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Pediatric Sudden Cardiac Death

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Impact of Proband Identification. 25. Role of Routine EKG and/or. Echo Screen. Athletes only? ... Impact of proband identification with subsequent family screen ... – PowerPoint PPT presentation

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Title: Pediatric Sudden Cardiac Death


1
Pediatric Sudden Cardiac Death
  • Robert M. Campbell, MD CMO, Childrens 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
  • 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.

4
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

5
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
6
Normal Echocardiogram
7
SCD Differential Diagnosis Structural/Functional
  • Hypertrophic Cardiomyopathy Thickening of the
    heart muscle

8
SCD Differential Diagnosis Structural/Functional
  • Coronary Artery Anomalies Congenital or
    Acquired

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

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

11
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

12
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)

13
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

14
SCD Differential Diagnosis Other
  • Commotio Cordis Blunt blow to the chest

15
SCD Profiles
Maron BJ, et al. JAMA. 1996276199-204.
16
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.

17
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

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

19
Neurocardiogenic Syncope (NCS)
Blood Pressure
  • Prodrome (warning signs)
  • Syncope (loss of consciousness) short duration
  • Occurs at the end of exercise, after exercising
    has stopped

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
  • 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

22
Diagnosis Pre-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

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 AEDs 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 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

34
Summary
35
Summary
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
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
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