ERDIE

ERDIE C.
C. FADREGUILAN,
FADREGUILAN, MD
MD
FPCP,
FPCP, FPCC
FPCC
Educational Background:
University of Santo Tomas, 1995
Santo Tomas Univ. Hospital, 1996

Post Graduate Training:

Residency Training in Internal Medicine –UP PGH
Fellowship in Adult Cardiology- UP PGH
Training in Clinical Cardiac Electrophysiology and Pacing
-Philippine Heart Center

Present Positions:
Consultant, Philippine Heart Center
SUDDEN CARDIAC DEATH

Erdie C. Fadreguilan, MD
 SUDDEN CARDIAC DEATH
• Definition:

“Natural death due to cardiac

causes heralded by abrupt loss of
consciousness within one hour of
the onset of acute symptoms”
 SCA- A Public Health Issue
SCD claims more The Facts:
lives each year than • Significant Killer in America
these other diseases
• 95% fatal without ICD
combined: protection5 - 98% survival
with ICD protection
• Nearly 1000 SCA deaths/day6
Lung
Cancer1
162,500 335,000 • ICDs are Class I indicated for
most at-risk Pts7
Breast • New guidelines clearly outline
41,400
Cancer1 at-risk groups7
AIDS2 14,000 • Studies show that ICDs are
SCD3 cost effective8
 Incidence of SCD in Specific Populations
and Annual SCD Numbers
GROUP

General population

Patients with high

coronary-risk profile

Patients with previous

coronary event

Patients with ejection

fraction <35%,
congestive heart failure

Patients with previous

out-of-hospital cardiac
arrest
Patients with previous
myocardial infarction,
low ejection fraction,
and ventricular
tachycardia 0 5 10 20 25 30 0 100,000 200,000 300,000

Incidence of Sudden No. of Sudden

Death Deaths
(% of group) Per Year
Myerburg RJ. Circulation.1998;97:1514-1521.
 What Is SCA?
• Electrical system in heart malfunctions
• Heart unexpectedly, abruptly stops beating
• Often caused by an abnormal heart rhythm
called ventricular fibrillation (VF)
– VF accounts for half of all cardiac deaths
• Rapid, chaotic heartbeat
• Lower heart chambers, or ventricles,
spasm
• Heart functioning stops
• Lack of oxygen in body, brain is dead
 Etiology of Sudden Cardiac Death
• An estimated 13 million people had coronary heart disease (CHD) in the U.S. in 2002.
1

• Sudden death was the first manifestation of CHD in 50% of men and 63% of women.
1

• CHD accounts for at least 80% of sudden cardiac deaths in Western cultures.
3

Etiology of Sudden Cardiac Death2,3

5% Other*

15% 80%
Cardiomyopathy Coronary
Heart
* ion-channel abnormalities, valvular or congenital heart disease, other
causes Disease
1
American Heart Association. Heart Disease and Stroke Statistics—2003 Update. Dallas, Tex.: American Heart
Association; 2002.
2
Adapted from Heikki et al. N Engl J Med, Vol. 345, No. 20, 2001.
3
Myerberg RJ. Heart Disease, A Textbook of Cardiovascular Medicine. 6th ed. P. 895.
 SUDDEN CARDIAC DEATH

• Epidemiology
– SCD due to Coronary Artery
disease: single most important
cause of death in adult population
– Ventricular Fibrillation: 75-80%
– Bradyarrhythmias: minority
– SCD in the absence of CAD, CHF:
5-10% of cases
 Arrhythmic Cause of SCD
12%
Other Cardiac
Cause

88%
Arrhythmic
Cause

.
Albert CM. Circulation. 2003;107:2096-2101
Underlying Arrhythmia of Sudden Cardiac
Arrest

Primary
VF
8% Torsades
de Pointes
13%

VT Bradycardia
62% 17%

Adapted from Bayés de Luna A. Am Heart J. 1989;117:151-159.

Sustained Monomorphic VT
72-year-old woman with CHD
VF with Defibrillation (12-lead ECG)
Torsades de Pointes
 SCA Survival & Mortality
Data
• At least 335,000 SCA deaths in the U.S.
each year
• Only 5 to 10% survive first episode of SCA
• Roughly two-thirds of SCA deaths occur
out-of-hospital
– 5% estimated survival rate

Seidl K, Senges J. Card Electrophysiol Rev. 2003;7:5-13.

Heart Disease and Stroke Statistics — 2005 Update. AHA. www.americanheart.org
Crespo EM, Kim J, Selzman KA. Am J Med Sci. 2005;329:238-246.
Zheng ZJ, et al. Circulation. 2001;104:2158-2163.
Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484.
SCA Resuscitation Success vs.
Time*
100

90
Chance of success reduced
80
7 - 10% each minute
70

60
%
Success 50

40
*Non-linear
30

20

10

0 1 2 3 4 5 6 7 8 9
Time (minutes)
Cummins RO. Annals Emerg Med. 1989;18:1269-1275.
Can SCA be Prevented?
• Research and clinical studies have
identified several conditions that put
patients at a high risk for SCA

• Patients can be evaluated for these known

risk factors before they experience a SCA

• Treatment options are available that can

prevent sudden cardiac death
Risk Factors for Sudden Cardiac
Arrest
• Previous Sudden Cardiac Arrest (SCA) Event
• Prior Episode of Ventricular Tachyarrhythmia
(VT)
• Previous Myocardial Infarction (MI)
• Coronary Artery Disease (CAD)
• Heart Failure
• Genetic diseases
– Long QT Syndrome
– Hypertrophic Cardiomyopathy (HCM)
– Brugada Syndrome
– ARVD
– SUDS
Sudden Cardiac Arrest Survivors
• Highest risk factor for Sudden Cardiac Arrest is
a previous SCA event

• 30 to 50% of SCA survivors will experience

another SCA event within one year

• First-degree relatives of SCA patients have a

50% higher risk of MI or primary cardiac arrest

Myerburg RJ. Heart Disease, A Textbook of Cardiovascular Medicine, 5th ed, Vol 1.
Philadelphia: WB Saunders Co;1997:ch 24.
Fogoros RN. Practical Cardiac Diagnosis: Electrophysiologic Testing, 2nd ed. Blackwell Science, pp 172.
The AVID Investigators. N Engl J Med. 1997;337:1576-1583.
Myerburg RJ. Ann Intern Med.. 1993;119:1187-1197.
Demirovic J. Progr Cardiovasc Dis. 1994;37:39-48.
Friedlander Y. Circulation. 1998;97:155-160.
 Prior Episode of VT
• VT with syncope or a low ejection
fraction (LVEF < 40%) leads to an
increased risk of Sudden Cardiac Arrest

• The one-year risk of Sudden Cardiac

Arrest for these patients ranges from 20
to 50%

Myerburg RJ. Heart Disease, A Textbook of Cardiovascular Medicine, 5th ed, Vol 1.
Philadelphia: WB Saunders Co;1997:ch 24.
Fogoros RN. Practical Cardiac Diagnosis: Electrophysiologic Testing, 2nd ed. Blackwell Science, pp
172.
The AVID Investigators. N Engl J Med. 1997;337:1576-1583.
SCA Relationship to MI
• A previous MI can be identified in as
many as 75% of SCA patients.
• A previous MI as a single risk-factor
raises the one-year risk of SCA by 5%.
• The five-year risk of SCA is 32% for
patients with all of these risk-factors:
• history of MI
• non-sustained, inducible, non-suppressible
VT
• LVEF ≤ 40%

Sudden Cardiac Arrest Fast Facts. HRS. www.hrsonline.org

Risk factors for sudden cardiac death. www.heartinstitute.org.au/Community/scdMain.asp
Buxton AE, et al. N Engl J Med. 1999;341:1882-1890.
 Time Dependence of Mortality Risk Post-MI
Prediction of Sudden Cardiac Death After Myocardial Infarction
in the Beta-Blocking Era1

Total

1
Mortality
18
• 700 post-MI patients;
8 ~ 95% on beta-blockers
1 15
2 years after discharge.

Cumulative Events (%)

5 • The epidemiologic
1 Cardiac 12 pattern of SCD was
2 Mortality different from that
9 9 reported in previous
Non-SCD
studies.
6 6
SCD
–Arrhythmia events did
3 3 not concentrate early
after the index event;
most occurred > 18
months post-MI.
2 4 60 2 4 60
0 0 0 0
Follow-Up (months) Follow-Up (months)

1
Huikuri HV. J Am Coll Cardiol. 2003;42:652-658.
Relation of Time from MI to ICD Benefit
in the MADIT-II Trial
Conv
16 ICD
14
14
12 11.6

10 9
7.8 8.4 8.2
8 7.2
% Mortality for Each

6 4.9
4
2
0
Time Period

1-17 mo 18 - 59 mo 60 - 119 mo > 120 mo

Time from MI
(n = (n = (n = (n =
Hazard 300)
.98 283)
0.52 284)
0.50 292)
0.62
Ratio (p = 0.92) (p = 0.07) (p = 0.02) (p = 0.09)

Wilber, D. Circulation. 2004;109:1082-1084.

 SCD Rates in Post-MI Patients
with LV Dysfunction
Total Mortality
30 28 28 Arrhythmic Mortality
Control Group Mortality at 2years

21
20 19.8
20 18
16 16
14
12
10 9.4
10 7

0
TRACE CAPRICORN EMIAT MADIT MUSTT MUSTT MADIT II*
Inducible Registry

Total Mortality ~20-30%; SCD

accounts for ~50% of the total deaths.
 “People who’ve had a heart attack
have a sudden death rate that is 4-6
times that of the general population.”1

“
People who have had a heart attack
and have LV dysfunction (</=40%) have
a sudden death rate that’s similar to a
CHF population.”
American Heart Association. Heart Disease and Stroke Statistics—2003
1

Update. Dallas, Tex.: American Heart Association; 2002.

SCD in Heart Failure 1, 2

• Despite improvements in
medical therapy, symptomatic
HF still confers a 20-25% risk
of premature death in the first
2.5 yrs after diagnosis.
– ≈ 50% of these premature
deaths are SCD (VT/VF)
1
Bardy G. The Sudden Cardiac Deatth-Heart Failure Trial
(SCD-HeFT) in Woosley RL, Singh S, Arrhythmia
Treatment and Therapy, Copyright 2000 by Marcel
Dekker, Inc. , pp. 323-342,
2
Sweeney MO PACE 2001;24:871-888.
 SCA Relationship to HF
• Patients with HF are overall at 6-9 times higher
risk for SCD than general population

• As HF progresses, pump failure (rather than SCA)

becomes relatively more likely as the cause of
death

• Reduced LVEF remains the single most important

risk factor for overall mortality and sudden
cardiac death.

Heart Disease and Stroke Statistics – 2005 Update. AHA. www. americanheart.org
 Severity of Heart Failure
Modes of Death

NYHA II NYHA III

CHF
12% CHF
Other 26% Other
24% Sudden 59%
64% Death 15% Sudden
(N = 103) Death
(N = 103)

NYHA IV SCA Pump Failure

CHF
33% Other NYHA Class II 64% 12%
56%
Sudden NYHA Class III 59% 26%
11% Death
(N = 27) NYHA Class IV 33% 56%
 LVEF and SCA Incidence
8
7.5%
7
6
% SCA Victims

5
4 5.1%
3
2
1 2.8%
0
0-30% 31-40% 41-50% >50% 1.4%

LVEF
Vreede-Swagemakers JJ. J Am Coll Cardiol. 1997;30:1500-1505.
 SCD

• Genetic Basis
– Short QT syndrome
– Long QT Syndrome
– Brugada Syndrome
– Hypertrophic Cardiomyopathy
– Arrhythmogenic Right Ventricular Cardiomyopathy
– Catecholaminergic Polymorphic Ventricular
Tachycardia
• Family history is a strong independent predictor of
susceptibility to SCD
 Long QT Syndrome
• Congenital disorder that may lead to
unexplained syncope, seizures, and SCA
• Either asymptomatic or are prone to
symptomatic and potentially lethal arrhythmias
• 60% have a positive family history of LQTS or
SCA
• Necessary to identify other family members at
risk

Schwartz PJ. Curr Probl Cardiol. 1997;22:297-351.

Smith WM. Ann Intern Med. 1980;93:578-584.
Garson A Jr. Circulation. 1993;87:1866-1872.
Long QT Syndrome in a 16-year-old girl
Hypertrophic Cardiomyopathy
• SCD is the most common cause of death
• Prevalence is about 0.2% of the general population
and about 10% of HCM patients are considered to
be at high risk of SCA
• Over a ten year period > 50% of high-risk patients
would experience SCA
• Most common cause of SCA in athletes under age
35

Myerburg RJ. Heart Disease, A Textbook of Cardiovascular Medicine. 5th ed, Vol 1.
Philadelphia: WB Saunders Co; 1997:ch 24.
Maron BJ. New Engl J Med. 2000;342:365-373.
Arrhythmogenic RV
Dysplasia
• RV myocardium is
progressively replaced
by fibrofatty tissue. LV is
normal
• LBBB morphology
• High risk of SCD if
arrhythmias are left
uncontrolled
• Risk stratification is
important
• Tx: Drugs, ICD, ablation
• Prevent arrhythmic
death influenced by
autonomic tone

Zipes and Jalife, 2001

Arrhythmogenic RV Cardiomyopathy
 Brugada Syndrome
• ECG pattern of ST segment
elevation in V1 to V3 with
RBBB morphology and
syncopal or sudden death
episodes in patients with
structurally normal hearts
• Male predominance
Brugada Syndrome
 Brugada Syndrome
• Incidence
– Ubiquitous
– Cause of 4-12% of all sudden deaths
• Prevalence ?
• Pharmacologic maneuvers with Na
blockers (Ajmaline, Flecainide,
Procainamide) may unmask abnormal
ECG
 Brugada Syndrome
• Etiology and Genetics
• SCN5A mutation
–Encodes Na channel
• Autosomal dominant pattern
• Males more prone to
phenotype (typical ECG and
ventricular arrhythmias)
 Brugada Syndrome
• Na channel blockers worsen
ECG findings
• Isoproterenol controls
electrical storm
• Prognosis and Treatment:
– Poor if left untreated
– ICD: Treatment of choice
 SUDS

• 1997 Dr. Koonlawee

Nademanee
• Thailand: “Lai Tai”
• Japan: “Pokkuri”
• Philippines: “Bangungut”
• “Dying in their sleep”
 SUDS
• Clinical Presentation:
– Agonal respiration
– Unresponsiveness after
labored respiration during
sleep or seizure-like
symptoms
– Normal cardiac function
 SUDS & Brugada
• No organic heart disease
• Males exclusively
• High incidence of inducible
polymorphic VT to VF
• High mortality rate
• Normalization of ECG on exercise
• EP mechanism same ??
 Treatment Options for SCA

• Defibrillation is the
only effective
treatment for SCA
• VF tends to rapidly
deteriorate into
asystole
• Asystole cannot be
successfully treated
with defibrillation
 Implantable Cardioverter Defibrillator
First-line therapy for patients at risk for SCA
• Small devices, pectoral
implant site
• Transvenous, single incision
• Local anesthesia; conscious sedation
• Short hospital stays
• Few complications
• Perioperative mortality < 1%
• Programmable therapy options
• Single- or dual-chamber therapy
• Battery longevity up to 9 years
• 80,000 implants/year (2000 E)1

1
Morgan Stanley Dean Witter. Investors Guide to ICDs. 2000.
 Key Randomized Clinical
Trials
ICD therapy for the secondary prevention of SCA

Mortality
(%)

Trial N Age (yrs) Mean LVEF Follow-up Control Control ICD P

(%) (mos) Therapy

AVID 1016 65 ± 10 35 18 ± 12 Amiodarone or 24.0 15.8 .02

sotalol

CIDS 659 64 ± 9 34 36 Amiodarone 29.6 25.3 .14

CASH 288 58 ± 11 45 57 ± 34 Amiodarone or 44.4 36.4 .08

metoprolol

Adapted from: DiMarco JP. N Engl J Med. 2003;349:1836-47. www.medscape.com

Young JB. Sudden cardiac death in heart failure. www.medscape.com
 2006 ACC/AHA/ESC Guidelines for the
Management of Ventricular Arrhythmias:
Secondary Prevention of SCD

ICD Class I Recommendation:

• Patients with a history of SCA, VF,
hemodynamically unstable VT, or unexplained
syncope

Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484

Patients with a previous cardiac arrest are at high risk for subsequent SCA
events but account for a small percentage of annual sudden deaths

SCD-HeFT,
MADIT II

AVID, CASH,
CIDS
MADIT I, MUSTT

Myerburg RJ, et al. Circulation. 1998. 97:1514-1521.

 Key Randomized Clinical Trials

ICD therapy for the primary prevention of SCA

Mortality (%)
Trial N Age Mean Follow-up Control Control ICD P
(yrs) LVEF (%) (mos) Therapy

SCD-HeFT 2521 60.1 25 45.5 Optimal 36.1 28.9 .007

Medical
Therapy
MADIT 196 63 ± 9 26 27 Conventional 38.6 15.7 .009

MADIT II 1232 64 ± 10 23 20 Optimal 19.8 14.2 .007

Medical
Therapy
MUSTT 704 67 ± 12 30 39 No EP-guided 48 24 .06
therapy

DEFINITE 458 58 21 29.0±14.4 Optimal 14.1 7.9 .08

Medical
Adapted from: DiMarco JP. N Engl J Med. 2003;349:1836-47. www.medscape.com
Therapy
Kadish A, et.al. N Engl J Med 2004;350:2151-8.
Young JB. Sudden cardiac death in heart failure. www.medscape.com
Heart Failure and Left Ventricular Dysfunction are
indicators of SCA risk

SCD-HeFT,
MADIT II
AVID, CASH,
CIDS
MADIT I, MUSTT

Myerburg RJ, et al. Circulation. 1998. 97:1514-1521.

 2006 ACC/AHA/ESC Guidelines for the
Management of Ventricular Arrhythmias: Primary
Prevention of SCD

ICD Class I Recommendations:

• Patients with ischemic cardiomyopathy who are at least 40
days post-MI with an LVEF ≤ 30 - 40% and NYHA functional
class II or III
• Patients with NYHA Class II-III, LVEF ≤ 30 - 35%, non-ischemic
cardiomyopathy
• Patients who are at high risk of SCA due to genetic disorders
such as long QT syndrome, Brugada syndrome, hypertrophic
cardiomyopathy and arrhythmogenic right ventricular dysplasia
(ARVD).

Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484

 2006 ACC/AHA/ESC Guidelines for the
Management of Ventricular Arrhythmias: Primary
Prevention of SCD

ICD Class II Recommendation:

• Ischemic and non-ischemic patients with NYHA functional
class I, LVEF ≤ 30-35%

Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484

Many methods to further risk stratify patients a
risk for SCA have been studied...
Test Objective Sensitivity Specificity Limitations
(%) (%)
But a reduced EF
remains the single
Echo Measurement of 55–65 75–80 most important risk
LVEF factor for overall
mortality and sudden
HR Assessment of low 38–62 75–88 Multiple non- cardiac death.
variability heart rate variability standardized methods
EP Study Induction of VA’s 48–73 65–93 Invasive, expensive
Signal Induction of late 56-68 74–81 Not useful in non-
Averaged potentials ischemic cardiomyopathy
ECG
(SAECG)
Microvolt Identification of 77–93 37–83 Cannot be used in AF
T-Wave repolarization
Alternans abnormalities
(MTWA)
Siddiqui A, Kowey PR. Curr Opin Cardiol. 2006;21:517-25.
Prior SG, et al. Eur Heart J, Vol 22:16:August 2001
 Evaluating Patients at Risk for SCA
• Electrophysiology Studies (EPS) have been helpful
in the diagnosis of cardiac arrhythmias including:
– Sinus and AV node dysfunction
– Conduction abnormalities
– Accessory pathways of conduction
– Inducibility of VT

• EPs can provide advanced treatments including

Implantable Cardioverter Defibrillators (ICDs) and
ablation therapy
 Conclusions
• The key to SCD prevention is to identify
high risk patients BEFORE they have a
SCA event. The majority of cases are in
patients with:

– Coronary artery disease, previous MI

– Low left ventricular ejection fraction
– Dilated cardiomyopathy and heart failure
INTERACTIVE SESSION
Which is NOT a recognized risk factor
for SCD?

a. Short QT interval
b. Brugada Syndrome
c. Hypertrophic Cardiomyopathy
d. Family History of SCD
The following are the common
clinical presentation of SUDS
except:

a. agonal respiration
b. unresponsiveness after
labored respiration during
sleep or seizure-like
symptoms
c. Depressed cardiac function
Thank You