Brugada syndrome: Difference between revisions
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==Background== | ==Background== | ||
*Consider as cause of syncope in patients with family history of sudden death | *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> | *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 | *Much more common in men (up to 9x), particularly Southeast Asian males | ||
*ECG shows incomplete [[RBBB]] with [[ST elevation]] in V1-V3 | *[[ECG]] shows incomplete [[RBBB]] with [[ST elevation]] in V1-V3 | ||
**In uncomplicated RBBB, usually there no ST change to slight ST depression | **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 | **If presenting symptom is chest pain rather than syncope, consider strongly STEMI | ||
==Clinical Features== | ==Clinical Features== | ||
*Typically asymptomatic | *Typically asymptomatic | ||
*Patients may have Vfib arrest or sudden death | *Patients may have Vfib 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> | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{ST elevation DDX}} | {{ST elevation DDX}} | ||
== | ==Evaluation== | ||
===ECG Criteria=== | ===ECG Criteria=== | ||
Note - ''ECG findings can be transient'' | Note - ''ECG findings can be transient'' | ||
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*Type 2-Elevated ST segment (>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 (<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 | ||
====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]] | [[File:Brugada.jpg]] | ||
==Management== | ==Management== | ||
*Cardiology consultation | *Cardiology consultation | ||
*See [[Electrical storm]] | |||
==Disposition== | ==Disposition== | ||
*Needs | *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 | *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 have | **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 antiepileptic medications | |||
***Certain [[tricyclic antidepressants]] | |||
***Certain [[SSRIs]] | |||
==External Links== | ==External Links== |
Revision as of 05:21, 5 June 2018
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 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
- Cardiac
- ST-segment elevation myocardial infarction (STEMI)
- Post-MI (ventricular aneurysm pattern)
- Previous MI with recurrent ischemia in same area
- Wellens' syndrome
- Coronary artery vasospasm (eg, Prinzmetal's angina)
- Coronary artery dissection
- Pericarditis
- Myocarditis
- Aortic dissection in to coronary
- Left ventricular aneurysm
- Left ventricular pseudoaneurysm
- Early repolarization
- Left bundle branch block
- Left ventricular hypertrophy (LVH)
- Myocardial tumor
- Myocardial trauma
- RV pacing (appears as Left bundle branch block)
- Brugada syndrome
- Takotsubo cardiomyopathy
- AVR ST elevation
- Other thoracic
- Metabolic
- Drugs of abuse (eg, cocaine, crack, meth)
- Hyperkalemia (only leads V1 and V2)
- Hypothermia ("Osborn J waves")
- Medications
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
- Early repolarization with J-point elevation in inferior leads, which is seen in 10% of Brugada syndrome[9]
- QRS widening > 120 ms in V2[10]
- QRS fragmentation (additional QRS complex spikes)[11][12]
- ST elevation during recovery after exercise
Management
- Cardiology consultation
- See Electrical storm
Disposition
- Needs EP consult and VF-inducing electrophysiologic study (EPS), though EPS is controversial prognosticator[13]
- Inpatient vs outpatient ICD placement
- Prophylactic ICDs may have risks of complications greater than benefits conferred[14][15]
- Antidysrhythmics trials have inconsistently shown clinical benefit, but potential VF-terminating and prevention meds may include quinidine, isoproterenol[16]
- Certain drugs must be avoided in pts with Brugada syndrome
- If starting or using new med, check www.brugadadrugs.org drug list
- In brief, commonly used meds to avoid include:
- Procainamide
- Bupivacaine
- Propofol
- Ketamine
- Tramadol
- Certain antiepileptic medications
- Certain tricyclic antidepressants
- Certain SSRIs
External Links
See Also
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Sarkozy A., Chierchia G.B., Paparella G.; Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol. 2 2009:154-161.
- ↑ 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.
- ↑ Take Y and Morita H. Fragmented QRS: What Is The Meaning? Indian Pacing Electrophysiol J. 2012 Sep-Oct; 12(5): 213–225.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.