brugada syndrome dr. raj santan

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40 year old male with :FeverRight parotid abscessLeft knee septic arthritis

k/c/o DM – on regular treatment with OHA’SO/E:

PR - 100/ mt ,regularB.P - 120/80 mmhg

C.V.S – S1S2+ , no murmurs

R.S – NVBS, b/l creps+C.N.S – NFND

P/A – Soft, no organomegaly

HR- 100/ mtAxis- LAD sinus rhythmST segment elevation ( coved in shape ) in V1 –

V3

T wave inversion in V1 –V3

Suggestive of BRUGADA SIGN

first described in 1992By Joseph brugada, Pedro brugada

and Ramon brugada

Is a clinical entity characterized by 1.a typical ECG pattern( RBBB and persistent ST elevation in precordialleads) and

2. sudden cardiac death

Inherited in an autosomal

dominant pattern

More common in males.

Higher prevalence in south east Asian

populations.

It is the major cause of sudden unexplained death syndrome (SUDS)

is the most common cause of sudden

death in young men without known

underlying cardiac disease

known colloquially in the Philippines

as bangungut (“to rise and moan in

sleep”), in Japan as pokkuri(“sudden and

unexpectedly ceased phenomena”) and in

Thailand as Lai Tai (“death during sleep”).

The mean age of sudden death is 41, with

the age at diagnosis ranging from 2 days

to 84 years.

20 % of the cases are associated with mutations in the gene coding for sodium channel SCN 5A, located in short arm of 3 rd chromosome

Loss-of-function mutations in this gene

lead to a loss of the action potential dome

of some epicardial areas of the right ventricle

Sodium channelopathy

Loss of action potential dome of some

epicardial areas of right ventricle

Transmural dispersion

of repolarization

Epicardial dispersion

of repolarization

ST-segment elevation and

the development of a

vulnerable window across

the ventricular wall

Facilitates phase 2 re-entry

which generates reentrant

extrasystole that captures

the vulnerable window and

precipitates VT/ V fib

coved ST segment elevation > 2 mm followed by negative T wave in V1 to V3 diagnostic , also called as

“Brugada sign”

saddle back configuration of ST elevation > 2 mm ending in a positive or biphasic T wave In V1 to V3 (atleast 2 leads)

ST segment

elevation < 1

mm with either

type 1 or type 2

morphology

Documented ventricular fibrillation (VF) or

polymorphic ventricular tachycardia (VT).

Family history of sudden cardiac death at

<45 years old .

Coved-type ECGs in family members.

Inducibility of VT with programmed

electrical stimulation .

Syncope.

Nocturnal agonal respiration.

Fever

Ischaemia

Hypokalaemia

Hypothermia

Post DC cardioversion

Drug induced

• Sodium channel blockers eg: Class IC

Flecainide, Propafenone class IA: ajmaline

and procainamide

• Calcium channel blockers

• Alpha agonists

• Beta Blockers

• Nitrates

• Cholinergic stimulation

• Cocaine

• Alcohol

Quinidine has been found to both

decrease the number of VF episodes and

correct spontaneous ECG changes,

possibly via inhibiting Ito channels