I have nothing to disclose. Research support from: Cardiac Risk in The Young

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1 I have nothing to disclose. Research support from: Cardiac Risk in The Young

2 Pre-participation screening of Young Athletes: Current Perspective Professor Sanjay Sharma Disclosures: None

3

4 SCD in Young Athletes Incidence approximately 1/50,000 Mean age at death in athletes 23 years-old 40% deaths in athletes aged < 18 years old More common in males than females (9:1) 90% deaths during or immediately after exertion

5 Causes of SCD in Sport Athletes Nonathletes SD/100,000 person yrs

6 Diagnosis Clinical and family history Cardiac auscultation 12-lead ECG/SAECG Echocardiography/CMR 24 hour ECG Exercise stress test Identify most conditions Pharmacological provocation tests Electrophysiological tests

7 Management Life style modification Pharmacological therapy Radiofrequency ablation Implantation of ICD Cardiac surgery

8 Pre-participation Screening Controversial Many antagonists Not funded by Government Discussions galvanised by a sudden death

9 Arguments For and Against Screening

10 Goals of Major Sporting Bodies The ultimate objective of the pre-participation screening of athletes is the detection of silent cardiovascular abnormalities that can lead to SCD. ACC 36 th Bethesda Conference, 2005 The main purpose of the consensus document is to reinforce the need for PPE medical clearance of all young athletes involved in organized sports programs to prevent athletic field fatalities - ESC Consensus Statement, 2005

11 Screening Athletes Condition History Examn ECG Echo HCM Pos/Neg Pos in 25% Positive Pos ARVC Pos/Neg Negative Positive Neg/Pos WPW Pos/Neg Negative Positive Neg LQTS Pos/Neg Negative Positive Neg Marfan Pos/Neg Positive Negative Pos CAA Pos/Neg Negative Negative Neg Myocarditis Pos/Neg Pos/Neg Pos/Neg Pos INCREASING COST

12 Screening Protocols

13 American Model Cheap. Easy to perform. Poor sensitivity and specificity Symptoms absent in almost 80% of athletic victims of sudden cardiac death Physical examination identifies few disorders capable of causing sudden death

14 Young competitive athlete Personal and family history Physical examination 12-lead rest ECG Negative findings Positive findings Eligibility for competition No cardiovascular disease Further examination Cardiovascular disease Management according to established protocols

15 Role of ECGs in Diagnosis of Cardiomyopathy HCM ARVC 95% 80%

16 Screening for HCM in Young Athletes Screening for HCM in Young Athletes Corrado NEJM 1998 Italy (Veneto region) ,735 competitive athletes were screened. 621 (1.8% of total) disqualified due to cardiac disorders due to cardiac disorders. Cardiac rhythm disorders 38.3% Hypertension 27.0% Valvular heart disease 21.4% HCM 3.0%

17 22 cases of HCM:- Diagnosis of HCM based on family history and murmur in 23% Abnormal ECG in 80%. Mean wall thickness 19 3 (mm); mn All disqualified from sport Diagnosis of HCM No deaths during follow-up period

18 510 athletes 11 with confirmed abnormalities H and examination identified 5 out of 11 Addition of ECG increased yield to 11 out of 11 Sensitivity 91%. Specificity 83%. Negative predictive accuracy 99.8% Sensitivity with H and E alone (45%)

19 Athletes cleared at national screening 4397 (98.8%) No cardiac diseases Efficacy of Italian ECG Programme for 37 (0.8%) Physiological LVH Excluding HCM 4450 Echocardiography (and other testing) 41 (0.9%) LVH 4 (0.1%) Grey zone NEGATIVE PREDICTIVE VALUE 99.8% Other structural disease 12 (0.3%) 1 HCM (0.025%)

20 114 asymptomatic individuals with HCM Mean age 22 ± 8. 72% male 2.6% Black 11 (10%) had a normal ECG. Mean LVWT 17 ± 2 mm Of the 11, 7 had a FH of HCM and 4 had a detectable murmur

21 Screening Athletes: Impact on SCD ,386 athletes (12-35 years) History, examination and 12-lead ECG Patient with abnormal findings investigated further Compared death rates pre-screening early screening late screening Death rates fell from 3.6/100,000/person years (prescreening to 0.4/100,000/person years following screening Reduction in deaths mainly from cardiomyopathies

22 TIME-TREND OF SUDDEN CARDIAC DEATH INCIDENCE IN ATHLETES VS NON-ATHLETES Veneto Region of Italy

23 Athletes Disqualified with ARVC Number 16 Early screening 2 Late screening 14 Age: 21 7 Sex (% Male) 94 Positive findings: FH (%) 12.5 ECG changes (%) 87.5 Ventricular arrhythmias (%) 62

24 Young competitive athlete Italian Model Personal and family history Physical examination Endorsed 12-lead rest ECG by Negative findings Eligibility for competition - ESC No cardiovascular disease - IOC Positive findings Further examination Cardiovascular disease - FIFA Management according to established protocols

25

26 Comparison of Italian and US sudden deaths rates Fact is: No changes in US athletes screened with PE+Hx Corrado et al. JAMA 2006; 296: Maron et al. Am J Cardiol 2009; in press

27

28 Concerns Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

29 Concerns Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

30 Goals of Major Sporting Bodies The ultimate objective of the pre-participation screening of athletes is the detection of silent cardiovascular abnormalities that can lead to SCD. ACC 36 th Bethesda Conference, 2005 The main purpose of the consensus document is to reinforce the need for PPE medical clearance of all young athletes involved in organized sports programs to prevent athletic field fatalities - ESC Consensus Statement, 2005

31 Goals of Major Sporting Bodies The ultimate objective of the pre-participation screening of athletes is the detection of silent cardiovascular abnormalities that can lead to SCD. ACC 36 th Bethesda Conference, 2005 The main purpose of the consensus document is to reinforce the need for PPE medical clearance of all young athletes involved in organized sports programs to prevent athletic field fatalities - ESC Consensus Statement, 2005

32 Prevalence of Cardiovascular Disorders at Risk of SCD Ref: Population Prevalence AHA (2007) Competitive athletes (U.S.) 0.3% Fuller (1997) 5,617 high school athletes (U.S) 0.4% Corrado (2006) 42,386 athletes age (Italy) 0.2% Wilson (2008) 2,720 athletes /children age (U.K.) 0.3% Bessem (2009) 428 athletes age (Netherlands) 0.7% Baggish (2010) 510 collegiate athletes (U.S.) 0.6%

33 Concerns Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

34 Athlete s ECG

35

36 Results of athletes screened in Veneto Corrado; JAMA 2006 Athletes screened: 42,386 Abnormal ECG: 3,914 (9%) Cardiac disorder: 879 (2%) All disqualified False Positive 7% Potentially lethal disorder: 91 (0.2%)

37 The Challenge Physiology Pathology Left Ventricular Hypertrophy Repolarisation anomalies

38 Diagnosis Clinical and family history Cardiac auscultation 12-lead ECG Echocardiography 24 hour ECG Exercise stress test Pharmacological tests Electrophysiological tests Familial Relatively rare Heterogeneous phenotypic manifestations Symptoms of disease usually absent ECG overlap with athlete s heart Evaluation in an expert setting is important

39 Concerns Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

40 Deaths in Athletes and Non-Athletes Aged 35 Years in Veneto Corrado; NEJM 1988 Deaths in Athletes and Non-Athletes Aged 35 Years in Veneto Corrado; NEJM 1988 CONDITION ATHLETES N = 49 NON-ATHLETES N = 220 TOTAL N = 269 CAD 9 (18.4) 36 (16.4) 45 (16.7) CAA 6 (12.2) 1 (0.5) 7 (2.6) HCM 1 (2) 16 (7.3) 17 (6.3)

41 Deaths Despite Screening with ECG False Negatives Anomalous coronary arteries Premature atherosclerotic coronary disease Acquired conditions Commotio cordis Myocarditis Electrolyte disorders Incomplete expression of cardiomyopathy and ion channel disease

42 Concerns Low incidence of sudden cardiac death High number of false positives Concerns relating to false negatives Cost Other issues

43 H/ E alone H/ E/ ECG Life years saved 0.56/ /1000 Incremental cost $133 $199 Projected cost $410,000,000 $736,000,000 Cost effective ratio $190,000 $76,000 QALY $301,000 $111,000

44 Concerns Low incidence of sudden cardiac death High number of false postives Concerns relating to false negatives Cost Other issues

45 WHO to screen? WHEN to screen? HOW often to screen? Screening Athletes WHICH screening protocol? WHAT is abnormal in an athlete? WHO will screen? WHO will pay for screening? WHERE is the infrastructure, personnel and expertise? WHO will manage the athlete with a diagnosis?

46 Conclusions 1. Screening with ECG identifies serious cardiac disorders and prevents SCD. 2. Screening with ECG will fail to identify most coronary artery abnormalities/disease. 3. Screening of athletes MUST take place in an EXPERT setting. 4. Most Governments are not in a position to deliver a de novo national screening programme.

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