Cardiac Channelopathies and

Sudden Cardiac Death:

Management & ICD Indication
} Introduction
} Brugada Syndrome
} Long QT Syndrome
} Short QT Syndrome
} Catecholaminergic Polymorphic VT (CPVT)
 ECG Pattern

Primary Prevention

Sudden Cardiac
Arrest

Secondary Prevention

Survivor
Grundy SM, et al. Assessment of cardiovascular risk by use of multiple-risk-factor assessment equations. Circulation. 1999;100:1481–92.
EHRA/HRS/APHRS Guidelines 2014
ESC Guidelines 2015
 20 18%
18
16

% Arrhythmic Death
14
12 11%
10
8%
8
6
4
2
0
1 Year 2 Years 3 Years

Pratt CM. Circulation. 1998;98(suppl I):1494-1495.

 Unexplained syncope
Non -syncopal VT w/symptoms
1.00
VF
Transient correctable VT/VF

Cumulative Survival (%)

Asymptomatic VT
.90 VT w/syncope

.80

.70 P = 0.007

.65
0 1 2 3
Years
Anderson JL, et al. Circulation. 1999;99:1692-1699.
} For secondary prevention, ICD implantation had no doubt

} What about primary prevention ?

} Are there any predictors so we get maximum benefit for ICD that
being implanted ?

} Risk Predictors is needed

} Clinical Predictors

} ECG Predictors

} Electrophysiological Predictors
} A history of syncope has been associated with an increased incidence
of future arrhythmic events in several studies, including a meta-
analysis

} Priori’s group have initially demonstrated that the association of

syncope and spontaneous type 1 ecg pattern has the best predictive
value to identify individuals at high risk, and not a history of syncope
as a single risk factor
} Genetic Markers A genetic defect on the SCN5A gene has not been
associated with a higher risk of future arrhythmic events in several
studies

} Suggesting that genetic analysis is a useful diagnostic parameter but it

is not helpful for risk stratification
} A spontaneous type 1 ECG pattern of BrS has been consistently
associated with a worse outcome in large studies
} A consistent finding in nearly all BrS series is that patients with
spontaneous type I ECG have a greater risk of arrhythmic events than
drug induced one

} The problem is that only a minority of patients have a consistent

spontaneous type 1 pattern when repeated ECGs are analyzed

C. Antzelevitch et al. / Journal of Arrhythmia 32 (2016) 315–339

Jafib, 2016
C. Antzelevitch et al. / Journal of Arrhythmia 32 (2016) 315–339
Long QT Syndrome (LQTS)
QT & QTc
 Schwartz Score

>4 : high probability

2-3 : intermediate

< 1 low probability

 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
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
Bidirectional VT
Clinical Diagnosis
Bidirectional VT
During EST
Thank You