I recently encountered two young athletes at our Sports Cardiology Center who exemplified some of the challenges with screening young athletes for underlying cardiac conditions. One was an 18-year-old football player, 220 lbs and muscular, who was diagnosed with a bicuspid aortic valve and an aortic root measuring 4.1cm after a soft systolic murmur was discovered during a pre-participation physical. He was advised to stop playing football, which would result in a loss of college funding. He was also advised to not lift more that 20lbs and not even lift his hands over his head! While there is a small risk of dissection with a dilated aorta, these recommendations were, in my view, excessive without a reasonable basis in data or recent guidelines.¹ They also terrified this young man. The second athlete was a 19 year old with aspirations to represent the United States at the Olympics in an endurance event. A screening ECG was normal except for left axis deviation. While not supported by any known guidelines, he proceeded to a maximal effort exercise stress test where his systolic blood pressure increased to >200mmHg. He was advised that he was in "immediate danger of a stroke" if he continued to exercise and was given a strict heart rate limit, which he should not exceed. He was also commenced on a beta-blocker. In my opinion, there were many errors in this sequence of events. Firstly, left axis deviation in isolation does not require any downstream testing according to the most recent recommendations for the interpretation of electrocardiograms in athletes and a stress test would not be the most rational next test in an asymptomatic athlete. The results of the stress test were then misinterpreted resulting in inappropriate disqualification and introduction of medical therapy, which was likely to result in significant side effects for a young athlete. Many studies have shown that it is not uncommon for young athletes, particularly endurance athletes with the ability to dramatically increase their cardiac output with exercise, to mount systolic blood pressures in excess of 200mmHg systolic with exercise.² It was a challenge in this instance to convince the young man that there was nothing wrong with him and he could safely return to sport without restriction. Unfortunately, in his case, the 'damage' had already been done to his career and confidence.

I have seen multiple examples of completely inappropriate restrictions placed on athletes by physicians. The reality is that most cardiologists do not want to deal with sports eligibility issues. They do not wish to accept the risks of adverse events in young, otherwise fit individuals and have not had training or experience in dealing with disqualification or eligibility issues. Furthermore, the price of a mistake is extraordinarily high. The deaths of young athletes have a profound effect on society and have resonated throughout the ages. Take the death of young Pheidippides – after announcing the victory of the Athenians over the Spartans, he reportedly dropped dead. He had just run 26.2 miles to delivery this news – the Marathon is run to this day in his honor. The cases of Hank Gathers and Reggie Lewis and countless others loom large in our collective memory. The treating cardiologists are, justifiably or not, tarnished with the assumption of poor management. All physicians, at one time or another, have made mistakes that will often haunt them throughout their careers. Few will have their presumed mistake so publicly broadcast as those who treat a well-known athlete who dies suddenly.

Eligibility decisions are often driven more by these emotions rather than a reasonable risk assessment. If the risk of a young person dying while driving a car is higher than participating in sport, why would we let them drive a car and not play their sport?

For those who are not passionate athletes, it is easy to not appreciate the importance athletes place on their sport. For many, their sport defines who they are. They have put in countless hours of training. Their parents have made huge sacrifices throughout their life to facilitate their passion. Their inherent self-image is as the football captain, the endurance runner, or the bodybuilder. For others, it is a ticket to a better life – college scholarships or even professional careers. They are dependent on their sport for their education, financial security, and some hope for fame and adulation. Many have built their entire social structure around their clubs and teammates. For the majority, sport is a release, a place to get away from stress and a means of maintaining an equilibrium in life. Remove that crutch and the results are often catastrophic.

For the physician faced with a question of sport participation in an athlete with heart disease, the focus is more often on the risk of competition and little thought goes into the risk of disqualification. I have seen teenagers, who were the brightest and best, sink into deep depression, become socially isolated from friends and family, gain enormous amounts of weight, and face sedentary lives with all of the resultant adverse effects associated with obesity and inactivity. I have seen young people's chance at a better life, because of professional careers or college scholarships, abruptly taken away based on small theoretical risks. I have seen bodybuilders reduced to tears and others turn to substance abuse. Most tragically, I had one bright young man attempt suicide after he was disqualified from football. They will uniformly tell me that their physician did an admiral job in making the diagnosis of their condition and instituted an appropriate medical management plan, but a lousy job of explaining what the implications of their diagnosis were on their sport. Usually, they are just told "don't do that anymore".

While many of these are athletes have been given overly stringent restrictions, some are athletes that I have had to disqualify myself. Unfortunately, there are situations where continuing to play is associated with a prohibitively high risk, or has been shown to accelerate the disease process and ultimately death. In these situations, I will adamantly advise against continued play. These are long consultations where family members should be present. We discuss at length the rationale behind this, we also discuss alternatives that will keep them involved in their sport and social network, such as helping with coaching, etc. This is not a one-off conversation and will often require a number of follow up consultations. I work closely with a psychologist who is available to provide support. Ultimately, the decision to disqualify should never be taken lightly.

It takes courage and conviction to permit an athlete to participate when there is a risk in what they are doing. But people are willing to take risks all the time. Extreme sports such as base jumping, big wave riding, and mountaineering are very high-risk with much higher mortality rates than most of the sports we restrict athletes from playing with cardiac disorders. What would the public reaction be if the physician general announced that all of these sports are now illegal?

Is it the treating physician's role to decide the risks others take? Obviously, some people are more risk adverse than others and so long as the risk is explained and every means of lowering that risk is taken, including the development of a clear emergency action plan, it is first up to the individual to decide whether or not to play. This concept is referred to as a "shared decision-making model" and should be instituted when reasonable.³

Thankfully, the most recent iteration of the recommendations for eligibility and disqualification of athletes with heart disease to participate in competitive sports is no longer as dogmatic and paternalistic as in the past.⁴ The guidelines recognize the role of the individual athlete to participate in the decision to continue to play sports. I am frequently surprised by athletes, whom I privately assume will be willing to accept any risk to continue to play, make the decision to stop competing themselves. As physicians, it is not our role to anticipate the decision an athlete might take, but rather to provide them with the best information regarding their individual risk of continuing to compete. We must address these risks, but counterbalance them against the risks of long-term adverse psychological, social, financial and indeed health risks of disqualification.

References

1. Braverman AC, Harris KM, Kovacs RJ, Maron BJ. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: Task Force 7: aortic diseases, including Marfan syndrome: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2398-2405.
2. Caselli S, Vaquer Segui A, Quattrini F, et al. Upper normal values of blood pressure response to exercise in Olympic athletes. Am Heart J 2016;177:120-28.
3. Baggish AL, Ackerman MJ, Lampert R. Competitive sport participation among athletes with heart disease: a call for a paradigm shift in decision making. Circulation 2017;136:1569-71.
4. Pelliccia A, Solberg EE, Papadakis M, et al. Recommendations for participation in competitive and leisure time sport in athletes with cardiomyopathies, myocarditis, and pericarditis: position statement of the Sport Cardiology Section of the European Association of Preventive Cardiology (EAPC). Eur Heart J 2019;40:19-33.

Keywords: Sports, Athletes, Exercise Test, Heart Rate, Aortic Valve, Blood Pressure, Fellowships and Scholarships, Systolic Murmurs, Follow-Up Studies, Mentors, Heart Valve Diseases, Electrocardiography, Risk Assessment, Cardiac Output, Stroke, Decision Making, Social Support, Aorta

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