Last night Dallas Stars Center Rich Peverly collapsed on the bench early in a game against Columbus and was rushed through a tunnel and stabilized. The 31-year-old Peverley ended up in good condition at a Dallas hospital. (Read full story here).

This is reminiscent of Detroit Red Wings player Jiri Fisher collapsing and being revived a few years ago and also brings to mind the tragic death of a young athlete, New York Rangers prospect Alexei Cherapanov.

Sadly this happens with some regularity and in many cases can be prevented. FOR MORE INFORMATION VISIT THE CANADIAN SADS FOUNDATION WEBSITE. It was great to see the deployment of an AED at the Stars game, the use of which likely saved Rich Peverly’s life.

Listen to my interview on TSN Radio 1050 here.

To help build awareness and to guide action in Canada and around the world, our research group at the Hospital for Sick Children in Toronto has recently compiled a list of recommendations for athletes to get screened for cardiac problems. Although there is a very small risk, and the screening procedures are not perfect (they don’t catch everything, and sometimes there are false positives), we do recommend that athletes get screened.

Here is our analysis and recommendations, and our contact information is at the bottom if you have any questions.

Cardiovascular Screening of Canadian Athletes for Prevention of Sudden Death
Review, Rationale & Recommendations

Prepared by: Greg D. Wells, Ph.D., Maryam Elmi, Robert Hamilton M.D.

October 13, 2008

Executive Summary

Objective
The purpose of this document is to provide background information on sudden cardiac death in athletes, to outline recommendations for pre-participation screening, and to summarize cost-benefit projections related to implementation of a screening program in Canada.

Significance
The current media interest in the issue of sudden cardiac death in athletes, a large and easily accessible athlete population in Canada, and the cardiovascular knowledge and expertise at the Hospital for Sick Children provides us with a unique opportunity to establish new screening recommendations and policy in Canada that has the potential to optimize the health of Canadian Athletes.

Recommendations
We suggest a three step pre-participation screening procedure for Canadian athletes: 1) a personal history, 2) a family history, and 3) a physical exam including 12-lead ECG. Selected cases with positive personal history, family history of potentially inherited cardiac disease, or positive physical or ECG findings should be evaluated by an appropriate cardiac specialist to qualify the athletes for sport participation.

Background
The sudden death of an athlete is a tragic event that has great impact on the family, the community, and the medical establishment (Basilico, 1999). There is a long history of sudden cardiac death in athletes dating back to the 5th Century B.C. The Greek herald named Pheidippides ran 26 miles from Marathon to Athens to announce a Greek military victory and died on the spot (Rich, 1994). There have been reports of this catastrophic event in modern times as well including Olympic gold medal skater Sergei Grinkov in 1995, professional basketball player Reggie Lewis in 1993, college basketball player Hank Gathers in 1990, Olympic volleyball champion Flo Hyman in 1986 (Drezner, 2000). Most recently, Alem Techale, Ethiopia’s 1,500 metre star, collapsed and died during a training run in early 2005. While secondary prevention such as automatic external defibrillator (AED) deployment may save some athletes, as in the case of Jiri Fischer (National Review of Medicine 3(1), Jan 16, 2006 Are Sports Bad for the Ticker), thus far survival has been less than expected among attempted resuscitations in intercollegiate athletes. (Heart Rhythm. 2006 Jul;3(7):755-9. Epub 2006 Mar 28.)

These rare events usually assume a high public profile given the perception that trained athletes are physically fit and able to tolerate extremes of physical endurance. In 1996, the American Heart Association consensus panel recommendations stated that pre-participation screening for young competitive athletes is recommended on ethical, legal, and moral grounds (Maron, 1996). In 2005, The European Society of Cardiology published a consensus document that recommends a common European screening protocol and reinforced the principle of the need for screening of young athletes who participate in organized sports programs (Corrado, 2005). Currently, there are no formal recommendations for pre-participation cardiovascular screening for Canadian athletes nor are any normative data available on the rates of cardiovascular risk factors in Canadian elite athletes.

Therefore, this document is to:
1.    To provide background information and a rationale for the implementation of a cardiovascular pre-participation screening of Canadian athletes.
2.    To report cost-effectiveness studies from other countries.
3.    To suggest recommendations for cardiovascular pre-participation screening of Canadian athletes.

The current media interest in the issue of sudden cardiac death in athletes, a large and easily accessible athlete population in Canada, and the cardiovascular knowledge and expertise at the Hospital for Sick Children provides us with a unique opportunity to establish new screening recommendations and policy in Canada that has the potential to optimize the health of Canadian Athletes. The results of the proposed cardiovascular testing will provide new information that can be used immediately to monitor the health of athletes in Canada and also will provide a foundation for future cardiovascular testing recommendations for not only elite athletes, but for young Canadians who are involved in organized physical activities at all levels.

Definition
Sudden cardiac death in an athlete has been defined as nontraumatic and unexpected sudden cardiac arrest that occurs within 6 hours of a previously normal state of health (Maron, 1983). Alternatively, Vancamp et al. (1995) defined it as death after symptoms that begin during or within one hour after participation on a high school athletic team, in which death was determined to be cardiac in nature. In athletes less than 35 years of age, congenital or inherited cardiovascular diseases are usually responsible (Drezner, 2000). This catastrophic event typically occurs during or shortly after training or competition, suggesting that intense physical exertion is a precipitating factor (Maron, 1996).

Prevalence
The prevalence of recognized cardiovascular disease in the young athletic population is low. However, sudden death due to heart attack remains the leading cause of non-traumatic death in athletes (Maron, 2005). Some reports have suggested an incidence of 1 in 100,000 athletes between the ages of 12 and 35 (Beckerman, 2004) although it is possible that these numbers are under-reported as there are no national databases in the United States or Canada that track sudden cardiac death in young athletes. Other research has reported more specific prevalence rates for cardiovascular disease in the athletic population including:
•    The prevalence of Hypertrophic Cardiomyopathy is between 1/500 and 1/1000 (Maron, 1995)
•    The prevalence of Long QT Syndrome is between 1/3000 and 1/5000 (Zareba, 2005)
•    The prevalence of Arrhythmogenic Right Ventricular Cardiomyopathy is at least 1/5000 (Frances, 2005) and possibly as high as 1/1000 (Peters, 2004)
•    The prevalence of Marfan Syndrome is between 1/10,000 and 1/5000 (Porciani et al., 2004)
•    The prevalence of Wolff-Parkinson-White Syndrome is between 1.5/1000 and 2.5/1000, (Singh, 2004) with 10 to 30 % of these pre-excited individuals going on to develop tachycardia, depending on the duration of follow-up (Conti, 2001)
Additionally, it is now suggested that children with asymptomatic pre-excitation may be at risk for lethal cardiac arrhythmias (Pappone, 2004). Interestingly, if the higher estimates for all diseases are added, the overall prevalence of risk in the population may be as high as 1/166! Fortunately, the most prevalent conditions listed above are largely detectable by the screening protocols that are presented later in this document. The diseases that have been identified above as risk factors for sudden cardiac death are described in more detail in the following section.

Specific Conditions associated with Sudden Cardiac Death in Athletes

Hypertrophic Cardiomyopathy (HCM): Being the leading cause of sudden cardiac death in young athletes, HCM is characterized by the enlargement of the cardiac muscle. In HCM patients, the aberrant alignment of the cardiac muscle cells (myocardial disarray) disrupts the heart’s electrical function. HCM is attributed to a mutation in the alpha or beta myosin heavy chain chromosome, which is most commonly inherited in an autosomal dominant pattern.

Long QT Syndrome (LQTS): LQTS is a heart condition in which there is an abnormally long delay between the depolarization and repolarization of the cardiac ventricles. This delay is associated with ventricular fibrillation which is the cause of sudden death in individuals with LQTS. The most common causes of LQTS are genetic (inherited in an autosomal dominant or autosomal recessive fashion) and drug-induced.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): ARVC is a heart condition manifested in the right ventricle in which the myocardium is replaced by fibrofatty tissue, starting in the subepicardial region, progressing to the endocardial surface and to the trabeculae of the right ventricle. The fibrofatty infiltration of the right ventricle is associated with myocardial atrophy due to apoptosis of myocytes. As a result, ventricular arrhythmias due to ARVD may lead to sudden cardiac death. The cause of ARVC is predominantly genetic, usually inherited in an autosomal dominant pattern.

Wolff-Parkinson-White Syndrome (WPW): WPW is a heart diseased marked by a pre-excitation of the heart ventricles due to the presence of the Bundle of Kent, an accessory electrical conduction pathway between the atria and the ventricles. The Bundle of Kent, being absent of extrinsic regulations, may lead to tachyarrhythmias and, consequently, ventricular fibrillation. This ventricular fibrillation, if not immediately corrected, is the cause of sudden cardiac death associated with WPW.

Marfan Syndrome: Marfan syndrome is an autosomal dominant connective tissue disorder characterized by unusually long limbs. Marfan symdrome is also manifested in the cardiovascular system, causing the leakage of the mitral or aortic valves. Marfan Syndrome carries the risk of aortic dissection or rupture, thus associated with sudden cardiac death. Although not a “red herring”, Marfan syndrome comes with a fairly obvious phenotype on physical examination.

Recommendations for Pre-participation Cardiovascular Screening

Background
Several major international organizations have presented recommendations for pre-participation cardiovascular screening for athletes; a) the American Heart Association, b) the International Olympic Committee in 2004, and c) the European Society of Cardiology. Given the similarity between the Canadian population, American and European populations, it is suggested here that the above recommendations be adopted for Canadian athletes. The specific recommendations from each organization are similar and can be summarized as presented in the “Lausanne Recommendations” from the International Olympic Committee Medical Commission:

Screening
Screening should be conducted for all participants in competitive activities from ages 12-35. There are three steps that make up the recommended screening procedure. They are as follows:

Step 1: Personal History
A personal history questionnaire by an examining physician to identify potentially detectable cardiovascular conditions to be administered at the beginning of competitive activities. The recommended questions are:
•    Have you ever fainted or passed out while exercising?
•    Have you ever had chest tightness?
•    Does running ever cause chest tightness?
•    Have you ever had chest tightness, cough, wheezing which made it difficult for you to participate in sports?
•    Have you ever had a seizure?
•    Have you ever been told you have epilepsy?
•    Have you ever been told to give up sports because of health problems?
•    Have you ever been told you have high blood pressure?
•    Have you ever been told you have high cholesterol?
•    Do you have trouble breathing or do your cough during or after activity?
•    Have you ever been dizzy during or after exercise?
•    Have you ever had chest pain during or after exercise?
•    Do you have or have you ever had racing of your heart or skipped heart beats?
•    Do you get tired more quickly than your friends do during exercise?
•    Have you ever been told you have a heart murmur?
•    Have you ever been told you have a heart arrhythmia?
•    Do you have any other history of heart problems?
•    Have you had a severe viral infection (for example myocarditis or mononucleosis in the last month)?
•    Do you have any allergies?
•    Have you routinely taken any medication in the past 2 years?

Step 2: Family History
The recommendations described here should be repeated every second year while participating in competitive activities. There are 3 parts to the family history examination, a general history, a physical exam, and diagnostic tests.
Part A: Family History: Questionnaire administered by examining physician.
•    Has anyone in your family less than 50 years old:
•    Died suddenly and unexpectedly?
•    Been treated for recurrent faining?
•    Had unexplained seizure problems?
•    Had unexplained drowning while swimming?
•    Had unexplained car accident?
•    Had heart transplantation?
•    Had a pacemaker or defibrillator implanted?
•    Been treated for an irregular heart beat?
•    Had heart surgery?
•    Experienced sudden infant death syndrome?
•    Been told they have Marfan syndrome?

These questions are designed to detect a) inherited cardiomyopathy such as hypertophic arrythmogenic RV (dilated), b) inherited heart rhythm problems such as cardiac ion channel diseases (long ans short QT syndrome, Brugada syndrome, Lenegre disease, catecholaminergic polymorphic VT, and c) connective tissue disorders.

Part B: Physical examination by physician including:
1. General:
•    Radial and femoral pulses
•    Marfan stigmata
2. Cardiac auscultation
•    Rate / rhythm
•    Murmur: systolic and diastolic
•    Systolic click
3. Blood pressure

Part C: Diagnostic test:

1. 12-lead ECG (after the onset of puberty). This test is to detect rhythm, conduction or re-polarization abnormalities.

Step 3: Positive Cases
Selected cases with positive personal history, family history of potentially inherited cardiac disease, or positive physical or ECG findings in Step 1 or 2 will require further evaluation by an appropriate cardiac specialist to qualify the athletes for sport participation. Further evaluation may include:
•    Trans-thoracic echocardiography
•    Maximal exercise testing
•    24-hour ECG monitoring
•    Exercise Protocols

Ethical Considerations
Several legal considerations must be taken into account when testing athletes for cardiovascular disease due to the special nature of the coach-athlete relationship and the potential pressure to participate in the sport. These considerations include:
•    Prior to the testing, the athlete (and parents or legal guardian if a minor) are entitled to receive proper information on the nature and objectives of the test, and on the medical consequences in case of positive results, in particular the need for further investigation, as well as preventive or therapeutic measures.
•    Special care should be taken to protect the privacy of the athlete. The results should be transmitted solely to the athlete and his / her legal guardians.
•    In the case of a positive test, subsequent investigations and testing must be administered as quickly as possible to minimize the potential impact of being withheld from practice and competition pending the results of additional testing.

References

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