[[Early repolarization]] with J-point elevation in inferior leads, which is seen in 10% of Brugada syndrome<ref>Sarkozy  A., Chierchia  G.B., Paparella  G.; Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol. 2 2009:154-161.</ref>
**QRS widening > 120 ms in V2<ref>Junttila  M.J., Brugada  P., Hong  K.; Differences in 12-lead electrocardiogram between symptomatic and asymptomatic Brugada syndrome patients. J Cardiovasc Electrophysiol. 19 2008:380-383.</ref>
**[http://www.ipej.org/1205/morita1.jpeg QRS fragmentation] (additional QRS complex spikes)<ref>Take Y and Morita H. Fragmented QRS: What Is The Meaning? Indian Pacing Electrophysiol J. 2012 Sep-Oct; 12(5): 213–225.</ref><ref>Morita  H., Kusano  K.F., Miura  D.; Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome. Circulation. 118 2008:1697-1704.</ref>
**ST elevation during recovery after exercise
[[File:Brugada.jpg]]


==Management==
*Cardiology consultation
*See [[Electrical storm]]


==Disposition==
*Needs EP consult and VF-inducing electrophysiologic study (EPS), though EPS is controversial prognosticator<ref>Viskin S and Rosso R. Risk of Sudden Death in Asymptomatic Brugada Syndrome: Not as High as We Thought and Not as Low as We Wished…But the Contrary. J Am Coll Cardiol. 2010;56(19):1585-1588.</ref>
*Inpatient vs outpatient ICD placement
**Prophylactic ICDs may have risks of complications greater than benefits conferred<ref>Sacher  F., Probst  V., Iesaka  Y.; Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study. Circulation. 114 2006:2317-2324.</ref><ref>Rosso  R., Glick  A., Glikson  M.; Outcome after implantation of cardioverter defibrillator in patients with Brugada syndrome: a multicenter Israeli study (ISRABRU). Isr Med Assoc J. 10 2008:435-439.</ref>
**Antidysrhythmics trials have inconsistently shown clinical benefit, but potential VF-terminating and prevention meds may include [[quinidine]], [[isoproterenol]]<ref>Postema PG, Wolpert C, Amin AS, Probst V, Borggrefe M, Roden DM, et al. Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date website (www.brugadadrugs.org). Heart Rhythm. 2009 Sep. 6(9):1335-41.</ref>
*Certain drugs must be avoided in pts with Brugada syndrome
**If starting or using new med, check [http://www.brugadadrugs.org/drug-lists/ www.brugadadrugs.org drug list]
**In brief, commonly used meds to avoid include:
***[[Procainamide]]
***[[Bupivacaine]]
***[[Propofol]]
***[[Ketamine]]
***[[Tramadol]]
***Certain [[antiepileptics|antiepileptic]] medications
***Certain [[tricyclic antidepressants]]
***Certain [[SSRIs]]


== Disposition ==
==External Links==
 
*[https://www.youtube.com/watch?v=NJL8An6uO1Y Amal Mattu ECG Case: March 4 2012]
*Pts need an AICD


==See Also==
==See Also==
*[[STEMI]]
*[[ST elevation]]
*[[ST Segment Elevation (DDX)]]


== Source ==
==References==
*UpToDate
<references/>


[[Category:Cards]]
[[Category:Cardiology]]

Revision as of 22:30, 1 October 2019

Background

  • Consider as cause of syncope in patients with family history of sudden death
  • 80% of Brugada syndrome diagnosed only after a cardiac arrest[1]
  • Autosomal dominant Na-channelopathy which increases the risk of sudden cardiac death (~10%/yr)[2]
  • Much more common in men (up to 9x), particularly Southeast Asian males
  • ECG shows incomplete RBBB with ST elevation in V1-V3
    • In uncomplicated RBBB, usually there is no ST change to slight ST depression
    • If presenting symptom is chest pain rather than syncope, consider strongly STEMI

Clinical Features

  • Typically asymptomatic
  • Patients may have Vfib cardiac arrest or sudden death
  • Diagnostic ECG findings transient and variable, with only 1/3 of serial ECGs diagnostic in confirmed spontaneous coved-type Brugada syndrome patients[3]

Differential Diagnosis

ST Elevation

Evaluation

ECG Criteria

Note - ECG findings can be transient

  • Type 1-Elevated ST segment (>2mm) descends with upward convexity to a TWI
  • Type 2-Elevated ST segment (>1mm) descends toward baseline then rises again (saddleback) to upright T wave
  • Type 3-Elevated ST segment (<1mm) descends toward baseline then rises again to upright T wave

Other ECG Pearls in Brugada Syndrome

  • V1-v2 lead placement in 2nd or 3rd IC spaces (rather than conventional 4th IC space) increases chance of recording type I pattern[4]
  • Other factors that increase type I ECG tracings[5][6][7]:
    • Fever
    • Night time
    • After heavy meals
    • Recovery phase of exercise[8]
  • ECG features placing pt at higher risk for symptomatic Brugada

Brugada.jpg

Management

Disposition

External Links

See Also

References

  1. Paul M., Gerss J., Schulze-Bahr E.; Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data. Eur Heart J. 28 2007:2126-2133.
  2. Cerrato N, Giustetto C, et al. Prevalence of Type 1 Brugada Electrocardiographic Pattern Evaluated by Twelve-Lead Twenty-Four-Hour Holter Monitoring. The American Journal of Cardiology.115(1). 2015. 52-56.
  3. Richter S., Sarkozy A., Veltmann C.; Variability of the diagnostic ECG pattern in an ICD patient population with Brugada syndrome. J Cardiovasc Electrophysiol. 20 2009:69-75.
  4. Shimizu W., Matsuo K., Takagi M.; Body surface distribution and response to drugs of ST segment elevation in Brugada syndrome: clinical implication of eighty-seven-lead body surface potential mapping and its application to twelve-lead electrocardiograms. J Cardiovasc Electrophysiol. 11 2000:396-404.
  5. Ikeda T., Abe A., Yusu S.; The full stomach test as a novel diagnostic technique for identifying patients at risk of Brugada syndrome. J Cardiovasc Electrophysiol. 17 2006:602-607.
  6. Shimeno K., Takagi M., Maeda K., Tatsumi H., Doi A., Yoshiyama M.; Usefulness of multichannel Holter ECG recording in the third intercostal space for detecting type 1 Brugada ECG: comparison with repeated 12-lead ECGs. J Cardiovasc Electrophysiol. 20 2009:1026-1031.
  7. Keller D.I., Huang H., Zhao J.; A novel SCN5A mutation, F1344S, identified in a patient with Brugada syndrome and fever-induced ventricular fibrillation. Cardiovasc Res. 70 2006:521-529.
  8. Makimoto H., Nakagawa E., Takaki H.; Augmented ST-segment elevation during recovery from exercise predicts cardiac events in patients with Brugada syndrome. J Am Coll Cardiol. 56 2010:1576-1584.
  9. Sarkozy A., Chierchia G.B., Paparella G.; Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol. 2 2009:154-161.
  10. Junttila M.J., Brugada P., Hong K.; Differences in 12-lead electrocardiogram between symptomatic and asymptomatic Brugada syndrome patients. J Cardiovasc Electrophysiol. 19 2008:380-383.
  11. Take Y and Morita H. Fragmented QRS: What Is The Meaning? Indian Pacing Electrophysiol J. 2012 Sep-Oct; 12(5): 213–225.
  12. Morita H., Kusano K.F., Miura D.; Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome. Circulation. 118 2008:1697-1704.
  13. Viskin S and Rosso R. Risk of Sudden Death in Asymptomatic Brugada Syndrome: Not as High as We Thought and Not as Low as We Wished…But the Contrary. J Am Coll Cardiol. 2010;56(19):1585-1588.
  14. Sacher F., Probst V., Iesaka Y.; Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study. Circulation. 114 2006:2317-2324.
  15. Rosso R., Glick A., Glikson M.; Outcome after implantation of cardioverter defibrillator in patients with Brugada syndrome: a multicenter Israeli study (ISRABRU). Isr Med Assoc J. 10 2008:435-439.
  16. Postema PG, Wolpert C, Amin AS, Probst V, Borggrefe M, Roden DM, et al. Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date website (www.brugadadrugs.org). Heart Rhythm. 2009 Sep. 6(9):1335-41.