Case 1 :

40 year old man presented to the emergency department after losing

consciousness while driving his car. He had felt nauseous and light
headed before managing to slow down and stop. A witness who
stopped to help found him slumped unconscious over the steering
wheel, and he regained consciousness a few seconds later.
On arrival in hospital he was fully alert although he felt light
headed and He has been investigated,the noncardic causes of
syncop are excluded & the cardic enzymes & echo are normal BUT;
(ECG) shows changes were identical to those seen in the ECGs
performed three years previously ,how to corrolate ECG findings
to clinical picture, what is next step for evaluation of this case ??
The same Pt. underwent check up investigations before
3 years, he had no symptoms & the heart & other
systems by clincal exam.were nomal.& imaging studies
including echo show no abnormalites,
though ECG shows specific changes ; does it has
Al-KWAIT University Hospital
Sanaa yemen
why
Brugada syndrome has recently attracted
great interest ??
becau
seFirst,
of
First
:
The disease takes the lives, in many
instaces as a first event, of previously healthy
indiviuals in their forties,during their most
productive years.

Second ,once thought to be very rare, the

BS is now recognized world wide & has a high
prevalance in certain parts of world.
 : Definition

Brugada syndrome (BS) is :

an inherited form of cardiac arrhythmia and sudden death

presenting with :
a typical electrocardiographic pattern of ST
segment elevation in leadsV1 to V3,
and displaying a right bundle branch block
QRS morphology

It is most frequently diagnosed in

YOUNG MALES (8:1 ratio of
males:females) of Asian origin.
 History
Interestingly, Of Brugada
the natives syndrome
knew the problem for many
decades.

In the Northeast of Thailand, this form of death was known

as Lai Tai (death during sleep).
The indigenous believe that the young men died during
sleep because widow ghosts came to take them away.
Many young men actually dress still as women to go to
sleep at night - with the hope that it would mislead the
widow ghost.

In Philippines the phenomenon was known as Bangungut

(scream followed by sudden death during sleep)
The first patient with this syndrome was seen in
In Japan was known as Pokkuri (unexpected sudden death
1986.
at night)..
The first paper including 8 patients was published
in 1992
Since then, there has been an exponential
increase in the number of patients recognized all
 The Incidence

the Brugada syndrome has progressed from being a

rare disease to one that is second only to automobile
accidents as a cause of death among young adults in
some countries.

Brugada syndrome is affecting 5/10 000 of the

general population, But ,is estimated to be
responsible for at least 4% of all sudden deaths and
at least 20% of sudden deaths in patients with
structurally normal hearts
 Clinical Characteristics
PT. presents with :
The syncope and cardiac arrest that
typically occurring in the 3ed and 4th
decade of life, and usually at rest or during
sleep.

The manifestations of BS are due to :

episodes of polymorphic ventricular
When the episodes syncopal
terminate
tachycardia-ventricular fibrilations attacks.
When the episodes are sudden
sustained death
Thus ,these manifestations can range widely:
at one end of the spectrum we have
asymptomatic individuals & at other end those
who die suddenly.
 Diagnostic Criteria @ Update 2005 @
Brugada syndrome is definitively diagnosed when :
 Type 1 ECG (elevation of the J wave 2 mm with (a
negative T wave )and ST segment that is coved type
and gradually descending) in more than one right
precordial lead (V1-V3)*, with or without
administration of a sodium channel blocker) and
 A. and/or B. below:
 A at least one of the following findings:
Documented ventricular fibrillation
Self-terminating polymorphic ventricular
tachycardia
A family Hx of sudden cardiac death
Coved-type ECGs in family members
Electrophysiologic inducibility
Syncope or nocturnal agonal respiration
 B An SCN5A mutation
The ECG manifestations of
the Brugada syndrome
The ECG manifestations of the
,Brugada syndrome
The dynamic ECG manifestations of
,the Brugada syndrome

coved (type 1). ‘saddle back

The ECG manifestations of
Brugada syndrome are often
concealed & dynamic but can be
unmasked or modulated by:
 sodium channel blockers,
blockers
 a febrile state,
 vagotonic agents,
 hyperkalemia, hypokalemia,
hypercalcemia,
 and alcohol and cocaine toxicity
 The use of Drug challenge to unmask BS

unmasked &
‘saddle 1, 2, 3, 4 min
becomes coved
back (type during i.v.
infusion of (type 1).
2) 50 mg ajmaline.
Circadian pattern of VF episodes in
patients with BS
 VF and sudden death in Brugada syndrome usually occur
at rest and at night.

 Circadian variation of sympathovagal balance,

hormones, and other metabolic factors are likely to
contribute to this circadian pattern.
We can notice
ventricular episodes
increase at mid night
due to Circadian
pattern of BS
 Genetic Factors Underlying
Brugada Syndrome
 The first and only gene to be linked to Brugada
syndrome is SCN5A, the gene that encodes for the
subunit of the cardiac sodium channel gene.

 SCN5A mutations account for 18% to 30% of Brugada

syndrome cases

• Genetic testing is recommended, however, to

• Support the clinical diagnosis,
• For early detection of relatives at
potential risk,
 Risk Stratification and
Electrophysiological (EP) study

Electrophysiological (EP) study:

This test may be performed to determine

the inducibility of arrhythmias in B S.

The predictive value of the EP study is

still debated.
 Risk Stratification

1. Patients have a relatively high risk for

sudden arrhythmic death, even in the absence
of a history of cardiac arrest: 8.2%
experienced sudden death
2.Individuals with a spontaneously abnormal type
1 ECG
carried a 7.7-fold higher risk of developing an
arrhythmic
event during a lifetime as compared with
individuals in
whom the ECG diagnostic of Brugada syndrome
was
 Risk Stratification
3. Programmed electrical stimulation that induces
a sustainedventricular arrhythmia is the strongest
marker of risk,
associated with an 8-fold higher risk of (aborted)
sudden death than in noninducible patients.

4. Male gender is another risk factor for

sudden death. Men
had a 5.5-fold higher risk of sudden death
than did women.
5. Familial forms of the disease are not
associated with a
worse prognosis than are sporadic cases because
a positive
 Therapeutic Recommendations for
Brugada Syndrome

Currently, an ICD is the only proven effective

treatment for the disease

Treatment with pharmacological

agents is usually unsuccessful
 The pharmacological approach

The only agent on the US market is

quinidine.
Class 1C antiarrhythmic drugs (eg,
flecainide and propafenone) and class IA
agents (eg,procainamide) are
contraindicated

Antiarrhythmic agents such as

amiodarone
and B-blockers have been shown to be
ineffective.
 The pharmacological approach

The most recent drug, cilostazol, which

normalizes the ST segment most likely by
augmenting the calcium current (ICa), as well as
by reducing I to secondary to an increase in
heart rate.

An experimental agent, tedisamil, with potent

action to block I to among other outward currents
has been suggested as a therapeutic candidate
 Recommendations
for ICD implantation

: Symptomatic patients

 1 Symptomatic patients
displaying the type 1 BrugadaECG
(either spontaneously or after
sodium channel blockade) Pt. should
+ presenting with aborted sudden undergo
death. ICD
2 Similar patients implantati
+ presenting related symptoms on
such as syncope, seizure, or
nocturnal agonal respiration also
 Recommendations
for ICD
implantation
Asymptomatic: patients

1 Asymptomatic patients
displaying a type 1 ECG
+ a family history of sudden
cardiac death
2 Asymptomatic
patients who develop a
type 1 ECG only after
sodium channel blockade
+ no family history
should undergo
EPS If inducible,
then the patient
should receive an
ICD.
should be closely
followed up
 The Prognosis
This syndrome has a very poor prognosis when left
untreated.

Untreated Brugada’s syndrome is associated with a 10%

mortality per year with up to 40% of patients experiencing
arrhythmias in the future.
 prognosis of asymptomatic individuals :
One third of patients having suffered from syncopal
episodes develops a new episode of polymorphic
ventricular tachycardia within 2 years.