Preparticipation Physical Evaluation PPE and Prevention of Sudden Cardiac Death

1
Preparticipation Physical Evaluation (PPE) and
Prevention of Sudden Cardiac Death

• Jack Stevens, MD, FACCDirector of Preventive
  Cardiology and Exercise Physiology, Sibley Heart
  Center
• Section Chief of Cardiology, Childrens
  Healthcare of Atlanta at Scottish Rite Assistant
  Professor of Pediatrics, Emory University School
  of Medicine

2
Sudden Cardiac Death (SCD) Overview

• Infrequent occurrence?
• 600 -1,000 children and adolescents
• 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
• SCD episodes may not be predictable or preventable

3
Introduction
A quiet premise do the basics better is
gaining attention amid the highly public medical
and technological advancements designed to save
lives following a cardiac arrest. (CPR Revived
, Dec 2005)
4
Advanced Screening with EKG or
Echocardiography Pros and Cons

• PRO
• Echo identifies HCM better than exam EKG may be
  superior echocardiography for HCM.
• EKG can identify asymptomatic cardiac conduction
  problems.
• Tests make everyone feel like they have done a
  better screening.

• CON
• EKG and echocardiographic screening have not been
  shown to reduce sudden cardiac deaths.
• False positives are high for both?leading to
  further testing (increased costs) and often
  inappropriate disqualification.
• Athletic heart
• Echocardiographic screening misses many potential
  causes of SCD, including some HCM.
• If athletes deserve advanced screening so do
  other students.
• Echo. screening needs to be repeated every 1-2
  years for HCM in the HS age group.
• Cost vs. benefit fairness for lower income
  groups?

5
Has PPE Worked?
Pre-participation screening appeared to be of
limited value for identification of underlying
cardiovascular abnormalities (Maron, 1996)
6
Sudden Cardiac Death (SCD) What Are We
Screening For?

• Structural/Functional
• Hypertrophic Cardiomyopathy (HCM)
• Coronary Artery Anomalies
• Aortic Rupture/Marfan
• Dilated Cardiomyopathy (DCM)
• Myocarditis
• Left Ventricular Outflow Tract Obstruction
• Mitral Valve Prolapse (MVP)
• Coronary Artery Atherosclerotic Disease
• Arrhythmogenic Right Ventricular Cardiomyopathy
  (ARVC)
• Post-operative Congenital Heart Disease

• Electrical
• Long QT Syndrome (LQTS)
• Wolff-Parkinson-White Syndrome (WPW)
• Brugada Syndrome
• Catecholaminergic PolymorphicVentricular
  Tachycardia (CPVT)
• Short QT Syndrome

• Other
• Drugs and Stimulants
• Primary Pulmonary Hypertension (PPH)
• Commotio Cordis

7
Sudden Cardiac Death (SCD) Differential
Diagnosis

• Structural/Functional
• Hypertrophic Cardiomyopathy (HCM)
• Coronary Artery Anomalies
• Aortic Rupture/Marfan
• Dilated Cardiomyopathy (DCM)
• Myocarditis
• Left Ventricular Outflow Tract Obstruction
• Mitral Valve Prolapse (MVP)
• Coronary Artery Atherosclerotic Disease
• Arrhythmogenic Right Ventricular Cardiomyopathy
  (ARVC)
• Post-operative Congenital Heart Disease

• Electrical
• Long QT Syndrome (LQTS)
• Wolff-Parkinson-White Syndrome (WPW)
• Brugada Syndrome
• Catecholaminergic PolymorphicVentricular
  Tachycardia (CPVT)
• Short QT Syndrome

• Other
• Drugs and Stimulants
• Primary Pulmonary Hypertension (PPH)
• Commotio Cordis

Familial / Genetic
8
Why Should We Expect That PPE Would Work?
9
SCD Differential Diagnosis Structural/Functional

• 1) Hypertrophic Cardiomyopathy
• Thickening of the heart muscle

Without Obstruction
With Obstruction
Normal
10
PPE FindingsHCM

• Exertional chest pain and/or dyspnea, arrhythmia
  symptoms, fatigue, syncope/near syncope/SCD
• EIB symptoms
• Post exertional syncope/SCD
• Cardiac murmur
• Family history of HCM, non-specific
  cardiomyopathy
• Family history of heart disease

Adabag, AS (2006)Nugent, AW (2005)Lipshultz and
Colan (2007)
11
PPE FindingsARVC

• Exercise-induced syncope/near syncope/SCD
• Exercise palpitations
• Family history of ARVC, SCD

Corrado, D (1998)Dalal, D (2005)Thiene, G (1998)
12
SCD Differential Diagnosis Primary Electrical
11) Long QT Syndrome (LQTS)

• Normal QT

• Prolonged QT

QT 315ms QTc 407ms
QT 410ms QTc 499ms
13
Figure 1Am Heart J 5459-68, 1957
a ECG July 20, 1953 During Rest Leads I, II,
III, IV R Q-T 0.50 sec. R-R 0.88 sec.
b ECG July 20, 1953After Stair-Running Leads
I, II, III, IV, R Q-T0.60 sec. R-R0.86 sec.
14
EKG Patterns T-Waves
LQT1broad based
LQT3 late onset
LQT2 bifid, notched
15
PPE FindingsLong QT Syndrome

• Exercise/emotion/startle syncope or seizure
  (occasionally misdiagnosed as neurologic or
  vasovagal)
• Drowning/near drowning
• SIDS
• Congenital deafness
• Family history of SCD, seizure, syncope, LQTS

Moss, AJ (1991)Tester DJ (2007)Choi, G (2004)
16
SCD Differential Diagnosis Structural/Functional

• Coronary Artery Anomalies Congenital or
  Acquired

17
Congenital Coronary Artery AnomaliesLeft From
The Right
18
PPE FindingsCongenital Coronary Artery
Abnormalities

• Exercise syncope, chest pain, dyspnea,
  palpitations, SCD
• No family history

Eckart, RE (2004)Basso, C (2000)
19
PPE Findings - CPVT (Catecholaminergic
Polymorphic Ventricular Tachycardia)

• Young patient age
• Exercise/emotion induced seizure/syncope
  (occasionally misdiagnosed as epilepsy)
• Drowning / near drowning
• Family history of CPVT, seizure/syncope/SCD

Leenhardt, A (1995)Priori, SG (2002)
20

PPE FindingsBrugada Syndrome

• Older patient age (3rd 4th decade)
• Syncope
• Family history of Brugada Syndrome, syncope, SCD

Priori, SG (2000)Sarkozy, A (2007)Priori, SG
(2002)
21
Other Causes of Athletic Collapse

• Heat Stress/Stroke Importance of conditioning,
  attention to environmental temperature and
  humidity, continuous access to water. Always take
  the opportunity to remind coaches in your
  community when summer and early fall practices
  start.
• Vasovagal Faint (Neurocardiogenic Syncope)

22
Neurocardiogenic Syncope (NCS)
Blood Pressure

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

23
Primary PreventionPre-participation Physical
Exam

• Goal Appropriately restrict appropriately clear
• Be thorough and conscientious
• Are there any warning signs or family history?

24
AHA Statement 2007
Medical History Personal history 1.
Exertional chest pain/discomfort 2.
Unexplained syncope/near syncope 3. Excessive
exertional and unexplained dyspnea/fatigue,
associated with exercise 4. Prior recognition
of a heart murmur 5. Elevated systemic blood
pressure Family History 6. Premature death
(sudden and unexpected, or otherwise) before age
50 years due to heart disease, in 1
relative 7. Disability from heart disease in a
close relative knowledge of certain conditions in family
members hypertrophic or dilated cardiomyopathy,
long-QT syndrome or other ion channelopathies,
Marfan syndrome, or clinically important
arrhythmias Physical examination 9. Heart
murmur 10. Femoral pulses to exclude aortic
coarctation 11. Physical stigmata of Marfan
syndrome 12. Brachial artery blood pressure
(sitting position)
Circulation 2007
25
AHA Statement 2007
Medical History Personal history 1.
Exertional chest pain/discomfort 2.
Unexplained syncope/near syncope 3. Excessive
exertional and unexplained dyspnea/fatigue,
associated with exercise 4. Prior recognition
of a heart murmur 5. Elevated systemic blood
pressure Family History 6. Premature death
(sudden and unexpected, or otherwise) before age
50 years due to heart disease, in 1
relative 7. Disability from heart disease in a
close relative knowledge of certain conditions in family
members hypertrophic or dilated cardiomyopathy,
long-QT syndrome or other ion channelopathies,
Marfan syndrome, or clinically important
arrhythmias Physical examination 9. Heart
murmur 10. Femoral pulses to exclude aortic
coarctation 11. Physical stigmata of Marfan
syndrome 12. Brachial artery blood pressure
(sitting position)

• Seizure
• Family history of seizure, syncope, accidental
  death or near drowning
• Pacemaker / AICD
• Congenital deafness

Circulation 2007
26
American Academy of PediatricsPPE 2005

• Have you ever passed out or nearly passed out
  DURING exercise?
• Have you ever passed out or nearly passed out
  AFTER exercise?
• Have you ever had discomfort, pain, or pressure
  in your chest during exercise?
• Does your heart race or skip beats during
  exercise?
• Has a doctor ever told you that you have
• High blood pressure
• High cholesterol
• A heart murmur
• A heart infection
• Has a doctor ever ordered a test for your heart?
  ECG, echocardiogram)
• Has anyone in your family died for no apparent
  reason?
• Does anyone in your family have a heart problem?
• Has any family member or relative died of heart
  problems or of sudden death before age 50?
• Does anyone in your family have Marfan syndrome?
• Physical Exam (4)

27
American Academy of PediatricsPPE 2005

• Have you ever passed out or nearly passed out
  DURING exercise?
• Have you ever passed out or nearly passed out
  AFTER exercise?
• Have you ever had discomfort, pain, or pressure
  in your chest during exercise?
• Does your heart race or skip beats during
  exercise?
• Has a doctor ever told you that you have
• High blood pressure
• High cholesterol
• A heart murmur
• A heart infection
• Has a doctor ever ordered a test for your heart?
  ECG, echocardiogram)
• Has anyone in your family died for no apparent
  reason?
• Does anyone in your family have a heart problem?
• Has any family member or relative died of heart
  problems or of sudden death before age 50?
• Does anyone in your family have Marfan syndrome?

• Exercise SCD, dyspnea, fatigue
• Family history of seizure, syncope, accidental
  death or near drowning
• SIDS
• Specific arrhythmic disorders

28
Cardiovascular Risk Assessment Form

• Has your child fainted or passed out DURING or
  AFTER exercise, emotion or startle?
• Has your child ever had extreme shortness of
  breath during exercise?
• Has your child had extreme fatigue associated
  with exercise (different from other children)?
• Has your child ever had discomfort, pain or
  pressure in his chest during exercise? 
• Has a doctor ever ordered a test for your childs
  heart?
• Has your child ever been diagnosed with an
  unexplained seizure disorder? or
  exercise-induced asthma not well controlled with
  medication?
• Are there any family members who had a sudden,
  unexpected, unexplained death before age 50?
  (including SIDS, car accident, drowning, others)
  or near drowning
• Are there any family members who died suddenly of
  heart problems before age 50?
• Are there any family members who have had
  unexplained fainting or seizures?
•  Are there any relatives with certain conditions,
  such as
• Enlarged Heart
• Hypertrophic cardiomyopathy (HCM)
• Dilated cardiomyopathy (DCM)
• Heart Rhythm problems Long QT syndrome
  (LQTS)
• Short QT syndrome
• Brugada syndrome
• Catecholaminergic ventricular tachycardia
• Arrhythmogenic right ventricular cardiomyopathy
  (ARVC)
• Marfan syndrome (aortic rupture)

29
Is This A Sports Clearance Form?
30
Cardiovascular Risk Assessment Form

• Has your child fainted or passed out DURING
  exercise, emotion or startle?
•  Has your child fainted or passed out AFTER
  exercise?
•  Has your child ever had extreme shortness of
  breath during exercise?
• Has your child had extreme fatigue associated
  with exercise (different from other children)?
• Has your child ever had discomfort, pain or
  pressure in his chest during exercise? 
• Has a doctor ever ordered a test for your childs
  heart?
• Has your child ever been diagnosed with an
  unexplained seizure disorder? or
  exercise-induced asthma not well controlled with
  medication?
• Are there any family members who had a sudden,
  unexpected, unexplained death before age 50?
  (including SIDS, car accident, drowning, others)
  or near drowning
• Are there any family members who died suddenly of
  heart problems before age 50?
• Are there any family members who have had
  unexplained fainting or seizures?
•  Are there any relatives with certain conditions,
  such as
• Enlarged Heart
• Hypertrophic cardiomyopathy (HCM)
• Dilated cardiomyopathy (DCM)
• Heart Rhythm problems Long QT syndrome
  (LQTS)
• Short QT syndrome
• Brugada syndrome
• Catecholaminergic ventricular tachycardia
• Arrhythmogenic right ventricular cardiomyopathy
  (ARVC)

• Any patient
• Any age
• Any time
• Any MD

• Ask the right questions
• Ask the right way (tell me about )

• Family accountability

• Right physician response

• Is this form too detailed?
• Compliance
• Correct, complete

31
Summary

• Warning signs frequently exist in
  patients/families at risk for SCD
• These symptoms may be subtle and nonspecific, but
  also misinterpreted or disregarded
• Diligent attention to the details of a PPE (or CV
  risk assessment questionnaire) is critical

32
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.

33
(No Transcript)
34

• Photo courtesy of Sports Illustrated
• Hank Gathers in VF carried off court
• Team physician carrying AED (R hand) was
  NOT trained in AED use

35

• Are School AEDs the
• Right Thing To Do?

36
Project S.A.V.E. Summary

• Project S.A.VE. Program at Childrens Healthcare
  of Atlanta available to any Georgia school to
  assist with SCD prevention
• S Sudden Cardiac Death
• A Awareness
• Warning signs, recognition, need for timely
  response
• Resources
• V Vision for Prevention
• ? SCD
• ? Collaboration
• E Education for the School Community
• Pre-Participation Evaluation process
• CPR training for staff and students
• AED program implementation