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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
Therapy

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

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
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


II: V1-V3 ST segment
elevation >2mm,
saddleback shape, pos
or biphasic T.
III: <1 mm elevation,
either coved or
saddleback.

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

0
-85mVolts

2
3

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

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 Discovery 5, 10341049 (December 2006) | doi:10.1038/nrd2112

Prevalence
In Thailand, estimated to be the second
leading cause of death in men <40, after
accidents.
In the Philippines, known as Bangungutscream followed by sudden death during
sleep- and in Japan as Pokkuriunexpected sudden death at night.
At the Carolinas Medical Center,
Charlotte, found in 0.4% of all EKGs.

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.

Prognosis
Risk Stratification based on1. 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

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.

SVA Noninducible, percent


(95% CI)

SVA Inducible, percent (95%


CI)

Spontaneously abnormal

4.1 (1.4-11.7)

27.2 (17.3-40.0)

Abnormal after drug


challenge

1.2 (0.2-6.6)

9.7 (2.3-33.1)

Spontaneously abnormal

1.8 (0.6-5.1)

14.0 (8.1-23.0)

Abnormal after drug


challenge

0.5 (0.1-2.7)

4.5 (1.0-17.1)

Prior syncope
EKG

No prior syncope
EKG

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


SCA- Sudden Cardiac Arrest
SVA- Sustained Ventricular Arrhythmia

Treatment
Implantable Cardiac Defibrillator
Prior History of SCA: 69% recur within 5 years.
SVA Noninducible, percent
(95% CI)

SVA Inducible, percent (95%


CI)

Spontaneously abnormal

4.1 (1.4-11.7)

27.2 (17.3-40.0)

Abnormal after drug


challenge

1.2 (0.2-6.6)

9.7 (2.3-33.1)

Spontaneously abnormal

1.8 (0.6-5.1)

14.0 (8.1-23.0)

Abnormal after drug


challenge

0.5 (0.1-2.7)

4.5 (1.0-17.1)

Prior syncope
ECG

No prior syncope
ECG

Drug Therapy?
Quinidine (Class IA) may blunt Ito.
Isoproterenol (Beta-adrenergic agonist) may augment L-type Ca++
current.

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

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: 13911396.
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.