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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

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
slide4
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.

1

0mVolts

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0

3

1

-85mVolts

4

4

4

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

slide9
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.
slide19
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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