brugada syndrome by dr. rabe3 alansi yemen

8/14/2019 BRUGADA SYNDROME BY DR. RABE3 ALANSI YEMEN

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On arrival in hospital he was fully alert although he felt lightheaded and He has been investigated,the noncardic causes ofsyncop are excluded & the cardic enzymes & echo are normal BUT;(ECG) shows changes were identical to those seen in the ECGsperformed three years previously,how to corrolate ECG findings

to clinical picture, what is next step for evaluation of this case ??

Case 1Case 1 ::40 year old man presented to the emergency department after losingconsciousness while driving his car. He had felt nauseous and lightheaded before managing to slow down and stop. A witness whostopped to help found him slumped unconscious over the steering

wheel, and he regained consciousness a few seconds later.


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The same Pt. underwent check up investigations before3 years, he had no symptoms & the heart & othersystems by clincal exam.were nomal.& imaging studiesincluding echo show no abnormalites,though ECG shows specific changes ; does it has


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Al-KWAIT University HospitalSanaa yemen


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Brugada syndromehas recently attractedgreat interest??

FirstFirst, The disease takes the lives, in manyinstaces as a first event, of previously healthyindiviuals in their forties,during their mostproductive years.

why

becau

se of::

SecondSecond ,once thought to be very rare, theBS is now recognized world wide & has a highprevalance in certain parts of world.


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Brugadasyndrome (BS) is :(BS) is :

an inherited form of cardiac arrhythmia and sudden deathan inherited form of cardiac arrhythmia and sudden death

presenting with :presenting with :

a typical electrocardiographicpattern ofSTSTsegmentsegment elevation in leadsV1 to V3leadsV1 to V3,,

andand displaying a right bundle branch blockQRS morphology

Definition:

It is most frequently diagnosed inYOUNG MALES (8:1 ratio ofmales:females

) of Asian origin.


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Interestingly, the natives knew the problem for manydecades.

In the Northeast of Thailand, this form of death was knownas Lai Tai (death during sleep).The indigenous believe that the young men died duringsleep 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 thewidow ghost.

In Philippines the phenomenon was known as Bangungut(scream followed by sudden death during sleep)

In Japan was known as Pokkuri (unexpected sudden deathat night)..

The first patient with this syndrome was seen in1986.The first paper including 8 patients was publishedin 1992Since then, there has been an exponentialincrease in the number of patients recognized all

History Of Brugada syndromeHist

ory Of Brugada syndrome


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Brugada syndromeis affecting 5/10 000 of thegeneral population, ButBut ,is,is estimated to beresponsible for at least 4% of all sudden deaths andat least 20% of sudden deaths in patients withstructurally normal hearts

the Brugada syndrome has progressed from being arare disease to one that is second only to automobileaccidents as a cause of death among young adults insome countries.

TheTheIncidenceIncidence


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Clinical Characteristics

PT. presents with :

The syncope and cardiac arrest thattypically occurring in the 3ed and 4thdecade of life, and usually at rest or duringsleep.

The manifestations of BS are due to :episodes ofpolymorphicventricular

tachycardia-ventricular fibrilationsWhen the episodesterminate

syncopalattacks.

suddendeath

When the episodes aresustained

Thus ,these manifestations can range widely:at one end of the spectrum we have

asymptomatic individuals & at other end thosewho die suddenly.


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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 typeand gradually descending) in more than one rightprecordial lead (V1-V3)*, with or withoutadministration of a sodium channel blocker) andand

A. and/orB. below:A at least one of the following findings:

Documented ventricular fibrillationSelf-terminating polymorphic ventricular

tachycardiaA family Hx of sudden cardiac deathCoved-type ECGs in family membersElectrophysiologic inducibilitySyncope or nocturnal agonal respiration

B An SCN5A mutation

Diagnostic Criteria @ Update 2005 @
http://www.genetests.org/servlet/access?qry=139&db=genestar&fcn=term&gtreport2=true&id=8888890&key=lyvQGez2WEEGw&format=framehttp://www.genetests.org/servlet/access?qry=139&db=genestar&fcn=term&gtreport2=true&id=8888890&key=lyvQGez2WEEGw&format=frame


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The ECG manifestations ofthe Brugada syndrome


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The ECG manifestations of the

,Brugada syndrome


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The dynamic ECG manifestations of

,the Brugada syndrome

saddle backcoved (type 1).


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sodium channel blockerssodium channel blockers, a febrile state, vagotonic agents, hyperkalemia, hypokalemia,

hypercalcemia, and alcohol and cocaine toxicity

The ECG manifestations ofBrugada syndrome are oftenconcealed & dynamic but can beunmaskedormodulatedby:


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1, 2, 3, 4 minduring i.v.

infusion of50 mg ajmaline.

saddleback (type

2)

unmasked &becomes coved

(type 1).

The use of Drug challenge to unmask BS


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VF and sudden death in Brugada syndrome usually occurat rest and at night.

Circadian variation ofsympathovagal balance,hormones, and other metabolic factors are likely tocontribute to this circadian pattern.

Circadian pattern of VF episodes inpatients with BS

We can noticeventricular episodesincrease at mid nightdue to Circadianpattern of BS


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Genetic Factors Underlying

Brugada Syndrome

The first and only gene to be linked to Brugadasyndrome is SCN5A, the gene that encodes for thesubunit of the cardiac sodiumchannel gene.

SCN5Amutations account for 18% to 30% of Brugadasyndrome cases

Genetictesting is recommended, however, to Support the clinical diagnosis, For early detection of relatives atpotential risk,


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Electrophysiological (EP) study

Electrophysiological (EP) study:This test may be performed to determinethe inducibility of arrhythmias in B S.

The predictive value of the EP study isstill debated.

Risk Stratification and


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Risk StratificationRisk Stratification

1. Patients have a relatively highriskforsudden arrhythmic death, even in the absenceof a history of cardiac arrest: 8.2%experienced suddendeath

2.Individuals with a spontaneously abnormal type1 ECG

carried a 7.7-foldhigherriskof developing anarrhythmicevent during a lifetime as compared withindividuals inwhom the ECG diagnostic of Brugada syndrome

was


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3.Programmedelectricalstimulation that inducesa sustainedventricular arrhythmia is the strongestmarker ofrisk,associated with an 8-fold higher risk of (aborted)

sudden death than in noninducible patients.

Risk Stratification

5.Familial forms of the disease are notassociated with aworse prognosis than are sporadic cases because

a positive

4. Male gender is another riskfactor forsudden death. Men

had a 5.5-fold higher risk of sudden deaththan did women.


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Thepharmacologicalapproach

The only agent on the US market isquinidine.

Class 1C antiarrhythmic drugs (eg,flecainide and propafenone) and class IAagents (eg,procainamide) arecontraindicated

Antiarrhythmic agents such asamiodaroneand B-blockers have been shown to be

ineffective.


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The most recent drug, cilostazol, whichnormalizes the ST segmentmost likely by

augmenting the calcium current (ICa), as well asby reducing I to secondary to an increase inheart rate.

An experimental agent, tedisamil, with potentaction to block I to among other outward currentshas been suggested as a therapeutic candidate

The pharmacological approach


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1 Symptomatic patients

displaying the type 1 BrugadaECG(either spontaneously or aftersodium channel blockade)+presenting with aborted sudden

death.

: Symptomatic patients

Pt. shouldundergoICDimplantation

2 Similar patients+presenting related symptomssuch as syncope, seizure, ornocturnal agonal respiration also

Recommendationsfor ICD implantation


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1 Asymptomatic patients

displaying a type 1 ECG+a family history of suddencardiac death

Asymptomatic: patients

should be closelyfollowed up

should undergoEPS If inducible,then the patientshould receive anICD.

2 Asymptomaticpatients who develop atype 1 ECGonly aftersodium channel blockade

+ no family history

Recommendationsfor ICDimplantation


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The PrognosisThe Prognosis

prognosis of asymptomatic individuals :One third of patients having suffered from syncopal

episodes develops a new episode ofpolymorphicventricular tachycardia within 2 years.

This syndrome has a very poor prognosis when leftuntreated.

Untreated Brugadas syndrome is associated with a 10%mortality per year with up to 40% of patients experiencingarrhythmias in the future.


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brugada syndrome by dr. rabe3 alansi yemen

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