Brugada syndrome
(Redirected from Brugada Syndrome)
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
- 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
- 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:
Higher Risk ECG Features
- Early repolarization with J-point elevation in inferior leads (seen in 10% of Brugada syndrome)[10]
- QRS widening > 120 ms in V2[11]
- QRS fragmentation (additional QRS complex spikes)[12][13]
- 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)[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
- Antidysrhythmics trials have inconsistently shown clinical benefit, but potential VF-terminating and prevention meds may include quinidine, isoproterenol[16]
- 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 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 antiepileptic medications
- Certain tricyclic antidepressants
- Certain SSRIs
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
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.
- ↑ 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
- ↑ 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.
- ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
- ↑ 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.
- ↑ 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.
- ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
- ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
- ↑ M Sharon, et al. ECG of the Month. Annals of Emergency Medicine. December 2019. 74(6);782-785
- ↑ 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.