1. Cardiovascular Preparticipation Sports Screening Are we doing enough?
Sanjay Lambore, MD, MSc, FAAP, May 2012
Updated by Ana Malinow, MD, March 2015

2. 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

3. 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.

4. 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?

5. 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

6. Medical History Physical Examination Personal history Family history
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

7. 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:

8. 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?

9. 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?

10. Question 2
What are the limitations to screening for cardiovascular pathology at the time of sports physical?

11. 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

12. The Italian (Veneto) experience
Since 1982, all athletes in Italy 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 to recommend national screening in EU and US

13. 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

14. 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

15. 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

16. Question 3 What is the incidence of sudden cardiac death in athletes?
What is the magnitude of the problem?

17. 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
-marathon runners = 1:50,000

18. 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.

19. 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

20. Question 4
What are the absolute cardiac contraindications to sports participation according to the th Bethesda Conference?

21. 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)
ARVC= arrhythmogenic right ventricular cardiomyopathy

22. 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

23. Question 5 What are the causes of sudden cardiac death in athletes?
What is the most common mechanism of death?

24. 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

25. 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

26. 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

27. Question 6
How prevalent is hypertrophic cardiomyopathy and what are the presenting symptoms?

28. Hypertrophic Cardiomyopathy
Most common cause of sudden cardiac death
Autosomal dominant inheritance with more than 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

29. Hypertrophic Cardiomyopathy contd.
Clinical features which may raise suspicion include:
-Family history of HCM
-Symptoms of left ventricular outflow obstruction
-A heart murmur

30. 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

31. 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

32. 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

33. 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)

34. 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

35. 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?

36. 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

37. Question 7
What is the “Athlete’s Heart” ?

38. 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

39. 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

40. Question 8
What is the second most common cause of sudden cardiac death in athletes?

41. 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

42. 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

43. Question 9
List the clinical findings of Marfan syndrome.

44. 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

45. Congenital LQTS Predilection for cardiac events in adolescence
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

46. Question 10
How do you calculate the QTc interval?

47. 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

48. 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 non- athletes alike

49. 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

50. 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;
Corrado D et al. Cardiovascular preparticipation screening….Europ. Protocol…Eur Heart J 2005;26:
36th Bethesda Conference: Eligibility recommendations for competitive athletes with cardiovascular abnormalities. J Am Coll Cardiol 2005;45:

51. Suggested reading
Michael H Crawford. Screening athletes for heart disease. Heart 2007;93:
Barry J Mason et al. Sudden deaths in young competitive athletes. Analysis of 1866 Deaths in the US, Circulation 2009;119;
Hensley N, Dietrick J et al. Hypertrophic Cardiolyopathy: A Review. Anestheisa-Analgesia. March : 3.
Cirino AL and Ho C. Hypertrophic Cardiomyopathy Overview. GeneReviews. NCBI. Last update Jan 16,
Behere SP, Shubkin CD and Wiendling SN. Recent Advances in the Understanding and Management of Long QT Syndrome. Curr. Opi. Ped. 2014, 26:

52. Thank You !