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

2

The Atlanta Journal ConstitutionSunday, September 7, 2003

3

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.”

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

DiseaseOther

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 3rd or 4th 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

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

16

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%

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 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%

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

• 6th vs 9th vs 12th 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 9th 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 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

35

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

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