Brugada syndrome
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 no ST change to slight ST depression
- If presenting symptom is chest pain rather than syncope, consider strongly STEMI
Clinical Features
- Typically asymptomatic
- ECG findings can be more prominent during times of illness or fever
- Patients may have Vfib arrest or sudden death
- Diagnostic EKG findings transient and variable, with only 1/3 of serial EKGs diagnostic in confirmed spontaneous coved-type Brugada syndrome pts[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
Management
- Cardiology consultation
Disposition
- Needs EP consult and VF-inducing electrophysiologic study (EPS), though EPS is controversial prognosticator[4]
- Inpatient vs outpatient ICD placement
- Prophylactic ICDs may have risks of complications greater than benefits conferred[5][6]
- Mortality around 10% per year without ICD placement[citation needed]
- Antidysrhythmics have no effect on prognosis
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.
- ↑ 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.