Pediatric Sudden Cardiac Death

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Pediatric Sudden Cardiac Death

• Robert M. Campbell, MDCMO, Childrens 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 cant be
  happening. Her heart cant be stopping.

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Sudden Cardiac Death (SCD) Overview

• Infrequent occurrence?
• ? 150K-1200K 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
• Hypertrophic Cardiomyopathy (HCM)
• Coronary Artery Anomalies
• Aortic Rupture/Marfan
• Dilated Cardiomyopathy
• Myocarditis
• Left Ventricular Outflow Tract Obstruction
• Mitral Valve Prolapse (MVP)
• Coronary Artery Atherosclerotic Disease
• Arrhythmogenic Right Ventricular Cardiomyopathy
  (ARVC)

• Electrical
• Long QT Syndrome (LQTS)
• Wolff-Parkinson-White Syndrome (WPW)
• Brugada Syndrome
• Catecholaminergic Ventricular Tachycardia
• Short QT Syndrome
• Post-operative Congential Heart Disease

• Other
• Drugs and Stimulants
• Primary Pulmonary Hypertension
• Commotio Cordis

Genetic/Familial
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Normal Echocardiogram
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SCD Differential Diagnosis Structural/Functional

• Hypertrophic Cardiomyopathy Thickening of the
  heart muscle

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SCD Differential Diagnosis Structural/Functional

• Coronary Artery Anomalies Congenital or
  Acquired

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SCD Differential Diagnosis Structural/Functional

• Aortic Rupture/Marfan Dilatation and thinning
  of the aorta

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SCD Differential Diagnosis Structural/Functional

• Dilated Cardiomyopathy Thinning and weakening
  of the heart muscle

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SCD Differential Diagnosis Structural/Functional

• Myocarditis Inflammation of the heart muscle
• Left Ventricular Outflow Tract Obstruction
  Blockage to the left ventricular outflow
• Mitral Valve Prolapse (MVP) Redundancy of
  mitral valve
• Coronary Artery Atherosclerotic Disease
  Coronary artery plaque and obstruction
• Arrhythmogenic Right Ventricular Cardiomyopathy
  (ARVC) Fatty infiltration of the right
  ventricular muscle

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SCD Differential Diagnosis Primary Electrical

• Long QT Syndrome (LQTS) Abnormal electrical
  reactivation (repolarization)
• Wolff-Parkinson-White Syndrome (WPW) Accessory
  pathway connecting the upper to lower heart
  chambers
• Brugada Syndrome Ventricular fibrillation 3rd
  or 4th decades rare in children
• Catecholaminergic Ventricular Tachycardia
  Exercise induced tachycardia
• Short QT Syndrome Abnormal electrical
  reactivation (repolarization)

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SCD Differential Diagnosis

• Primary Electrical
• Post Operative Congenital Heart Disease
• TGA Senning/Mustard
• Fontan repair
• LV outflow obstruction
• Others
• Other
• Stimulants Ephedra, cocaine, etc.
• Primary Pulmonary Hypertension (PPH)
  Elevated blood pressure in lung arteries

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SCD Differential Diagnosis Other

• Commotio Cordis Blunt blow to the chest

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SCD Profiles
Maron BJ, et al. JAMA. 1996276199-204.
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SCD Profiles (cont.)

• Maron. JAMA 1996.

• Italian Experience
• ARVC leading cause of SCD
• HCM, coronary artery anomalies less common
• Corrado. J AM Coll Cardiol 2003.

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SCD Profiles (cont.)

• Maron. JAMA 1996.

• Italian Experience Corrado. J AM Coll
  Cardiol 2003.

• Nontraumatic Sudden Death During Military Basic
  Training (Escart. JACC 2004)
• N126 83 exercise-related
• 64/126 Cardiac
• 39/64 Coronary Artery Anomalies (all LCA from
  right sinus of Valsalva)
• 13/64 Myocarditis
• 8/64 HCM/LVH

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Other Causes of Athletic Collapse

• Heat Stress/Stroke
• Vasovagal Faint (Neurocardiogenic Syncope)

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Neurocardiogenic Syncope (NCS)
Blood Pressure

• Prodrome (warning signs)
• Syncope (loss of consciousness) short duration
• Occurs at the end of exercise, after exercising
  has stopped

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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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DiagnosisPre-Participation Evaluation
(PPE)Awareness of Warning Signs

• Patient History
• Fainting (syncope) or seizure during exercise,
  excitement or startle
• Consistent or unusual chest pain and/or shortness
  of breath during exercise
• Past detection of a heart murmur or increased
  systemic blood pressure
• Prescription, OTC, and other medications/suppleme
  nts

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DiagnosisPre-Participation Evaluation
(PPE) Awareness of Warning Signs

• Family History
• Premature death or significant disability from
  cardiovascular disease in close relatives younger
  than 50 years of age
• Syncope, seizures, SIDS, accidental death,
  congenital deafness
• Specific knowledge of the occurrence of certain
  conditions
• HCM, DCM, Marfans, LQTS, clinically important
  arrhythmias, pacemaker implantation, early onset
  coronary artery disease, ARVC, PPH, Brugada
• 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 DiseaseImpact 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 AEDs 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 youll need and where
  theyll 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
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Summary
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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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Sudden Cardiac Arrest in the Young Coalition
Goals/Objectives

• Goal No Deaths
• Objectives
• All children screened with family history
  questions
• All MDs (primary care) knowledgeable about
  further screening
• Family health history document for every family
• All school and community sports coaches and staff
  are knowledgeable about the warning signs of SCD
  and the importance of a timely emergency response
• CPR training is encouraged for both school staff
  and students
• Community and school PAD initiatives are supported