Brugada syndrome: Difference between revisions

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==Pathophysiology==
==Background==
*Brugada syndrome is a rare, inherited arrhythmic disorder causing an increased risk of syncope and sudden death due to ventricular fibrillation.
*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


==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<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>


Genetically-linked sodium channelopathy, characterized by incomplete RBBB with ST elevation in V1 and V2, and an increased risk of sudden cardiac death
==Differential Diagnosis==
{{ST elevation DDX}}


Note - EKG findings can be TRANSIENT
==Evaluation==
===ECG Criteria===
[[File:Brugada.jpg|thumb|600px|Brugada ECG findings by type.]]
*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




==EKG Criteria==
''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>''


====ECG Unmasking Factors<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><ref>ANTZELEVITCH, C., & BRUGADA, R. (2002). Fever and Brugada Syndrome. Pacing and Clinical Electrophysiology, 25(11), 1537–1539. doi:10.1046/j.1460-9592.2002.01537.x</ref>====
*Type 1 ECG findings can be transient and "unmasked" by the following:
**[[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>


Type 1 - 2 mm J-point elevation a gradually descending ST segment and a negative T-waveType 2 - saddle back pattern with at least 2 mm J-point elevation and at least 1 mm ST elevation with a positive or biphasic T-waveType 3 - saddle back pattern with less than 2 mm J-point elevation and less than 1 mm ST elevation with a positive T-wave  ==Disposition==
====Higher Risk [[ECG]] Features====
*[[Early repolarization]] with J-point elevation in inferior leads (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


===Diagnosis===
*Diagnosed when a patient has Burdada's ECG pattern (see above) AND documented ventricular tachydysrhythia or history consistent with ventricular tachydysrythmia (e.g. [[syncope]], sudden cardiac death)<ref>M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785</ref>


a/w polymorphic v tach, v fib.
==Management==
''Acute ED management depends on presenting symptoms''
===Incidental Brugada Pattern on ECG (Otherwise Asymptomatic)===
*No acute treatment


mortality ~10% / yr
===Concerning Cardiac Symptoms===
*Cardiology consultation and likely admission
**Consider 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>


pts need an AICD
===Active Tachydysrhythmia===
*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>
*See [[electrical storm]] for 3 or more episodes of sustained [[ventricular tachycardia]], [[ventricular fibrilation]], or ICD shocks within 24 hours


===Drugs to be Avoided===
*If starting or using new med, check [http://www.brugadadrugs.org/drug-lists/ www.brugadadrugs.org drug list]
*Commonly used meds to avoid include:
**[[Procainamide]]
**[[Bupivacaine]]
**[[Propofol]]
**[[Ketamine]]
**[[Tramadol]]
**[[Lithium]]
**Except for quinidine, class I antiarrhythmic drugs, particularly sodium channel blockers such as procainamide and flecainide should be avoided
**Certain [[antiepileptics|antiepileptic]] medications
**Certain [[tricyclic antidepressants]]
**Certain [[SSRIs]]


==Source==
==Disposition==
*Incidental finding with no suggestive family or patient history (e.g. syncope, tachydysrhythmia, sudden cardiac death)--> education and general cardiology referral<ref>M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785</ref>
*Suggestive family or patient history --> disposition in consultation with cardiology<ref>M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785</ref>
*Active tachydysrhythmia --> admission<ref>M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785</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>


Adapted from ....Mattu (lecture)
==External Links==
*[https://www.youtube.com/watch?v=NJL8An6uO1Y Amal Mattu ECG Case: March 4 2012]
*[http://www.emdocs.net/ecg-pointers-syncope-and-brugada-syndrome/ emDocs - ECG Pointers: Syncope and Brugada Syndrome]


==See Also==
*[[ST elevation]]


==References==
<references/>


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

Latest revision as of 01:09, 3 September 2021

Background

  • Brugada syndrome is a rare, inherited arrhythmic disorder causing an increased risk of syncope and sudden death due to ventricular fibrillation.
  • 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

Brugada ECG findings by type.
  • 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


v1-v2 lead placement in 2nd or 3rd IC spaces (rather than conventional 4th IC space) increases chance of recording type I pattern[4]

ECG Unmasking Factors[5][6][7][8]

  • Type 1 ECG findings can be transient and "unmasked" by the following:
    • Fever
    • Night time
    • After heavy meals
    • Recovery phase of exercise[9]

Higher Risk ECG Features

Diagnosis

  • Diagnosed when a patient has Burdada's ECG pattern (see above) AND documented ventricular tachydysrhythia or history consistent with ventricular tachydysrythmia (e.g. syncope, sudden cardiac death)[14]

Management

Acute ED management depends on presenting symptoms

Incidental Brugada Pattern on ECG (Otherwise Asymptomatic)

  • No acute treatment

Concerning Cardiac Symptoms

  • Cardiology consultation and likely admission
    • Consider EP consult and VF-inducing electrophysiologic study (EPS), though EPS is controversial prognosticator[15]

Active Tachydysrhythmia

Drugs to be Avoided

Disposition

  • Incidental finding with no suggestive family or patient history (e.g. syncope, tachydysrhythmia, sudden cardiac death)--> education and general cardiology referral[17]
  • Suggestive family or patient history --> disposition in consultation with cardiology[18]
  • Active tachydysrhythmia --> admission[19]

Inpatient vs Outpatient ICD Placement

  • Prophylactic ICDs may have risks of complications greater than benefits conferred[20][21]

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. ↑ ANTZELEVITCH, C., & BRUGADA, R. (2002). Fever and Brugada Syndrome. Pacing and Clinical Electrophysiology, 25(11), 1537–1539. doi:10.1046/j.1460-9592.2002.01537.x
  9. ↑ 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.
  10. ↑ Sarkozy A., Chierchia G.B., Paparella G.; Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol. 2 2009:154-161.
  11. ↑ 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.
  12. ↑ Take Y and Morita H. Fragmented QRS: What Is The Meaning? Indian Pacing Electrophysiol J. 2012 Sep-Oct; 12(5): 213–225.
  13. ↑ 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.
  14. ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
  15. ↑ 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.
  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.
  17. ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
  18. ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
  19. ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
  20. ↑ 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.
  21. ↑ 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.
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