Download Project SAVE: Sudden Cardiac Death

Pediatric
Sudden Cardiac Death
Robert M. Campbell, MD
CMO, Children’s Healthcare of Atlanta
Sibley Heart Center
Director, Sibley Heart Center Cardiology
Division Director of Cardiology,
Department of Pediatrics,
Emory University School of Medicine
The Atlanta Journal Constitution
Sunday, September 7, 2003
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The Atlanta Journal Constitution
Sunday, September 7, 2003
‘There was this beautiful young
lady laying there,
and I kept thinking,
“This can’t be happening.
Her heart can’t be stopping.”
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Sudden Cardiac Death (SCD)
Overview
 Infrequent occurrence?
• ? 1:50K-1:200K athletes
• No accurate or mandatory reporting
 Caused by rare cardiac defects, trauma, or
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stimulants
+ Warning signs/symptoms
When SCD occurs, stories are big
• Emotional responses from parents, coaches,
friends, and the community
In this day and age, children are pushing and
getting pushed harder
SCD episodes may not be predictable or
preventable
Sudden Cardiac Death (SCD):
Differential Diagnosis
Structural/Functional
1) Hypertrophic Cardiomyopathy
(HCM)*
2) Coronary Artery Anomalies
3)
Aortic Rupture/Marfan*
4)
Dilated Cardiomyopathy*
Myocarditis
Left Ventricular Outflow Tract
Obstruction
Mitral Valve Prolapse (MVP)
Coronary Artery
Atherosclerotic Disease*
Arrhythmogenic Right
Ventricular Cardiomyopathy
(ARVC)*
5)
6)
7)
8)
9)
Electrical
10) Long QT Syndrome (LQTS)*
11) Wolff-Parkinson-White
Syndrome (WPW)
12) Brugada Syndrome*
13) Catecholaminergic Ventricular
Tachycardia*
14) Short QT Syndrome
15) Post-operative Congential Heart
Disease
Other
16) Drugs and Stimulants
17) Primary Pulmonary
Hypertension*
18) Commotio Cordis
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*Genetic/Familial
*
Normal Echocardiogram
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SCD Differential Diagnosis:
Structural/Functional
1) Hypertrophic Cardiomyopathy:
Thickening of the heart muscle
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SCD Differential Diagnosis:
Structural/Functional
2) Coronary Artery Anomalies:
Congenital or Acquired
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SCD Differential Diagnosis:
Structural/Functional
3) Aortic Rupture/Marfan:
Dilatation and thinning of the aorta
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SCD Differential Diagnosis:
Structural/Functional
4) Dilated Cardiomyopathy:
Thinning and weakening of the heart muscle
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SCD Differential Diagnosis:
Structural/Functional
5) Myocarditis: Inflammation of the heart muscle
6) Left Ventricular Outflow Tract Obstruction:
Blockage to the left ventricular outflow
7) Mitral Valve Prolapse (MVP): Redundancy of
mitral valve
8) Coronary Artery Atherosclerotic Disease:
Coronary artery plaque and obstruction
9) Arrhythmogenic Right Ventricular
Cardiomyopathy (ARVC): Fatty infiltration of the
right ventricular muscle
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SCD Differential Diagnosis:
Primary Electrical
10) Long QT Syndrome (LQTS): Abnormal electrical
reactivation (repolarization)
11) Wolff-Parkinson-White Syndrome (WPW):
Accessory pathway connecting the upper to lower
heart chambers
12) Brugada Syndrome: Ventricular fibrillation 3rd or
4th decades; rare in children
13) Catecholaminergic Ventricular Tachycardia:
Exercise induced tachycardia
14) Short QT Syndrome: Abnormal electrical
reactivation (repolarization)
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SCD Differential Diagnosis
Primary Electrical:
15) Post Operative Congenital Heart Disease:
 TGA Senning/Mustard
 Fontan repair
 LV outflow obstruction
 Others
Other:
16) Stimulants: Ephedra, cocaine, etc.
17) Primary Pulmonary Hypertension (PPH):
Elevated blood pressure in lung arteries
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SCD Differential Diagnosis:
Other
18) Commotio Cordis: Blunt blow to the chest
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SCD Profiles
CAD 2%
MVP 2%
ARVD 3%
Other 6%
Dilated C-M 3%
HCM
36%
Myocarditis 3%
AS 4%
Tunneled LAD
5%
Ruptured Ao
5%
Cardiac Mass
10%
Coronary
anomalies
19%
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Maron BJ, et al. JAMA. 1996;276:199-204.
SCD Profiles (cont.)
Maron. JAMA 1996.
CAD 2%
MVP 2%
ARVD 3%
Other 6%
Dilated C-M 3%
HCM
36%
Myocarditis 3%
AS 4%
Tunneled LAD
5%
Ruptured Ao
5%
Cardiac Mass
10%
Coronary
anomalies
19%
 Italian Experience:
• ARVC leading cause of SCD
• HCM, coronary artery anomalies
less common
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Corrado. J AM Coll Cardiol 2003.
SCD Profiles (cont.)
CAD 2%
MVP 2%
ARVD 3%

Maron. JAMA 1996.
Other 6%
Dilated C-M 3%
HCM
36%
Myocarditis 3%
AS 4%
Tunneled LAD
5%
Ruptured Ao
5%
Cardiac Mass
10%
Coronary
anomalies
19%

Italian Experience: Corrado. J AM Coll Cardiol 2003.

Nontraumatic Sudden Death During
Military Basic Training (Escart. JACC 2004)
A. N=126; 83% exercise-related
B. 64/126 Cardiac
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•
39/64 Coronary Artery Anomalies (all LCA from right sinus of
Valsalva)
•
13/64 Myocarditis
•
8/64 HCM/LVH
Other Causes of Athletic “Collapse”
 Heat Stress/Stroke
 Vasovagal Faint
(Neurocardiogenic Syncope)
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Neurocardiogenic Syncope (NCS)
Upright Position
Blood Pooling in Lower Body
Filling of Heart
Heart Contractility
(Nervous System)
Paradoxical
Slow Heart Rate
and/or
Blood Pressure
 Prodrome (warning signs)
 Syncope (loss of consciousness) short duration
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 Occurs at the end of exercise, after exercising has stopped
PPE: Does It Work?
 Appropriately restrict; appropriately clear
 Be thorough and conscientious
 Are there any warning signs?
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Diagnosis:
Pre-Participation Evaluation (PPE)
Awareness of Warning Signs
1) Patient History
a) Fainting (syncope) or seizure during exercise,
excitement or startle
b) Consistent or unusual chest pain and/or
shortness of breath during exercise
c) Past detection of a heart murmur or increased
systemic blood pressure
d) Prescription, OTC, and other
“medications/supplements”
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Diagnosis:
Pre-Participation Evaluation (PPE)
Awareness of Warning Signs
2) Family History
a) Premature death or significant disability from
cardiovascular disease in close relatives younger
than 50 years of age
b) Syncope, seizures, SIDS, accidental death,
congenital deafness
c) Specific knowledge of the occurrence of certain
conditions:
 HCM, DCM, Marfan’s, LQTS, clinically important
arrhythmias, pacemaker implantation, early
onset coronary artery disease, ARVC, PPH,
Brugada
3) Physical Exam
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Project SAVE PPE Objectives
 Support use of standarized PPE Form
 Identify patients/families at higher risk for SCD
based upon PPE Form response
 Increase general awareness of SCD warning signs
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Familial Disease:
Impact of Proband Identification
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Role of Routine EKG and/or
Echo Screen
 Athletes only?
• ~ 8 million young athletes in US
(Maron, NEJM, Sept. 2003)
• Any child potentially at risk although exercise
increases risk
• 6th vs 9th vs 12th grade?
• School athletics only?
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Role of Routine EKG and/or
Echo Screen (cont.)
 What age for screen?
• 50% LQTS patients who die succumb before
9th grade
• HCM may have a pre-hypertrophic phase
 For example:
– Normal echo at age 10, but…
– Abnormal echo at age 20
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Role of Routine EKG and/or
Echo Screen (cont.)
 Screen for what diagnoses?
• HCM only?
OR
• Comprehensive echo and EKG screening for any
cause
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Role of Routine EKG and/or
Echo Screen: Summary
 Unfavorable cost: benefit ratio
 False positives and false negatives
 Negative screen does not exclude disease
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Project SAVE PPE Recommendation
Comprehensive medical evaluation
if positive PPE or signs/symptoms
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Secondary Prevention: Resuscitation
 What can be done to treat children and
adolescents who suffer sudden cardiac death and
ventricular fibrillation, despite primary prevention
efforts?
• Rapid CPR
• Early Defibrillation
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Automated External Defibrillator
(AED)
What is an AED?
 A device that looks for
shockable heart rhythms and
delivers a defibrillator shock,
if needed.
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It is small, portable,
automatic, and simple to
operate.
Are School AED’s the
“Right Thing To Do”?
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Key Elements of a School AED
Program
 Assign a project coordinator
 Champion the idea and raise awareness
 Review laws and regulations and consult your legal counsel
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or risk manager
Coordinate with local EMS
Arrange for medical direction
Identify your response team
Choose your equipment and vendor
Design policies and procedures
Assess how many AEDs you’ll need and where they’ll do the most good
Estimate costs for equipment , training and PR
Fund your budget
Train responders and plan for refresher training
Acquire and deploy AEDs and other supplies
Promote your program to raise awareness and support
Build quality assurance into your operation
Medtronic
Summary
Project SAVE:
Children’s Healthcare of Atlanta
SCD Program
I.
Differential Diagnosis
and Scope of SCD
Problem
1)
Sudden Cardiac Death: causescommon arrhythmia incidence
2) Sudden Arrhythmia Death Syndromes
Foundation Warning Signs
II.
Diagnosis and
Primary Prevention
3)
4)
5)
6)
7)
8)
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Symptomatic vs. Asymptomatic
Patients
Vital Signs, Family History
Impact of proband identification with
subsequent family screen
Pre-Participation Evaluation Form;
appropriate restriction or clearance
Universal awareness of warning signs
Medical referral based on focused
history and/or symptoms
Summary
Project SAVE:
Children’s Healthcare of Atlanta
SCD Program
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CPR: ABC’s
10) 911
11) Defibrillation; AED
program implementation
III.
Secondary Prevention
9)
IV.
Resources and
Associated Issues
12) Promote CPR/AED training for staff
and students
13) Promote consultation and
educational materials for schools
14) Coordination of research/registry of
SCD events
Summary
Project SAVE
 S: Sudden Cardiac Death
 A: Awareness
 Warning signs
 Resources
 V: Vision for Prevention
  SCD
  Collaboration
 E: Education for the School Community
 Pre-Participation Evaluation process
 AED
 CPR
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Project SAVE Recommendations
 Universal awareness of warning signs
 Conscientious use of PPE Form and process
 Comprehensive screen of high risk patients and
families
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Sudden Cardiac Arrest in the Young Coalition:
Goals/Objectives
Goal: No Deaths
Objectives:
1) All children screened with family history questions
2) All MD’s (primary care) knowledgeable about further
screening
3) Family health history document for every family
4) All school and community sports coaches and staff are
knowledgeable about the warning signs of SCD and the
importance of a timely emergency response
5) CPR training is encouraged for both school staff and
students
6) Community and school PAD initiatives are supported
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