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

2 The Atlanta Journal Constitution Sunday, September 7, 2003

3 ‘There was this beautiful young lady laying there, and I kept thinking, “This can’t be happening. Her heart can’t be stopping.”

4 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

5 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 Disease Other 16) Drugs and Stimulants 17) Primary Pulmonary Hypertension * 18) Commotio Cordis

6 Normal Echocardiogram

7 SCD Differential Diagnosis: Structural/Functional 1) Hypertrophic Cardiomyopathy: Thickening of the heart muscle

8 SCD Differential Diagnosis: Structural/Functional 2) Coronary Artery Anomalies: Congenital or Acquired

9 SCD Differential Diagnosis: Structural/Functional 3) Aortic Rupture/Marfan: Dilatation and thinning of the aorta

10 SCD Differential Diagnosis: Structural/Functional 4) Dilated Cardiomyopathy: Thinning and weakening of the heart muscle

11 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

12 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 3 rd or 4 th decades; rare in children 13) Catecholaminergic Ventricular Tachycardia: Exercise induced tachycardia 14) Short QT Syndrome: Abnormal electrical reactivation (repolarization)

13 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

14 SCD Differential Diagnosis: Other 18) Commotio Cordis: Blunt blow to the chest

15 Maron BJ, et al. JAMA. 1996;276: SCD Profiles

16  Italian Experience: ARVC leading cause of SCD HCM, coronary artery anomalies less common Corrado. J AM Coll Cardiol  Maron. JAMA SCD Profiles (cont.)

17  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  Italian Experience: Corrado. J AM Coll Cardiol SCD Profiles (cont.)

18 Other Causes of Athletic “Collapse”  Heat Stress/Stroke  Vasovagal Faint (Neurocardiogenic Syncope)

19 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)

20 PPE: Does It Work?  Appropriately restrict; appropriately clear  Be thorough and conscientious  Are there any warning signs?

21 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”

22 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

23 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

24 Familial Disease: Impact of Proband Identification

25 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 6 th vs 9 th vs 12 th grade? School athletics only?

26 Role of Routine EKG and/or Echo Screen (cont.)  What age for screen? 50% LQTS patients who die succumb before 9 th grade HCM may have a pre-hypertrophic phase  For example: –Normal echo at age 10, but… –Abnormal echo at age 20

27 Role of Routine EKG and/or Echo Screen (cont.)  Screen for what diagnoses? HCM only? OR Comprehensive echo and EKG screening for any cause

28 Role of Routine EKG and/or Echo Screen: Summary  Unfavorable cost: benefit ratio  False positives and false negatives  Negative screen does not exclude disease

29 Project SAVE PPE Recommendation Comprehensive medical evaluation if positive PPE or signs/symptoms

30 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

31 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.

32 Are School AED’s the “Right Thing To Do”?

33 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

34 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 and Primary 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

35 Summary Project SAVE: Children’s Healthcare of Atlanta SCD Program III. Secondary Prevention 9) CPR: ABC’s 10) ) 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

36 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

37 Project SAVE Recommendations  Universal awareness of warning signs  Conscientious use of PPE Form and process  Comprehensive screen of high risk patients and families

38 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