Transcript 돌연사

Sudden Cardiac Death
Yong Hoon Kim, M.D.,Ph.D.
Division of Cardiology, Department of Internal Medicine,
School of Medicine,
Kangwon National University
돌연사

급사

급성심장사

젊은 야구선수 안타치고 2루 밟고 의식 소실 뒤
심장마비

북한 김일성 심장마비 사망

젊은 수련의가 숙소에 잠깐 쉬러 간 뒤 숨진 상
태로 발견

감기 한번 안 걸린 원로교수가 연구실 의자에서
흉통 호소한 뒤 사망
Definition of SCD

The unexpected natural death from a cardiac cause
heralded by abrupt loss of consciousness
within a short time period, generally less than 1 hour from
the onset of symptoms.

Preexisting heart disease may or may not have been
known to be present, but the time and mode of death are
unexpected.
Pinto DS et al. The Heart. 2001:1015-1048
Definition of SCD

Any cardiac death occuring out of the hospital or taking
place in the emergency room or dead on arrival in the
emergency room.
Gillum RF et al. Circulation 1989;79:756-765
Time References in SCD
A Prodromes
B Onset of
terminal event
C Cardiac
arrest
D Biological
death
New or worsening
Abrupt change in
Sudden
Failure of
cardiovascular
clinical status:
Collapse
resuscitation
-------------------
Or
Arrhythmias
Loss of
Failure of
Chest pain
Hypotension
effective
electrical,
Palpitations
Chest pain
circulation
mechanical, or
Dyspnea
Dyspnea
Weakness
Light headedness
symptoms
Days to months
CNS function
Loss of
after initial
consciousness
resuscitation
Instantaneous to 1h
Minutes to weeks
경과

1 단계 (전구기)
- 수일 또는 수개월전부터 흉통, 호흡곤란, 심계항진, 피로감
- 25%에서는 아무 증상이 없다가 첫 증상으로 돌연사발생
2 단계
- 급성증상의 시작으로 돌연사가 발생하기 직전이나 1시간
이내에 부정맥, 저혈압, 흉통, 호흡곤란의 증상 발생
3 단계
- 돌연사의 원인인 부정맥의 발생으로 심기능은 정지, 의식은
소실되나 즉각적인 치료로 소생이 가능
4 단계
- 생물학적 사망으로 모든 생체기능이 비가역적으로 중지
Epidemiology
 In the World, 3,000,000 SCD/year
 Survival rate < 1%
 In USA, 250,000 to 400,000 SCD/year
 Survival rate 5%
 Unfortunately,
SCD is first presentation of cardiac disease in 33%~50% of
patients
Mark Josphson et al. Circulation.2004;109:2685-2691
Magnitude of SCA in the U.S
500,000
# deaths/year
400,000
300,000
200,000
100,000
0
AIDS
Breast
Cancer
Lung
Cancer
Stroke
SCA
Mark Josphson et al. Circulation.2004;109:2685-2691
Epidemiology
In Korea, > 20,000 SCD/year
→ > 41/100,000 (2005~2006)
돌연사의 약 3/4이 집에서 발생
돌연사의 약 2/3 가 목격자가 있다.
The Incidence of SCD in Specific Populations
in
U.S.A.
Group
0.1~0.2%/year
General population
300,000/year
Pts with high coronaryrisk profile
Pts with previous
coronary event
Pts with ejection
fraction<35%,
congestive heart failure
Pts with previous out-ofhospital cardiac arrest
Pts with previous
myocardial
infarction,low ejection
fraction, and ventricular
tachycardia
0
5 10 15 20 25 30 35 0
Incidence of Sudden Death
(% of group)
100000
200000
No. of Sudden Deaths
per Year
300000
The Incidence of SCD

Age
: two ages of peak incidence
1) birth ~ 6 months of age
(the sudden infant death syndrome)
2) 45 ~ 75 years of age

Heredity : congenital long QT interval syndromes
hypertrophic cardiomyopathy
right ventricular dysplasia
Brugada syndrome
The Incidence of SCD

Gender : huge preponderance in males compared with females
because of the protection females before menopause
M: 3.8-fold excess incidence of sudden coronary death
Kannel WB et al. The Franingham study. 1982
Mechanisms of SCD
ANATOMIC
/FUNCTIONAL
SUBSTRATE
CAD
Cardiomyopathy
Right ventricular dysplasia
Valvular
Congenital
Primary
electrophysiological
Neurohormonal
Asystole
Developmental
VT
Inflammatory
Infiltrative
VF
Reentry
Automaticity
Triggered activity
Block
Cell-to-cell uncoupling
ARRHYTHMIA MECHANISMS
Neuro/endocrine
Drugs
Electrolytes, pH, pO2
Ischemic/reperfusion
Hemodynamic
Stretch
Arising/Stress/Sleep
TRANSIENT
INITIATING EVENTS
Venn Diagram showing
Interaction of
Various factors
Causes of SCD
Acute Mechanical
Causes : 5%
Coronary Artery
Disease : 75~80%
Non-structural Heart
Disease : 5~10%
Structural Heart
Disease : 10~15%
VHD 5% Normal 3%
Other
DCM 10%
Spasm Long QT
CAD 80%
Normal 9%
CAD 42%
VHD
Men
Other 22%
Women
DCM 18%
HCM 26%
CAD 3%
Coronary anomaly
14%
Commtion cordis
20%
Young Athletes
Progression of atheromatous
plaque
Coronary Artery Disease -1

Coronary atherosclerosis
Acute myocardiacl infarction
Chronic ischemic heart disease with transient supply-demand imbalnace
– thrombosis, spasm. physical stress

Congenital abnormailties of coronary arteries
Anomalous origin from pulmonary arteries
Other coronary arteriovenous fistular
Origin of right coronary artery from left sinus of Valsalva
Hypoplastic or aplastic coronary arteries
Coronary-intracardiac shunt
Coronary Artery Disease -2

Coronary artery embolism

Coronary artetitis
PAN, progressive systemic sclerosis, giant cell arteritis
Mucocutaneous lymph node syndrome (Kawasaki disease)
Syphilitic coronary ostial stenosis

Micellaneous mechanical obstruction of coronary a.
Coronary artery dissection

Functional obstruction of coronary arteries
Coronary artery spasm
Myocardial bridge
Myocardial disease and heart failure

Chronic congestive heart failure
Ischemic cardiomyopathy
Idiopathic congestive cardiomyopathy
Alcoholic cardiomyopathy
Hypertensive cardiomyopathy
Post-myocarditis cardiomyopathy
Postpartum cardiomyopathy

Acute cardiac failure
Massive acute myocardial infarction
Acute myocarditis
Inflammatory, Infiltrative, Neoplastic and
Degenerative process
 Viral myocarditis
 Sarcoidosis
 Progressive systemic sclerosis
 Amyloidosis
 Hemochromatosis
 Idiopathic giant cell myocarditis
 Chaga’s disease
 Cardiac gangilonitis
 Arrhythmogenic right ventricular dysplasia (ARVD)
 Neuromuscular disease
Non-structural Heart Disease

Long QT syndrome
Congenital long QT syndrome
Acquired long QT syndrome
: Drug effect, Electolyte abnormality, Toxic Substance
Hypothemia, CNS injury

Brugada syndrome

Sudden unexplained death syndrome of Southeast Asia (SUDS)

Familial sudden death syndrome

Primary ventricular fibrillation

WPW syndrome (rapid antidromic conduction)

Severe electrolyte abnormalities
Acute Mechanical Causes

Aortic rupture

Ventricular rupture

Commotio cordis – blunt chest trauma
• Fatal cardiac arrest without detectable structural damage to the heart
as a result of blunt impact to chest
• various mechanism : V-fib etc.
Specific dieases and SCD
Ventricular Hypertrophy

Independent risk factor for SCD
 contributing to mechanisms of potentially lethal arrhythmias
Anderson KP. J Cardiovasc Pharmacol,1984

Underlying conditions
- Hypertensive heart disease
- HCMP with or without obstruction
- Primary pulmonary HTN with RV hypertrophy
- RV overload d/t congenital heart disease
Transverse section through
Ventricles
Normal Cardiac Crosssection
Hypertrophied
Cardiac Cross-section
Hypertrophic CMP

Prevalence :
- 0.2% in young adult
2-4% SCD in adult & 4-6% in adolescent
- SCD : 67% of 48 deaths (n=254,6YF/U) Br Heart J,1982;48:1
55% of 49 deaths (n=190) Circ Res,1974;(S2)35:179
- SCD as 1st clinical manifestation

Gene mutation
- the genes that encode the cardiac muscle sarcomere
( -tropomyosin & -myosin heavy chain mutation )

Mechanism : Unclear - arrhythmia, ischemia
- abrupt hemodynamic change
Risk factors for SCD in Patients with HCMP

Major risk factors
Cardiac arrest (ventricular fibrillation)
Spontaneous sustained ventricular tachycardia
Family history of sudden death
( sudden death from hyperthrophic cardiomyopathy in two or
more first-degree relatives younger than 40 years of age )
Rick A. Nishimura et al. NEJM 2004;350:1320-7

Minor risk factors
Unexplained syncope
(two or more episodes of syncope within one year)
Left ventricular wall thickness > 30 mm
Abnormal blood pressure on exercise
(rise more than 25mmHg from baseline or decrease of more
than 10mmHg from maximal pressure during exercise)
Nonsustained ventricular tachycardia
Left ventricular outflow obstruction
Microvascular obstruction (on nuclear imaging or MRI )
High-risk genetic defect
Rick A. Nishimura et al. NEJM 2004;350:1320-7
A high risk ! (HCMP)
( warranting prophylactic implantation of an
automatic defibrillator)
≥ 1 major risk factors
or
≥ 3 minor risk factors.
Rick A. Nishimura et al. NEJM 2004;350:1320-7
Valvular Heart Disease

Aortic Stenosis :
- one of common causes of SCD before valve surgery era
- after AVR : still at risk for SCD
 arrhythmias, valve dysfunction, CAD
- 21% of 298 post-op deaths
(peak : 3rd week, plateau : after 8 months )
Circulation 1985;72:753
Dissected Heart
showing
Valve Openings
Rheumatic
Congenital Heart Disease

SCD by arrhythmia
: 4 congenital conditions
- Tetralogy of Fallot
- Transposition of the great arteries
- Aortic stenosis
- Pulmonic obstruction
Zipes DP et al. Circulation. 1998;98:2334-2351
SCD without structural heart disease
- solely “electrical” basis 
Wolff-Parkinson-White syndrome

Long QT syndrome

The Brugada syndrome

Short-coupled torsade de pointes

Cathecholamine- induced polymorphic ventricular
tachyarrythmia

Idiopathic VF
Eric F.D. Wever et al. JACC. 2004;43:1137-1144.
Strategies for Prevention of SCD

Effective treatment in patients at high risk with
previously documented episodes of SCD

Identification of high-risk populations before an
episode of SCD
돌연사의 위험인자
돌연사에서의 소생, 심실빈맥의 병력, 좌심실
기능부전, 심실조기수축, 돌연사의 가족력
 남자
 비만
 흡연
 고혈압
 당뇨병
 스트레스
 과음

Cardiac Arrest Survivor Evaluation
1. History & physical examination
- Precipitating events
- Associated conditions
- Therapeutic and/or illicit drug usage
- Family history
2. ECG & laboratory tests
- Rhythm and/or conduction abnormalities
- Ischemia, infarction hypertrophy
- Electrolyte or metabolic abnormalities
Cardiac Arrest Survivor Evaluation
3. ECG monitoring
4. Echocardiography
5. Stress testing
for provocation of ischemia or arrhythmia
6. Cardiac catherization
7. Electrophysiologic study
ECG Patterns Predicting SCD
 Congenital long QT
 Brugada syndrome : RBBB & ST elevation in V1-V3
 RV dysplasia : negative T in right precordial leads
& epsilon wave at the end of QRS
 Large infarction pattern
 DCMP pattern : low voltage in the limb leads with
normal voltage or LBBB in precordial leads
 HCMP : LBBB or high QRS voltage
& prominent Q waves in lateral leads
or giant negative T waves in precordial leads
치료
응급치료를 시행하지 않으면 3-5분내 뇌장애,
곧 사망에 이른다.
 돌연사에서 소생한 환자를 적절히 장기치료하지
않으면 1년 내에 약 30%, 2년 내 45%가 재발.

기초구명법
호흡, 맥박, 피부색 관찰하여 돌연사 여부 확인
 의심되거나 확인되면 119,129 전화
 심장압박으로 심폐소생술실시

고등구명법

돌연사로부터 소생 후 병원으로 옮겨 호흡을 적
적히, 부정맥 조절, 혈역학상태(혈압, 심박출량)
를 안정시키고, 신체 장기의 혈액순환을 회복.
소생 후 장기치료

돌연사의 원인이 급성 심근허혈인지 부정맥인지
구분한다.

급성심근허혈 : 항허혈제, 관상동맥성형술, 관상
동맥우회로술

부정맥 : 항부정맥제, 삽입형 제세동기, 전극도
자절제술, 수술
Before & After PTCA with Stenting
예후

심폐소생술의 신속성, 원인 부정맥의 종류, 원인
질환의 종류에 따라 다르다.

신속한 심폐소생술, 신속한 제세동, 신속한 고등
구명법을 하면 호전시킬 수 있다.

일반인에 대한 심폐소생술 교육에 매우 중요
예후

원인 부정맥에 따른 예후 (살아서 퇴원할 가능성)
- 심실빈맥 : 67%
- 심실세동 : 25%
- 무수축 : 5%↓
급성 심장병 – 양호
 만성 심장병 – 나쁨

예방

동맥경화를 예방 : 고혈압, 당뇨, 고지혈증, 비만,
흡연 조절

관상동맥질환, 심근증, 심장판막증과 같은 구조적
질환, 심실빈맥과 같은 심실성 부정맥 → 심장내과
진료를 요함

돌연사가 발생하기 수시간 또는 수일전 가슴통증,
호흡곤란, 두근거림, 현기증, 피로감 → 심장내과
진료를 요함
Atrial
Ventricle