Pediatric Sudden Cardiac Death
Robert M. Campbell, MDCMO, 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
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The Atlanta Journal ConstitutionSunday, 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 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
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Sudden Cardiac Death (SCD): Differential Diagnosis
Structural/Functional
1) Hypertrophic Cardiomyopathy (HCM)*
2) Coronary Artery Anomalies
3) Aortic Rupture/Marfan*
4) Dilated Cardiomyopathy*5) Myocarditis 6) Left Ventricular Outflow Tract
Obstruction 7) Mitral Valve Prolapse (MVP)
8) Coronary Artery Atherosclerotic Disease*
9) Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)*
*Genetic/Familial
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
DiseaseOther
16) Drugs and Stimulants
17) Primary Pulmonary Hypertension*
18) Commotio Cordis
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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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Other 6%CAD 2%
HCM36%
Coronary anomalies
19%
Cardiac Mass10%
Ruptured Ao 5%
Tunneled LAD 5%
AS 4%
Myocarditis 3%
Dilated C-M 3%
ARVD 3%MVP 2%
Maron BJ, et al. JAMA. 1996;276:199-204.
SCD Profiles
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Italian Experience:• ARVC leading cause of SCD
• HCM, coronary artery anomalies less common
Corrado. J AM Coll Cardiol 2003.
Maron. JAMA 1996.
SCD Profiles (cont.)
Other 6%CAD 2%
HCM36%
Coronary anomalies
19%
Cardiac Mass10%
Ruptured Ao 5%
Tunneled LAD 5%
AS 4%
Myocarditis 3%
Dilated C-M 3%
ARVD 3%MVP 2%
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Nontraumatic Sudden Death During Military Basic Training (Escart. JACC 2004)
A. N=126; 83% exercise-related
B. 64/126 Cardiac
• 39/64 Coronary Artery Anomalies (all LCA from right sinus of Valsalva)
• 13/64 Myocarditis
• 8/64 HCM/LVH
Maron. JAMA 1996.
Italian Experience: Corrado. J AM Coll Cardiol 2003.
SCD Profiles (cont.)
Other 6%CAD 2%
HCM36%
Coronary anomalies
19%
Cardiac Mass10%
Ruptured Ao 5%
Tunneled LAD 5%
AS 4%
Myocarditis 3%
Dilated C-M 3%
ARVD 3%MVP 2%
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Other Causes of Athletic “Collapse”
Heat Stress/Stroke
Vasovagal Faint (Neurocardiogenic Syncope)
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Neurocardiogenic Syncope (NCS)
Prodrome (warning signs) Syncope (loss of consciousness) short duration Occurs at the end of exercise, after exercising has stopped
Blood Pressure
Heart Contractility
Upright Position
Blood Pooling in Lower Body
Filling of Heart
Paradoxical Slow Heart Rate and/or
(Nervous System)
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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.
It is small, portable, automatic, and simple to operate.
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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
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
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Summary
Project SAVE:
Children’s Healthcare of Atlanta SCD Program
I. Differential Diagnosis and Scope of SCD Problem
1) Sudden Cardiac Death: causes-common arrhythmia incidence
2) Sudden Arrhythmia Death Syndromes Foundation Warning Signs
II. Diagnosis andPrimary Prevention
3) Symptomatic vs. Asymptomatic Patients
4) Vital Signs, Family History
5) Impact of proband identification with subsequent family screen
6) Pre-Participation Evaluation Form; appropriate restriction or clearance
7) Universal awareness of warning signs
8) Medical referral based on focused history and/or symptoms
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Summary
Project SAVE:
Children’s Healthcare of Atlanta SCD Program
III. Secondary Prevention 9) CPR: ABC’s
10) 911
11) Defibrillation; AED program implementation
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
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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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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
Sudden Cardiac Arrest in the Young Coalition: Goals/Objectives