Predicting and Preventing

Sudden Cardiac Death in The Young

Erika Maharani
Sardjito General Hospital
Yogyakarta
1 9 MEN
in will experiences SUDDEN CARDIAC DEATH,
most before age 70 years
 Outline

• Definition SCD in The Young

• Etiology SCD in The Young
• Predicting and Preventing SCD
• Electrocardiogram Review of SCD
Definition of SCD in The Young
 Definition of Sudden Death (SD)

• Sudden Death or its synonyms are generally defined as natural, unex- pected
death within 1 hour of the onset of symptoms.
• When death occurs unwitnessed within 24 hours of being seen alive and
functioning normally, this is also termed Sudden Death.
• Depending on the underlying cause
• SD from a cardiac cause
• SD due to noncardiac causes, for example, intracranial hemorrhage, epilepsy,
pulmonary embolism, or asthma.
• Four temporal elements : prodromes, onset of the terminal event, cardiac
arrest, and biological death.

Circ Arrhythm Electrophysiol. 2010;3:96-104

 Definition of Sudden Death (SD)

• Sudden Death or its synonyms are generally defined as natural, unex- pected
death within 1 hour of the onset of symptoms.
• When death occurs unwitnessed within 24 hours of being seen alive and
functioning normally, this is also termed Sudden Death.
• Depending on the underlying cause
• SD from a cardiac cause
• SD due to noncardiac causes, for example, intracranial hemorrhage, epilepsy,
pulmonary embolism, or asthma.
• Four temporal elements : prodromes, onset of the terminal event, cardiac
arrest, and biological death.

Circ Arrhythm Electrophysiol. 2010;3:96-104

 Sudden Cardiac Death: Size of The Problem

1 – 10 10-75 3M
Per 100.000 Per 100.000 <1%
in those in those Cases worldwide Survival rate
1 – 35 y.o. 35 – 64 y.o. annually

European Heart Journal (2015) 36, 2793–2867

Circ Arrhythm Electrophysiol. 2010;3:96-104
Etiology of SCD in The Young
Different Age Etiology
Age-related Distribution of 470 Sudden Cardiac Deaths
in Persons Aged 1–35 Years

Male deaths constituted 67% of

all sudden cardiac deaths

Winkel BG, et al. European Heart Journal (2011) 32, 983–990

 Causes of Sudden Cardiac Death in The Young
Structural
Heart Disease
• Ischemic heart disease
• Nonischemic cardiomyopathy
• Valvular heart disease
• Congenital heart disease
• Hypertrophic cardiomyopathy
• Arrhythmogenic right ventricular
dysplasia
• Anomalous coronary artery origin
Primary
Electrophysiological
Conditions
• Congenital long-QT syndromes
• Short-QT syndrome
• Wolff-Parkinson-White syndrome
• Brugada syndrome
• Catecholaminergic polymorphic
ventricular tachycardia
Kirchhof P, et al. Heart 2006;92:1873–1878
Etiologies for SCD in The Young

Clinical Medicine 2018 Vol 18, No 2: s17–s23

Predicting and Preventing SCD
 Predicting of Sudden Cardiac Death
in Special Settings

General population for the risk of SCD

Screening

Family members of SCD victims

Patients with documented or suspected

ventricular arrhythmias

Esthes M. Circulation. 2011;124:651-656

Multiple Approaches to Prevent SCD in The Young

Thiene G. Clinical Medicine 2018 Vol 18, No 2: s17–s23

“ECG signs of inheritable arrhythmogenic diseases
seems to be an important part of clinical practice and
can contribute to the early identification of patients
at risk of Sudden Cardiac Death”

European Heart Journal doi:10.1093/eurheartj/ehv316

Electrocardiogram Review of SCD
“ECG signs in patients at risk of Sudden Cardiac Death”

Kirchhof P, et al. Heart 2006;92:1873–1878

 Brugada Syndrome (BrS)
Clinical Presentation and Diagnosis

• 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
Brugada Syndrome (BrS)
Electrocardiogram of BrS
Brugada Syndrome (BrS)
Electrocardiogram of BrS
Brugada Syndrome (BrS)
ECG Features
 Wolf Parkinson White Syndrome
Reentry around Accessory Pathway

• Ventricular preexcitation on the ECG during sinus rhythm

• Widened QRS complex - delta wave
• Narrow QRS complex tachycardia of 170 to 250 bpm
Wolf Parkinson White Syndrome
Electrocardiogram
Wolf Parkinson White Syndrome
Malignant ECG
Long QT Syndrome (LQTS)
QT & QTc
 Schwartz Score

>4 : high probability

2-3 : intermediate

< 1 low probability

Long QT Syndrome (LQTS)
Clinical Implication
 Short QT Syndrome (SQTS)
Diagnosis Criteria

≥ 4 : high probability

3 : intermediate

 2 low probability
Short QT Syndrome (SQTS)
Electrocardiogram
 Catecholaminergic Polymorphic VT (CPVT)
Clinical Implication

• 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
Catecholaminergic Polymorphic VT (CPVT)
Bidirectional VT During Exercise Stress Test
Catecholaminergic Polymorphic VT (CPVT)
Bidirectional VT During Exercise Stress Test
Hypertrophic Cardiomyopathy
Clinical Presentation

• Symptoms include :
• Shortness of breath with exercise
• chest pain (usually with exercise)
• Diziness (at rest or with exercise)
• blackouts
• Palpitations
• No symptoms

• Most common cause of death in young

people
• Risk of sudden death ~ 1% per year
• Intensive exercise can increase risk
Hypertrophic Cardiomyopathy
Electrocardiogram of HCM
Arrhythmogenic RV Cardiomyopathy
Arrhythmogenic RV Cardiomyopathy
Clinical Presentation

• Arrhythmogenic RV dysplasia, ARVC,

and arrhythmogenic cardiomyopathy
• More patients came due to ventricular
arrhythmias compare to RV dysfunction.
• ARVC/D - the heart muscle of the right
ventricle (RV) is replaced by fat and/or
fibrous tissue.
• It accounts for up to 17% for all SCD in
the young
 Arrhythmogenic RV Cardiomyopathy
Electrocardiogram

Triangle of dysplasia” in ARVC/D

 Arrhythmogenic RV Cardiomyopathy
Electrocardiogram

Epsilon waves are caused by postexcitation of the myocytes in the right ventricle (V1-V2).
It is the most characteristic finding in arrhythmogenic RV dysplasia (ARVD/C)
 Arrhythmogenic RV Cardiomyopathy
Electrocardiogram

Epsilon waves are caused by postexcitation of the myocytes in the right ventricle (V1-V2).
It is the most characteristic finding in arrhythmogenic RV dysplasia (ARVD/C)
The first presenting symptom may be sudden cardiac death
 Take Home Message

• Sudden cardiac death is the most common medical cause of death in the
young.
• There remains significant debate over the best strategy to prevent
sudden cardiac death in the young and the role of the electrocardiogram
in preparticipation screening.
• ECG signs of inheritable arrhythmogenic diseases seems to be an
important part of clinical practice and can contribute to the early
identification of patients at risk of Sudden Cardiac Death
Matur Nuwun