Cardiac Channelopathies and

Sudden Cardiac Death:

ECG Review
} Introduction
} Brugada Syndrome
} Long QT Syndrome
} Short QT Syndrome
} Catecholaminergic Polymorphic VT (CPVT)
 Age
Etiology

• Over 35 yrs of age

• Coronary Heart Disease
• Under 35 yrs
• Cardiomyopathies
• Channelopathies
• Congenital Heart Disease
• Myocarditis
Circ Res. 2015;116:1887-1906
 “ECG and echocardiographic
signs of inheritable
arrhythmogenic diseases a
seems to be an important part of
clinical practice and can
contribute to the early
identification of patients at risk
of SCD”
European Heart Journal doi:10.1093/eurheartj/ehv316
Brugada Syndrome
Long QT Syndrome
Short QT Syndrome
} BrS could be responsible for 4%–12% of all SCD and up to 20% of SCD
in patients with structurally normal hearts
} The current diagnosis of BrS is based on :
◦ Type I ECG pattern
◦ Any of the following clinical features:
– documented VF, PVT,
– Inducibility of VT with programmed electrical stimulation,
– Family history of SCD at younger than 45 years of age
– Covered-type ECG in family members
– Unexplained syncope
– Nocturnal agonal respiration
Current ESC Guideline
Diagnosis of BrS

European Heart Journal doi:10.1093/eurheartj/ehv316

 Proposed Shanghai Score System
J-Wave syndromes expert consensus conference
report

C. Antzelevitch et al. / Journal of Arrhythmia 32

(2016) 315–339
} Unmask the Brugada Pattern Type 1

} Intravenous administration of Na+ channel blocking drugs

} Useful in bringing out Type 1 Brugada pattern on the ECG when ECG
changes are not diagnostic
Long QT Syndrome (LQTS)
QT & QTc
 Schwartz Score

>4 : high probability

2-3 : intermediate

< 1 low probability

Current ESC Guideline
Diagnosis of LQTS

European Heart Journal doi:10.1093/eurheartj/ehv316

 Long QT Syndrome
Case Ilustration

} Female 34 y.o.
} Frequent Syncope
} Witnessed VT/VF
} SCA Survivor
} Referred from Jember
Measure QTC 550 ms
Long QT Syndrome (LQTS)
Types
Long QT Syndrome (LQTS)
Clinical Implication
QT Interval
Short – Normal – Long
 Short QT Syndrome
Diagnosis Criteria

} Female : < 370 ms

} Male : < 360 ms
} Cardiac arrest, unexplained
syncope

Points ≥ 4 :High-probability
Point 3 :Intermediate-probability
Poin ≥2 :Low-probability
Current ESC Guideline
Diagnosis of Short QT Syndrome

European Heart Journal doi:10.1093/eurheartj/ehv316

Short QT Syndrome
ECG
Catecholaminergic
Polymorphic VT (CPVT)
 CPVT
Prevalence

} CPVT is a pathological condition whereby intense physical exercise or

acute emotional stress can trigger abnormal heartbeat
} estimated prevalence of 1:10,000
} CPVT commonly manifests at an early age and has poor spontaneous
outcome
Current ESC Guideline
CPVT
Bidirectional VT
Clinical Diagnosis
Bidirectional VT
During EST
Thank You