Brugada syndrome
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Brugada Syndrome. Morning Report June, 2008 Jessie Stewart. Why Present Brugada?. 1. Lots of us missed it. 2. A new discovery- first described in 1992. 3. Drs. Josep, Pedro and Ramon Brugada. Where are we going?. Primary Goal Understanding Brugada Prevalence Presentation Prognosis

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Brugada Syndrome

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Brugada syndrome

Brugada Syndrome

Morning Report

June, 2008

Jessie Stewart


Why present brugada

Why Present Brugada?

1. Lots of us missed it.

2. A new discovery- first described in 1992.

3. Drs. Josep, Pedro and Ramon Brugada.


Where are we going

Where are we going?

  • Primary Goal

  • Understanding Brugada

  • Prevalence

  • Presentation

  • Prognosis

  • Therapy


Brugada syndrome

Goal

Recognize Brugada I: coved ST segment in V1-V3, >2mm elevation, inverted T wave.


Brugada syndrome is

Brugada Syndrome is…

  • A sodium channel abnormality that predisposes to sudden cardiac death.

  • Characterized by specific EKG patterns:

    • Type I is diagnostic when combined with the right clinical picture.

    • Types II and III raise suspicion for Brugada but they are only diagnositic if they can be converted to Type I during challenge with a sodium channel blocker.

    • These patterns are dynamic and inducible.


Type i diagnostic

Type I- Diagnostic

  • V1-V3 (as least two leads) ST segment elevation >2mm, “coved” shape, inverted T-wave.

  • Coupled with

    • Documented VFib

    • Polymorphic VT

    • FH of sudden cardiac death <45 yo

    • Type I EKG in family members

    • VT inducable in EP lab

    • Syncope

    • Nocturnal agonal respiration


Types ii and iii suggestive

Types II and III- Suggestive

  • II: V1-V3 ST segment elevation >2mm, “saddleback” shape, pos or biphasic T.

  • III: <1 mm elevation, either coved or saddleback.


Scn5a gene

SCN5A gene

  • Codes for cardiac sodium channel that opens during phase 2 of the action potential. In Brugada, it opens poorly in RV epicardial cells.

  • Autosomal dominant inheritance

  • 20-30% of cases have anbl SCN5A gene.

  • 80+ mutations, differing prognosis.

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Priori, S. G. et al. Circulation 1999;99:674-681


Brugada syndrome

Defective sodium channels: shorter AP (phase 0), deeper notch (phase I), and shorter phase 2.

Creates juxtaposition of depolarized and repolarized cells, setting up possibility of PHASE 2 RENTRY, closely grouped PVCs, and VT or V Fib.

On EKG, ST segment not at baseline because no longer have uniform depolarization of the entire ventricle.

Nattel and Carlsson Nature Reviews Drug Discovery5, 1034–1049 (December 2006) | doi:10.1038/nrd2112


Where are we going1

Where are we going?

  • Primary Goal

  • Understanding Brugada

  • Prevalence

  • Presentation

  • Prognosis

  • Therapy


Prevalence

Prevalence

  • In Thailand, estimated to be the second leading cause of death in men <40, after accidents.

  • In the Philippines, known as Bangungut- scream followed by sudden death during sleep- and in Japan as Pokkuri- unexpected sudden death at night.

  • At the Carolinas Medical Center, Charlotte, found in 0.4% of all EKGs.


Presentation

Presentation

  • Sudden cardiac arrest often the first symptom.

  • More common at night, esp when sleeping.

  • Ages 22-65- mean age of sudden death 41 +/- 15 years.


Where are we going2

Where are we going?

  • Primary Goal

  • Understanding Brugada

  • Prevalence

  • Presentation

  • Prognosis

  • Therapy


Prognosis

Prognosis

Risk Stratification based on-

1. Prior History of SCA: 69% recur within 5 years.

2. History of syncope

3. EKG abnormal at baseline or only after drug challenge?

4. Is a SVA inducible in the EP lab?

SCA- Sudden Cardiac Arrest

SVA- Sustained Ventricular Arrhythmia


Prognosis1

Prognosis

In 547 patients with type 1 Brugada syndrome with no prior history of SCD, the probability of SCA or VF during follow-up (average 2 years)

- Overall 8.2% with SCA or VFib.

Adapted from Brugada, J, Brugada, R, Brugada, P, Circulation 2003; 108:3092

SCA- Sudden Cardiac Arrest

SVA- Sustained Ventricular Arrhythmia


Where are we going3

Where are we going?

  • Primary Goal

  • Understanding Brugada

  • Prevalence

  • Presentation

  • Prognosis

  • Therapy


Treatment

Treatment

  • Implantable Cardiac Defibrillator

Prior History of SCA: 69% recur within 5 years.


Drug therapy

Drug Therapy?

  • Quinidine (Class IA) may blunt Ito.

  • Isoproterenol (Beta-adrenergic agonist) may augment L-type Ca++ current.


Brugada syndrome

Goal

Recognize Brugada I: coved ST segment in V1-V3, >2mm elevation, inverted T wave.


References

References

  • Antelevitch C et al. Brugada Syndrome: Report of the Second Consensus Conference. Heart Rhythm 2005. 2(4):429-440.

  • Benito and Brugada. Recurrent syncope: an unusual presentation of Brugada syndrome. Nature Clinical Practice 2006. 3(10): 573-577.

  • Brugada, J, Brugada, R, Brugada, P. Determinants of Sudden Cardiac Death in Individuals With the Electrocardiographic Pattern of Brugada Syndrome and No Previous Cardiac Arrest. Circulation 2003; 108:3092.

  • Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome: a multicenter report. J Am Coll Cardiol. 1992; 20: 1391–1396.

  • UpToDate. Brugada Syndrome and Sudden Cardiac Arrest.

  • Priori, S. G. et al. Genetic and Molecular Basis of Cardiac Arrhythmias: Impact on Clinical Management Part III. Circulation 1999;99:674-681.


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