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Transcript
Cardiovascular Preparticipation
Sports Screening
Are we doing enough?
Sanjay Lambore, MD, MSc, FAAP, May 2012
Updated by Ana Malinow, MD, March 2015
Objectives:
At the end of this learning experience,
viewers will be able to:
 Discuss the role of sports preparticipation examination
in preventing sudden cardiac death
 Enumerate the causes of sudden cardiac death in
athletes
 Discuss the significance of exhaustive history and
clinical findings to rule out cardiac disease in young
athletes
 Discuss American Heart Association (AHA)
recommendations for cardiovascular screening of
young athletes
Case
Anthony was a 16 year old, 6 feet 3 inches tall young man who
aspired to be a professional basket ball player. He played for his
school’s varsity team. He had a perfectly normal sports physical
exam at his pediatrician’s office one month ago.
During the second match of the season, he suddenly collapsed
and died on the court.
Question 1
 What are the goals of cardiovascular
preparticipation sports screening?
 What are the AHA recommendations for
preparticipation screening for
cardiovascular abnormalities in competitive
young athletes?
 What are the current PIAA (Pennsylvania
Interscholastic Athletic Association)
preparticipation forms?
Goals
of cardiovascular preparticipation
screening of athletes.
Goals of screening are to:
 Identify cardiac disorders and prevent sudden
unexpected death
 Prevent disease exacerbations
 Meet legal requirements
The 14-Element AHA Recommendations for
Preparticipation Cardiovascular Screening for
Competitive Athletes
 Medical History
 Personal history
 Family history
 Physical Examination
Click Here to View The 14-Element AHA Recommendations for Preparticipation Cardiovascular
Screening of Competitive Athletes
Source: AHA/ACC Scientific Statement.
Circulation, 2014
* Parental verification recommended for HS
and MS athletes
† Judged not to be vasovagal origin; of
particular concern when occurring during or
after physical exertion
‡ Refers to heart murmurs judged likely to be
organic and unlikely to be innocent
§Preferably taken in both arms
From:
http://circ.ahajournals.org/content/130/15/13
03/T1.expansion.html
PIAA Comprehensive Initial
Preparticipation Physical
Evaluation Section 5: Health
History
 Click Here to View the PIAA Comprehensive
Initial Preparticipation Physical Evaluation
Section 5 Health History
 Your state might have a different PPE
 Do you know which form your state uses?
PIAA Section 6: Comprehensive
Initial Preparticipation Physical
Evaluation and Certification of
Authorized Medical Examiner
 Click Here to View the PIAA Comprehensive
Initial preparticipation Physical Evaluation
and Certification of Authorized Medical
Examiner
 Your state might have a different PPE
 Do you know which form your state uses?
Question 2
 What are the limitations to screening for
cardiovascular pathology at the time of
sports physical?
Limitations to screening
sudden cardiac death in athletes
 Due to low prevalence of sudden death rate
(<1%), no screening program will be cost
effective.
 The idea of preventing such a low frequency
event is challenging and the efficacy of the
screening program is difficult to prove.
 Due to low prevalence rate of sudden death in
sports - poor sensitivity and positive predictive
values of the methods
The Italian (Veneto)
experience
 Since 1982, all athletes in Italy 12-35 y/o have been
mandated to have PPE and 12-lead ECG
 Partly subsidized by national health system
 Performed by accredited sports medicine MD
specialists
 Study by Corrado et al. (2006) showed that 12-lead
ECG reduced SD mortality in young athletes by 90%
(4.3 to 0.87:100,000 person years)
 Prompted the European Society of Cardiology in
2005 to recommend national screening in EU and US
Should a 12-lead ECG be used
as a universal screening tool?
 Very controversial topic
 ECGs have greater sensitivity than H&PE, however,




they have:
Reduced sensitivity with false positive (FP) rates up
to 90%
LV remodeling and changes in ECGs in young
patients are a potential source of false negative
(FN) dx
Misplacement of leads results in misdiagnosis
Lack of standardization of interval durations
12-lead ECG, continued
 Upper limit of normal for QT duration suitable for LQTS
can be problematic
 Overlap in the ECGs of healthy young athletes and
patients with hypertrophic cardiomyopathy can be
challenging
 A FP rate of 5% in 10 million athletes = 500,000 requiring
further testing to r/o heart disease (with only 1,500
pediatric cardiologists in the US)
 Positive predictive value if prevalence is < 1% becomes
trivial
Position of American Heart
Association
 AHA recommends a screening methodology –
systematic medical history and physical examination
using the 14-element questionnaire
 AHA panel does not recommend routine use of 12- lead
ECG
 Lack of adequate economic resources
 Absence of specialized physicians to perform
screening and interpretation
Question 3
 What is the incidence of sudden cardiac death
in athletes?
 What is the magnitude of the problem?
Magnitude of the problem
sudden cardiac death in athletes
 Sudden cardiac death (SD) is the most common medical
cause of death in athletes
 However, SD in US athletes is rare (~75 athletes/yr)
 There are ~7.5 million competitive interscholastic athletes.
Overall, including youth, middle school, masters-level,
college, junior college, and professionals, there are ~ 10
million athletes in the US
 The sudden cardiac death rate among
-high school athletes = 1:100,000-200,000
-marathon runners = 1:50,000
Comparative frequencies of death
attributable to all causes for
individuals <25 y/o

Click Here to View the Comparative frequencies of death attributable
to all causes in young individuals aged under 25 years.
Gender
sudden cardiac death in athletes
Male predominance (male / female ratio 9:1)
due to:
 Higher sports participation rate of males
 More intensive training load and level of athletic
achievement of males
 Greater prevalence and /or phenotypic
expression of cardiomyopathies and premature
coronary diseases in young males
Question 4
 What are the absolute cardiac
contraindications to sports participation
according to the 2005 36th Bethesda
Conference?
Absolute Contraindication to Sports
Participation
 Pulmonary vascular disease with cyanosis and large right to
left shunt
 Severe pulmonary hypertension
 Severe aortic stenosis/regurgitation and mitral
stenosis/regurgitation
 Cardiomyopathies, including HCM and ARVC
 Vascular form of Ehlers-Danlos syndrome
 LQTS (if genotype+/phenotype-, may participate except in
pts. with LQT1, who should refrain from competitive
swimming)
Absolute Contraindication to
Sports Participation
 Coronary artery anomalies
 Catecholaminergic polymorphic ventricular
tachycardia
 Acute phase of pericarditis
 Acute phase of myocarditis (at least 6 months)
 Acute phase of Kawasaki disease (at least 8 weeks)
 Brugada Syndrome (advisable)
 Some presentations of Marfan Syndrome
Question 5
 What are the causes of sudden cardiac death
in athletes?
 What is the most common mechanism of
death?
Causes
sudden death in athletes

~ 40% from hypertrophic cardiomyopathy and
unexplained left ventricular hypertrophy

15-20% from coronary artery anomalies

<5%




Myocarditis
Ruptured aortic aneurysm (Marfan syndrome)
Arrhythmogenic right ventricular cardiomyopathy
Aortic valve stenosis
Causes
sudden death in athletes, continued







Atherosclerotic coronary disease
Dilated cardiomyopathy
Mitral valve prolapse
Drugs – cocaine, amphetamine
WPW syndrome
Trauma – commotio cordis
Sickle cell trait
 <1% Long QT syndrome, Brugada syndrome
Mechanism of death in CD
 Most common mechanism is ventricular
tachyarrhythmia
 In Marfan Syndrome and disorders associated
with aortic dissection the mechanism is aortic
dissection/rupture
Question 6
 How prevalent is hypertrophic
cardiomyopathy and what are the presenting
symptoms?
Hypertrophic Cardiomyopathy
 Most common cause of sudden cardiac death
 Autosomal dominant inheritance with more than
1500 mutations on 16 genes; genetic testing may
identify family members with the mutation even if
they have not yet developed clinical signs
 Important to refer to genetics if HCM is present in
the family
 Prevalence in general population of 1:500
Hypertrophic Cardiomyopathy
contd.
 Clinical features which may raise suspicion include:
-Family history of HCM
-Symptoms of left ventricular outflow
obstruction
-A heart murmur
Clinical Features of HCM
 History
 Mostly asymptomatic (80%); sudden death at sports
often is the first clinical manifestation
 Dyspnea in 90% of symptomatic patients
 Syncope
 occurs due to inadequate cardiac output on
exertion or from cardiac arrythmia; it identifies
children with significant increased risk of sudden
death and warrants an urgent evaluation and
aggressive treatment
Clinical Features of HCM
contd.
 Presyncope
 refers to “Graying Out Spells” in erect posture
relieved by immediately lying down; this is a
common symptom in teenagers but a cardiac
etiology should be ruled out
 Exercise intolerance
 Angina
 Dizziness
Physical findings - HCM
 Arrythmia – atrial fibrillation, atrial flutter, ventricular
ectopy, ventricular tachycardia and ventricular fibrillation
 Double apical impulse – forceful left atrial contraction
against a highly noncompliant left ventricle
 First heart sound is normal. 2nd heart sound could be
paradoxically split
 3rd and 4th heart sounds – atrial systole against a highly
noncompliant left ventricle
Physical findings - HCM
contd.
 Systolic ejection crescendo-decrescendo murmur
heard best between apex and left sternal border
 Radiates to the suprasternal notch (not to carotids or
neck)
 Diminishes with increase in preload (Valsalva
maneuver, squatting)
 Increases with decrease in preload (diuretics or
standing)
Physical findings - HCM
contd.
 Holosystolic murmur of mitral regurgitation (apex and left axilla)
 Diastolic decrescendo murmur of aortic regurgitation is heard in
10% of children with HCM
 The physical finding most suggestive of hypertrophic
cardiomyopathy is a systolic murmur accentuated in the standing
position
 REMEMBER: THERE MAY BE NO FINDINGS ON AUSCULTATION
Question - HCM
 You see a 13 yr old boy for sports PE who
wants to play football. Father has HCM. The
patient was seen by Cardiology 3 yrs ago and
was told that his heart was fine. His exam in
your office is completely normal.
 Would you certify him to play football? Would
you order an ECG, chest xray, genetic testing
or cardiology consult?
Answer
Recommend Cardiology consult and genetics testing
 HCM – Familial HCM has autosomal dominant inheritance






caused by sarcomere gene mutations
50% inheritance chance in the offspring
Phenotypic expression can vary in the same family
HCM may not be evident on echo until young adulthood
Regular cardiac evaluations (perhaps yearly) are
recommended
Other family members should be counseled about their risks
and genetic testing should be offered
See: GeneReviews http://www.ncbi.nlm.nih.gov/books/NBK1768/
Question 7
 What is the “Athlete’s Heart” ?
Athlete’s Heart
 Remodeling of cardiac muscle by athletic training.
Enlargement of the left ventricular cavity due to
increased stroke volume. Cardiac output not
affected due to low heart rate
 Degree of left ventricular enlargement may reach
dimensions of pathological heart
 ECG findings may overlap diagnostic criteria for
HCM
Athlete’s Heart
contd.
 In addition to structural changes, the ECG may
reveal arrhythmias and conduction
abnormalities
 Athletes with left ventricular enlargement or
hypertrophy who do not have HCM should be
cleared to participate in sports by cardiology
 Within a few weeks to months of detraining,
morphologic changes seen in Athlete’s Heart
regress
Question 8
 What is the second most common cause of
sudden cardiac death in athletes?
Congenital Coronary Artery
Anomalies
 Second leading cardiac cause of death in the
playing field
 Left main coronary artery originating at an acute
angle from the right sinus of valsalva and traveling
between the aorta and main pulmonary artery
 Young athlete with history of chest pain or syncope
particularly with exercise – myocardial ischemia
Congenital Coronary Artery
Anomalies
 Findings on PE and ECG – normal
 Transthoracic or transesophageal echo and MRI for
diagnosis. Coronary arteriography - definitive
 Clinical diagnosis requires a high index of suspicion
 particularly important ; surgical correction
Question 9
 List the clinical findings of Marfan syndrome.
Marfan Syndrome
 Cardiac – Aortic root dilatation, aortic dissection and mitral
valve prolapse
 Sudden cardiac death is due to dissection and rupture of the
aorta
 Skeletal, ocular, pulmonary – Kyphoscoliosis, pectus
deformities, increased arm span, hypermobility, pes planus,
lens dislocation, myopia and history of spontaneous
pneumothorax
 WATCH FOR THOSE TALL BASKETBALL GUYS FOR
PHYSICALS
Congenital LQTS
 LQTS is the most common potentially lethal inheritable
arrythmia
 Prolonged QTc interval accompanies a history of syncope
 Jervell and Lange-Nielsen syndrome -Sensorineural
deafness – involves LQTS associated gene mutations in both
parents
 Predilection for cardiac events in adolescence
 A history of near-drowning is the symptom most
suggestive of prolonged QT syndrome on the
preparticipation questionnaire
Question 10
 How do you calculate the QTc interval?
QTc Interval
 Bazett Formula:
QTc=QT (Sec)/square root R-R (Sec)
 R-R interval should extend from the R wave in
the QRS complex you are measuring QT to the
preceding R wave.
Normal values:
 0.44 sec is 97th percentile for neonates 3-4 days
old.
 Less than or equal to 0.44 sec in children and
adults
Future Directions
 Standardizing history and physical forms
 Mandating the use of history and physical forms during
PPE
 Creating accreditation criteria for clinicians who
perform the PPE
 More widespread dissemination of AEDs, proven to be
effective in saving young lives, athletes and nonathletes alike
Take-home Points of Preparticipation
Screening
 The AHA recommends using a 14-element questionnaire (personal
Hx, family Hx, and PE) to screen young athletes for CV disease
 The most common cause of sudden cardiac death is hypertrophic
cardiomyopathy (HCM)
 Clinical features of HCM include dyspnea, syncope, pre-syncope,
exercise intolerance, angina and dizziness
 PE finding most suggestive of HCM is a systolic murmur accentuated
in the standing position
 Any of these findings, plus a FHX of HCM should prompt a referral to
cardiology
 Absolute contraindications to sports participation include HCM,
pulmonary vascular disease, LQTS, pulmonary HTN, severe Ao/MV
stenosis/regurgitation, coronary artery anomalies and others
Suggested reading
 Maron BJ, Friedman RA et al. Assessment of the 12-Lead ECG as a
Screening Test for Detection of Cardiovascular Disease in Healthy
General Populations of Young People (12-25 Years of Age): A
Scientific Statement From the American Heart Association and
the American College of Cardiology. Circulation. 2014; 1303-1334.
http://circ.ahajournals.org/content/130/15/1303.full
 Corrado D et al. Cardiovascular preparticipation
screening….Europ. Protocol…Eur Heart J 2005;26:516-524
 36th Bethesda Conference: Eligibility recommendations for
competitive athletes with cardiovascular abnormalities. J Am Coll
Cardiol 2005;45:1312-1375
Suggested reading
 Michael H Crawford. Screening athletes for heart
disease. Heart 2007;93:875-879
 Barry J Mason et al. Sudden deaths in young
competitive athletes. Analysis of 1866 Deaths in the
US, 1980-2006. Circulation 2009;119;1085-1092
 Hensley N, Dietrick J et al. Hypertrophic
Cardiolyopathy: A Review. Anestheisa-Analgesia.
March 2015. 120: 3.
 Cirino AL and Ho C. Hypertrophic Cardiomyopathy
Overview. GeneReviews. NCBI. Last update Jan 16,
2014.
 Behere SP, Shubkin CD and Wiendling SN. Recent
Advances in the Understanding and Management of
Long QT Syndrome. Curr. Opi. Ped. 2014, 26: 727-733