Preventing Sudden Cardiac Death in Youth Sports

Victoria L. Vetter, M.D., MPH

Professor of Pediatrics

Perelman School of Medicine at the University of Pennsylvania

Division of Cardiology, The Children’s Hospital of Philadelphia

Sudden Cardiac Arrest (SCA) is a condition in which the heartbeat

stops suddenly and unexpectedly. It is the immediate loss of

electrical heart function. This usually is caused by ventricular

fibrillation (VF), an abnormality in the heart's electrical system

which causes the heart to quiver and blood flow to the body and

brain to stop.

Sudden Cardiac Death (SCD) occurs if emergency treatment with

CPR/AED or spontaneous recovery does not occur.

Scope of the Problem

SCA is the leading cause of death in young athletes – accounting for 75% of all deaths.

Maron; Circulation 2006

Who is At Risk?

2010-2011 High School Sports Participants

7,667,955

60 % of HS Students

Females 3,173,549 41%

Males 4,494,406 59%

Athlete: A participant in a sport, exercise, or game requiring physical skill. Dictionary.com

>30 million US children not on school sports teams

Type of Sport and SCD

Epidemiology of SCD in Children

• Incidence: 3-6/100,000 per-yrs

~1,000 deaths/yr in USA

• Greatest in 10-19 yr age group

• Male: Female 4:1

• Two-thirds occur with exercise

or activity

• Estimated 1:9000 (Military 18-

35 yrs) to 1:27,000 (Atkins) to

1:200,000 (Maron)

4.4

6.37

0.6

4.9

13

2.7

6.2

2.8

0 5 10 15

Drezner

Atkins

Maron

Liberthson

Eckart

Corrado

Shen

Winkel

Only % of cases found in media reports

Death Rates 2005-2007: 5-17 yrs/100,000

SCD 3-6/100,000 person-years

High School/College Basketball Players

Etiology of Sudden Cardiac Death in Children

• Cardiomyopathy

HCM, ARVC , DCM, LVNC

• Primary Electrical Disease

LQTS, SQTS, Brugada

CPVT, Primary VF

WPW

• Congenital Heart Disease

– AF, VT/VF, SSS, CHB

• Coronary Artery Anomalies

• Acquired Heart Disease

Myocarditis

Drugs

Marfan Syndrome

Commotio Cordis/Blow to chest

HCM

43%

LQT/Electrical

Disease

15%

Marfan

5%

WPW

2%

CHD

8%

ARVD/CM

5%

Cor Art

12%

Myocarditis

5%

Commotio

Cordis

5%

Parent Heart Watch Database 2007

Prevalence: 1:500- 1:3000

What We Don’t Know about SCA in the Young

• We don’t know how often these conditions result in death.

• We don’t know how prevalent the SCA high risk conditions are in the young.

• So…are we just looking at the tip of the iceberg with our current information?

There is no required reporting or Registry for SCA/SCD in the US

ECG Abnormal in 95%

Diagnosis of HCM Hypertrophied, non-dilated left ventricle

Long QT Syndrome Characteristics

• Prolonged QT Interval

• Syncope/Fainting

• Malignant Ventricular Arrhythmias Torsades de Pointes

• Sudden Death

Survival Following Sudden Cardiac Arrest

• SCA in athletes is a catastrophic event with a low survival rate (11-16%)

•Low survival rate demands re-evaluation of our current screening and prevention practices.

How Can We Prevent Sudden Cardiac Death

Primary Prevention Preparticipation Evaluation/PPE

–Goal of Screening is to detect potentially life-threatening conditions (Prevalence 0.2-0.4% by estimate, or 1:250-500 young persons).

–Risk stratification to determine who will have a SCA is imperfect.

Methods of Preparticipation Evaluation

• History

– Personal and Family

• Physical Exam

– Murmurs

– Hypertension

– Stigmata of Marfan Syndrome

– Femoral Pulses

• ECG • Echocardiogram • Genetic testing

Preparticipation Physical Evaluation –4th Edition

• Provides a uniform comprehensive history and physical form

• Endorsed by 6 national organizations

• Describes the important questions

AAFP, AAP, ACSM, AMSSM, AOSSM, AOASM

Have you ever passed out or nearly passed out during or after exercise?

Have you ever had discomfort, pain, pressure, or tightness in your chest during exercise?

Do you get lightheaded or feel more short of breath than expected during exercise?

Does your heart ever race or skip beats (irregular beats) during exercise?

Has a doctor ever told you that you have: any heart problem, high blood pressure, high cholesterol, a heart murmur, a heart infection, or an unexplained seizure disorder?

Has a doctor ever ordered a test for your heart (for example, ECG or echocardiogram)?

Has any family member or relative died of heart problems or had an unexpected or unexplained sudden death before age 50 (including drowning, car accident, or sudden infant death syndrome)?

Has anyone in your family had unexplained fainting, seizures, or near drowning?

Does anyone in your family have a heart problem, pacemaker, or implanted defibrillator?

Does anyone in your family have: hypertrophic cardiomyopathy, Marfan syndrome, arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, short QT syndrome, Brugada syndrome, or catecholaminergic polymorphic ventricular tachycardia?

PPE 4th Edition Recommended Personal & Family History Questions

US AHA Strategy to Deal with the Problem

2007: AHA 12 element program: The recommendations included

”…a complete medical history and physical examination, including brachial artery blood pressure measurement.”

Comprehensive screen repeated every 2 years for high school athletes with annual updates

Noninvasive testing was not recommended but not, “actively discouraged.”

Maron et al: Circ. 2007

Problems with Identification of Conditions that Cause SCA

• Symptoms

Occur in 30-50%

May not be present prior to the SCA

SCA 1st symptom: 30-80%

• Family History

Often not known

Positive family history: 20-30%

• Physical Exam

The conditions that cause Sudden Cardiac Arrest may be subtle and not apparent on routine physical exam.

No study has shown that History & Physical can adequately find athletes at risk and prevent SCD.

Methods of Preparticipation Evaluation

Use of ECG to Screen for Cardiac Conditions Associated with SCA

WPW HCM

LQTS

75-95% Abnormal

ECG Rarely Identifies • CPVT • Coronary Artery Anomalies Not identified by H & P either

Who Uses the ECG to Screen for SCD?

Japan 1973 1st, 7th, 10th graders

Italy 1979 12-35 yo athletes

12 EU countries

IOC

Italian Athletic Screening Program

Medical Protection Athletes Act – 1979

All 12-35 yo who compete in

sports History ,Physical Exam, and ECG • More cases found with ECG than

history & physical exam • ECG had 77% greater power to

detect HCM than History and Physical Exam alone

22

82%

23%

0

5

10

15

20

25 # HCM

ABNECG

Pos.FH/M

0.07%

Corrado NEJM 1998

Would Identifying the Condition Make a Difference?

Italian Athletic Screening Program

Corrado, JAMA 2006

1979-2004: 42,386 athletes screened in Padua, Italy

89% Decrease in Sudden Cardiac Death

Concerns Regarding ECG Screening

• Low disease prevalence, Low PPV

• Current ECG standards may not correctly identify all abnormalities.

Develop more specific new reference standards with norms for race, ethnicity and gender.

• False positives anxiety and costs

Improve test characteristics

• Athletic training affects ECG

Recent data suggests this can be distinguished from pathologic changes

• Limited manpower to interpret ECG:

Use remote reading and computers

• Disqualification concerns: 2%0.2%

Corrado 2009

•Lack of proof for ID of conditions and prevention of SCA Same for ECG and History and Physical

ECG Screening Cost Effectiveness/Year of Life Saved

$0

$5,000

$10,000

$15,000

$20,000

$25,000

$30,000

$35,000

$40,000

$45,000

$50,000

$8,800

$15,926

$44,000 $42,900 $40,855

Japan Italy Neo Nevada HS Wheeler Anderson

Cost Effectiveness Analysis

Fuller et al. , Med Sci Sports Exerc 2000

Am Heart J 2011

Previously undiagnosed CV abnormalities 10.75% –Hypertension >98th % 5% (20) –Important CV Abnormalities 2.5% (10)

400 healthy children –Ages 5-19 years recruited from pediatric practices in Philadelphia area –Questionnaire with patient medical and family history –Physical Exam, ECG, Echocardiogram

Community School Screening Model CHOP Heart Health Screening Study

• 2781 children 5-19 years screened

• Conditions requiring treatment:17

• 2 Atrial Septal Defects

• 7 LQTS

• 7 WPW

• 1 Marfan Syndrome

• 2 Potential cardiomyopathies for follow-up

• Other potentially significant

• Ventricular couplets, 2AV block, Aortic root dilation

Prevention of Sudden Cardiac Death

–Secondary Prevention

•Lifestyle modification, Medication, ICD implants

•Implement CPR/AED Programs –AEDs can be safely placed in schools

National Registry for AED Use in Sports Survey

Total Schools 1710 high schools with on-site AED programs

Interval 2006-07

Emergency Response Plan 83%

Practice Plan Annually 40%

SCA 36 (2.1%)

14 student athletes (16, 14-17 yrs)

22 non-students (57, 42-71 yrs)

Witnessed SCA 35 (97%)

Bystander CPR 34 (95%)

AED shock 30 (83%)

Survival to Hospital Discharge 23 (64%)

9 of 15 students

14 of 22 non-students Drezner et al, Circulation 2009; 120: 518-525

Who Has Laws for AEDs in Schools?

• 24 states have some type of legislation regarding AEDs in schools but only 17 mandate AEDs on a continuing basis

• 7 provide a lower level of coverage

• Other states have laws on CPR, AED teaching

STATE SCHOOL AED LEGISLATION

Ongoing Programs Al, AR, CT, FL, DE, GA, IL, MD,ME, NV, NY, OH, OK, OR,SC,TN,TX

One time Program or limited by funds or group (e.g. College only)

CA, CO, MI, ND, NH,PA,RI

CPR/AED Instruction WI, IA

What happens next? It’s up to YOU……………………

Make a difference in YOUR community. Thank you!