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Morning Report
June, 2008
Jessie Stewart
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
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
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?
Prognosis
In 547 patients with type 1 Brugada syndrome with no prior history of SCD, the
Spontaneously abnormal
4.1 (1.4-11.7)
27.2 (17.3-40.0)
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)
0.5 (0.1-2.7)
4.5 (1.0-17.1)
Prior syncope
EKG
No prior syncope
EKG
Treatment
Implantable Cardiac Defibrillator
Prior History of SCA: 69% recur within 5 years.
SVA Noninducible, percent
(95% CI)
Spontaneously abnormal
4.1 (1.4-11.7)
27.2 (17.3-40.0)
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)
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