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Shared decision-making critical for athletic participation by patients with heart disease

The decision process behind sports participation for patients with heart disease has changed over time. Previously, a decision was primarily made on strict formulas that often recommended against participation. However, in recent years, new research has suggested that sports participation by patients with heart disease may be acceptable in some cases that were previously considered too risky, and the ways in which decisions are being made are also changing. What is less clear is whether ECG screenings are necessary before allowing presumably healthy young athletes to play.

“The classic paradigm of sports participation was very paternalistic,” Michael S. Emery, MD, assistant professor of clinical medicine at Indiana University School of Medicine, Indianapolis, told Cardiology Today. “It was very much along the lines of, ‘We, in general, don’t recommend you participate in intense athletics’. We often restricted patients with heart disease to participate in low-intensity competitive athletics. Classic was golf and curling if you had heart disease.”

However, new research and greater understanding of the risks and benefits of athletic participation in this population has paved the way for increased support for allowing participation in more cases, particularly if the patient is monitored closely.

One important trend is a movement toward shared decision-making, which involves not only the health care professional and the patient, but also coaches, teams, schools and, depending on age, parents and caregivers.

Rachel J. Lampert, MD, from Yale University School of Medicine, said decision-making about athletic participation by patients with heart disease has become more individualized as risks have become better understood.
Rachel J. Lampert, MD, from Yale University School of Medicine, said decision-making about athletic participation by patients with heart disease has become more individualized as risks have become better understood.

Source: John Curtis/Yale Medicine Office of Communications. Reprinted with permission

This paradigm shift to shared decision-making was a major focus in a paper published in October in Circulation by Aaron L. Baggish, MD, FACC, FACSM, associate professor of medicine at Harvard Medical School and director of the Cardiovascular Performance Program at Massachusetts General Hospital; and Michael J. Ackerman, MD, PhD, professor of medicine, pediatrics and pharmacology and director of the Long QT Syndrome/Genetic Heart Rhythm Clinic and the Windland Smith Rice Sudden Death Genomics Laboratory at the Mayo Clinic in Rochester, Minnesota; and Rachel J. Lampert, MD, professor of internal medicine (cardiology) at Yale University School of Medicine.

“These medical and legal uncertainties represent opportunities, not obstacles,” Lampert, Ackerman and Baggish wrote. “The time has come to acknowledge with full transparency that sport participation [for] athletes with CVD is a complex clinical topic that remains shrouded by persistent scientific and clinical uncertainty. Simplified ‘yes’ vs. ‘no’ decisions are almost always suboptimal, particularly when made through the lens of paternalism.”

Traditional counseling

Decisions regarding participation or disqualification in sports had been predominantly based on recommendations from the 2005 Bethesda guidelines for sports eligibility, which focused on athletes of all ages. Those guidelines listed each heart disease, from systemic hypertension to hypertrophic cardiomyopathy, and a “yes” or “no” answer as to whether a patient can return to play after they have already been diagnosed with a particular CVD, and a health care professional would then consult them accordingly. The recommendations featured in the document were based on expert opinion, current knowledge about disease processes in nonathletic individuals and available evidence from limited registries on sudden cardiac death in sports.

Until several years ago, if there was uncertainty about whether to disqualify a patient from sports activity, it was common for the patient to not return to play. Regardless of whether the cardiologist had doubt, the decision was traditionally approached in a conservative, risk-averse manner. Risks include sudden cardiac death from conditions such as long QT syndrome and hypertrophic cardiomyopathy, though it is important to distinguish nonpathological changes in cardiac morphology associated with athletic training from cardiac diseases.

Dermot M.J. Phelan, MD, PhD, FACC
Dermot M.J. Phelan

“Doctors have traditionally focused on the disease process and the management of the disease. They’ve done an excellent job doing that, but they have not focused at all on the impact of the disease on athletic performance,” Dermot M.J. Phelan, MD, PhD, FACC, staff cardiologist in the section of cardiovascular imaging in the Robert and Suzanne Tomsich Department of Cardiovascular Medicine at Cleveland Clinic; medical director of the Sports Cardiology Center in the Sydell and Arnold Miller Family Heart and Vascular Institute at Cleveland Clinic; and assistant professor at the Cleveland Clinic Lerner College of Medicine at Case Western Reserve University, told Cardiology Today. “There may be a risk involved with permitting a young athlete with heart disease to participate in sports, and if something bad happens to them, then ... the blame is often laid at the doctor’s feet. Traditionally, doctors have not wanted to take on that risk and as a result have been very conservative.”

Updated guidelines

Since the 2005 Bethesda guidelines, updated guidelines have been released, including the 2015 scientific statement by the American Heart Association and the American College of Cardiology. The AHA/ACC scientific statement provided a more tailored approach to deciding whether patients should participate in sports or not based on their CV condition and its severity (see Sidebar below).

The idea of participation in patients who are asymptomatic even in the presence of a CV condition for some conditions was introduced in this set of guidelines. In this patient population, health care professionals were encouraged to counsel on the risks and benefits of continued participation and what to be aware of if symptoms begin to worsen.

The 2015 scientific statement also provided each recommendation within a class and level structure based on whether a patient could be considered to return to play or be disqualified.

“The inclusion of class II recommendations really changes the landscape of counseling and decision-making because now it’s not binary — ‘yes’ or ‘no’, ‘you’re in’ or ‘you’re out’ — but rather it’s now really a decision that needs to be considered,” Lampert told Cardiology Today.

The focus on arrhythmias and other CV conditions in guidelines for the treatment of athletes stems from the awareness of sudden cardiac death, although its incidence has not increased over the past 10 years and some would argue that it is rare. According to a database analysis published in the Mayo Clinic Proceedings in 2016, sudden cardiac death occurs between 1 in 43,000 athlete-years and 67,000 athlete-years in college athletes; for high school athletes, sudden cardiac death occurs between 1 in 46,000 athlete-years and 919,000 athlete-years. The rate of sudden cardiac death varies from 1 in 3,000 athlete-years in NCAA Division I male basketball athletes to 1 in 917,000 athlete-years in high school athletes in Minnesota, according to an analysis published in Circulation in 2015. This has led health care professionals to concentrate more on the athlete and ensure that the screening process is done properly and to enforce a shared decision-making approach.

Updates to athletic participation guidelines
Sources: Baggish AL, et al. Circulation. 2017;in press.
Maron BJ, et al. J Am Coll Cardiol. 2015;doi:10.1016/j.jacc.2015.09.032.

Debate over ECG screening

The question of whether to perform an ECG for preparticipation screening has been a focus of ongoing debate among sports cardiologists. The AHA and ACC do not currently recommend universal ECG screening, whereas the European Society for Cardiology strongly supports screening. Available research is also conflicting. A study from Italy published in JAMA in 2006 showed that instituting ECG screenings decreased the number of young athletes who died, but a study in Israel published in the Journal of the American College of Cardiology in 2011 did not show a change in overall mortality.

In a recent study published in the Journal of the American Heart Association, Harshil Dhutia, BSc, from the cardiology clinical and academic group of St. George’s University of London, and colleagues used the new international criteria, which reduced the number of ECGs that were originally read as abnormal.

Health care professionals who favor ECG screenings generally understand that the data aren’t perfect, but support that screening improves the yield of CV diagnoses in the absence of physical findings and sometimes unreliable family histories.

Although ECG screening may be helpful, a number of changes are needed to properly utilize the screening method, according to some experts. ECGs require careful interpretation, because athletes have different ECG patterns than older adults. An educated workforce can prevent false positives and negatives.

In a debate on ECG screening in athletes at the American College of Cardiology Scientific Session in March, Francesco Fedele, MD, professor in the department of cardiovascular, respiratory, nephrology, anesthesia and geriatric science at Sapienza University of Rome and president of the Italian Federation of Cardiology, said according to one study, adding ECGs to the family history and physical examination portions of screening “would save 2.06 life-years per 1,000 young athletes.”

“The benefit of screening goes beyond the identification of the student’s risk of [sudden cardiac death] because it often triggers the evaluation of first-degree relatives who may also be at risk from inherited cardiac diseases.” Fedele said. “Cardiac screening with ECG has the potential of saving additional lives.”

During that same ACC session, Bryan C. Cannon, MD, FACC, FHRS, pediatric cardiology fellowship director at Mayo Clinic, said during a presentation on the disadvantages of ECG screening that while the cost of saving per human life cannot be valued, “there are too many issues right now to implement universal ECG screening.”

ECG screening does not immediately help diagnose a patient with sudden cardiac arrest, as it sometimes develops over time, Cannon said. “There was a study of 61 family members with a genetic defect,” he said. “Thirty-four percent had a normal echo and ECG when they were first screened, including nine children and 12 adults. When they followed serial echocardiography over the next 6 years, 60% developed new hypertrophy. So if you screen somebody at one point, it may not mean that they don’t have the underlying condition or disease.”

Alfred Bove, MD, PhD
Alfred Bove

If an arrhythmia is detected, its significance must be characterized. It is important to determine whether an arrhythmia that a patient may be experiencing is caused by something like too much coffee, not enough sleep or another factor that would cause it to be more serious, Alfred Bove, MD, PhD, emeritus professor of medicine at Temple University Lewis Katz School of Medicine, told Cardiology Today.

John P. Higgins, MD, MPhil, MBA, FACC, FACP, FAHA, FACSM
John P. Higgins

“Right now, we’ve gotten pretty good at identifying those at high risk with screening by augmenting history and physical with ECGs and ultrasounds,” John P. Higgins, MD, MPhil, MBA, FACC, FACP, FAHA, FACSM, professor of medicine at McGovern Medical School at UTHealth in Houston, chief of cardiology at Lyndon B. Johnson General Hospital, and director of exercise physiology at the Institute for Sports Medicine and Human Performance at the Memorial Hermann Health System, said in an interview. “We’re getting better at finding the conditions, but once we’ve found them ... we don’t want to say, ‘You can’t work out.’ Rather, we should tailor an approach where they can still do most of what they were doing before, but make minor changes ... modifying the exercise(s) that we think might be putting them at high risk during the activity and/or managing the condition better. In 2017, more and more athletes with properly managed cardiac conditions are playing sports than in the past.”

Different levels of athletics

There are different levels of screening throughout the various levels of athletes, from school-age to collegiate to elite, but the advice given to athletes after they are evaluated generally remains the same, Phelan told Cardiology Today.

Michael S. Emery, MD
Michael S. Emery

The current recommendation is for competitive athletes to at least undergo the AHA 14-point history and physical or the 4th edition of the Preparticipation Physical Evaluation, which have been endorsed by the American Medical Society of Sports Medicine and American Academy of Pediatrics, according to Emery.

However, rules and guidelines for evaluations are not as strict for the estimated 8 million school-age students participating in sports, Richard J. Kovacs, MD, the Q.E. and Sally Russell Professor of Cardiology, clinical director of the Krannert Institute of Cardiology, Indiana University School of Medicine, told Cardiology Today. School-aged athletes often visit a family doctor for a physical and a questionnaire and receive a signed form stating whether he or she can return to sports.

“That is not a detailed screening process, and yet it’s appropriate because there are so many athletes and you can’t start doing detailed technical screening on them,” Bove said.

Decision-making for athletes at the school level involves the parents and the doctor. When a decision is made, communication with the school is important regarding safety drills involving automatic external defibrillators and other methods necessary for the athlete to participate.

Although it is important to begin screening for athletic participation at an early age, some cardiac conditions do not manifest until after adolescence, Higgins said.

“If you do one-time screening when a child is 12 or 13 years of age, it’s possible that everything may come back reasonably normal, but then if you were to screen them a few years later, after they have been engaging in a lot more sports and competitive activities, you may find a condition that manifests itself only under extreme activity,” Higgins told Cardiology Today. “That is why most large boards worldwide recommend some sort of screening every 1 or 2 years for very competitive athletes.”

Evaluations for college-age and elite athletes are more detailed than for school-age students. Motivations to continue to play are comparable in both college and elite athletes, as livelihood and scholarships depend on the athlete’s participation.

After an elite athlete is screened, results of the evaluation are reported back to the sports team. Sometimes the team may be risk-averse and go against a doctor’s recommendations if they suggest that the athlete is able to play, especially if there is institutional memory regarding an on-the-field tragedy.

Michael J. Ackerman, MD, PhD
Michael J. Ackerman

For college athletes, universities across the country require different levels of screening. Some schools may require an ECG, physical and medical history, while others perform fewer screening tests than that. Regardless, some schools aim to screen, identify, treat and keep athletes, while others screen, identify, disqualify and eliminate athletes, Ackerman said.

No matter what level of sports an athlete is participating in, there are differing opinions as to how to screen the patients and the recommendations that follow. In a research letter published in Circulation in June, Lampert and colleagues reported that in a series of 440 patients with implantable cardioverter defibrillators who participated in rigorous competitive sports, none experienced failure-to-terminate arrhythmia or personal injury.

Appropriateness of participation

A patient’s specific heart condition is a crucial component of deciding whether they can return to play sports or be disqualified.

“There will be some cardiac conditions where continuing to participate in sport will make the cardiac condition worse or will accelerate the progression of the cardiac condition,” Phelan told Cardiology Today. “In those instances, it’s very important that the athletes discontinue the sport because otherwise they are absolutely accelerating the progression of their disease.”

Although there are not data on every heart condition, there is some critical information on some conditions and what level of sports participation, if any, is best for the patient. For example, patients with long QT syndrome who are properly risk-assessed and treated have low risk for disease-triggered events if they return to play in sports, Ackerman said.

Patients with arrhythmogenic right ventricular cardiomyopathy are recommended not to play sports based on an increasing amount of data. Vigorous exercise in patients with this condition leads to worsening of the underlying cardiomyopathy, which may lead to HF and sudden death, Lampert said.

Patients with the same disorder may have different outcomes. Recommendations for athletes with hypertrophic cardiomyopathy should be dependent on the patient’s level of risk, experts told Cardiology Today.

The type of sport an athlete plays is also an important part of the decision-making process. The Mitchell classification of sports (see Table below) characterizes sports by the amount of static and dynamic activity, or strength vs. endurance, Emery said. This scale helps health care professionals determine the optimal activity level for an athlete based on the condition he or she has.

Table. Mitchell classification of intensity of sports

Continued monitoring

Once an athlete has been screened and cleared to play, a team-based approach to caring for and monitoring the patient is important, experts said.

Richard J. Kovacs, MD
Richard J. Kovacs

“That is a challenge for the cardiovascular team, and should involve the cardiologist, the team physician, the athletic trainers, the parents and the coaches in order to minimize the risk of those patients’ participation,” Kovacs said. “That means not only adherence to the drug or device therapy that’s been recommended, if there has been a recommendation, but also, even with the best of drug and device therapy, there can be cardiac events, and ... everybody involved in the care of the athlete should be familiar with emergency action plans with use of automatic external defibrillators and availability of those devices to resuscitate athletes with known or unknown cardiovascular abnormalities on the field of play.”

Ackerman said he asks school-age patients who have been cleared to play sports to maintain several conditions to be able to continue participation. Some of the conditions include full agreement with the parents, the school-age athlete and the doctor. If one of these people disagree with the patient’s participation, then the athlete is disqualified. The coach, team officials and other entities are contacted about the patient’s condition and necessary safety drills, which includes the athlete having his/her own AED as part of his/her own sports safety gear. and a 911 protocol if an event occurs.

“Akin to an athlete with a life-threatening peanut allergy who is equipped with his/her own EpiPen, our athletes should have their own AED,” Ackerman said.

Ackerman said that despite insisting on this piece of safety gear, he has never had one of his more than 200 athletes with some form of genetic heart disease require an AED rescue in more than 17 years of observation.

Research continues

Much progress has been made over the years to improve the decision-making process of participation or disqualification for sports participation depending on a patient’s CV condition. However, experts Cardiology Today spoke with said more information is needed to make the decision process as informed as possible for health care professionals, athletes, families, teams and others who may be involved.

Quantifying risk for adverse outcomes for each CV condition an athlete can be a valuable tool in making a decision. Patients with the same CV condition sometimes do not have consistent events such as sudden cardiac arrest or death; thus, determining predictors of events would be helpful in assessing patients.

Aaron L. Baggish, MD, FACC, FACSM
Aaron L. Baggish

“Before we can help patients and their families and their teams and their schools make decisions about participation, we really need to better understand in a quantitative fashion what the risk of bad things happening is,” Baggish told Cardiology Today. “We simply can’t educate people and help them make decisions effectively now because we don’t understand the risk calculus.”

One area of further research and awareness is on specific CV conditions, as many current recommendations are based on expert opinion. For example, evidence-based data are available on patients with long QT syndrome and those with ICDs. However, arrhythmogenic right ventricular cardiomyopathy is another CV condition in which more data are needed on the effects of endurance training and disease acceleration, and whether CV health is replenished once training stops.

Further, quality of life is an oft-overlooked topic when disqualifying an athlete from sports participation. Disqualification from sports can affect patients’ lives, including depression, social isolation, suicidal ideation and attempts, weight gain and other risks common with a sedentary lifestyle, experts told Cardiology Today.

Other anticipated research findings should come from a current NIH-funded study (LIVE-HCM/LIVE-LQTS) on patients with either hypertrophic cardiomyopathy or cardiomyopathies and long QT syndrome, with a goal to analyze activity levels and the association with quality of life as well as the disease-triggered event rates in these two specific disease populations.

Research is also moving toward the molecular level to understand how the heart functions and using that information to identify subclinical abnormalities, Bove said. Molecular data may help health care professionals correct various cardiac conditions in athletes, he said.

Experts Cardiology Today interviewed also called for a strategy to educate health care professionals on how to treat this specific patient population.

“There is knowledge required to understand the safety and levels of participation in sports,” Bove said. “We don’t have a board exam or anything in sports cardiology. We’re trying to work toward programs where you can go and spend a week learning sports cardiology.”

In October of this year, a sports cardiology curriculum was published in JACC, which details the formation of a council to define skills that are required to practice sports cardiology effectively. The ACC also runs an annual program called Care of the Athletic Heart, which highlights clinical care of the athlete.

Well-informed decisions

The ACC and AHA recognized sports cardiology as a separate subspecialty in 2011, and more cardiologists are now qualified to help make decisions about athletes with CVD.

“Recognition of the subspecialty of sports cardiology has helped athletes who have heart disease,” Phelan said. “The advice that you will get from someone with a special interest in this area may be very different than what you would get from other cardiologists who do not have a specified interest in sports cardiology.”

Although there are more data needed to treat school-age, collegiate and elite athletes, the movement toward shared decision-making is helping both health care professionals and patients make the right decision regarding return to play.

“Maybe our role is to inform, educate and empower athletes and their families to make a well-informed decision,” Ackerman told Cardiology Today. “With that approach of shared decision-making, we were willing to honor or respect an athlete’s and their family’s right to stay engaged in competitive sport or return to competitive sport, despite being contrary to the previous guidelines.”

Shared decision-making has been involved in the care of athletes for years, but newer statements have accentuated its importance.

“This concept of shared decision-making between athletes, doctors and schools ... isn’t brand new since the 2015 guidelines,” Lampert said. “Presumably all of the patients in our ICD Sports registry have had this type of discussion as they were returning to play, but particularly now with the 2015 guidelines emphasizing class II recommendations, we really need to encourage and be talking about the concept of shared decision-making.” – by Darlene Dobkowski

Disclosures: Baggish, Bove, Emery, Higgins, Kovacs and Phelan report no relevant financial disclosures. Lampert reports she received research funding from Boston Scientific, Medtronic and St. Jude Medical/Abbott, received honoraria from Medtronic and served on an advisory board for Medtronic. Ackerman reports he consults for Audentes Therapeutics, Boston Scientific, Gilead Sciences, Invitae, Medtronic, MyoKardia and St. Jude Medical and may receive potential equity/royalties from AliveCor, Blue Ox Health Corporation and StemoniX.

The decision process behind sports participation for patients with heart disease has changed over time. Previously, a decision was primarily made on strict formulas that often recommended against participation. However, in recent years, new research has suggested that sports participation by patients with heart disease may be acceptable in some cases that were previously considered too risky, and the ways in which decisions are being made are also changing. What is less clear is whether ECG screenings are necessary before allowing presumably healthy young athletes to play.

“The classic paradigm of sports participation was very paternalistic,” Michael S. Emery, MD, assistant professor of clinical medicine at Indiana University School of Medicine, Indianapolis, told Cardiology Today. “It was very much along the lines of, ‘We, in general, don’t recommend you participate in intense athletics’. We often restricted patients with heart disease to participate in low-intensity competitive athletics. Classic was golf and curling if you had heart disease.”

However, new research and greater understanding of the risks and benefits of athletic participation in this population has paved the way for increased support for allowing participation in more cases, particularly if the patient is monitored closely.

One important trend is a movement toward shared decision-making, which involves not only the health care professional and the patient, but also coaches, teams, schools and, depending on age, parents and caregivers.

Rachel J. Lampert, MD, from Yale University School of Medicine, said decision-making about athletic participation by patients with heart disease has become more individualized as risks have become better understood.
Rachel J. Lampert, MD, from Yale University School of Medicine, said decision-making about athletic participation by patients with heart disease has become more individualized as risks have become better understood.

Source: John Curtis/Yale Medicine Office of Communications. Reprinted with permission

This paradigm shift to shared decision-making was a major focus in a paper published in October in Circulation by Aaron L. Baggish, MD, FACC, FACSM, associate professor of medicine at Harvard Medical School and director of the Cardiovascular Performance Program at Massachusetts General Hospital; and Michael J. Ackerman, MD, PhD, professor of medicine, pediatrics and pharmacology and director of the Long QT Syndrome/Genetic Heart Rhythm Clinic and the Windland Smith Rice Sudden Death Genomics Laboratory at the Mayo Clinic in Rochester, Minnesota; and Rachel J. Lampert, MD, professor of internal medicine (cardiology) at Yale University School of Medicine.

“These medical and legal uncertainties represent opportunities, not obstacles,” Lampert, Ackerman and Baggish wrote. “The time has come to acknowledge with full transparency that sport participation [for] athletes with CVD is a complex clinical topic that remains shrouded by persistent scientific and clinical uncertainty. Simplified ‘yes’ vs. ‘no’ decisions are almost always suboptimal, particularly when made through the lens of paternalism.”

Traditional counseling

Decisions regarding participation or disqualification in sports had been predominantly based on recommendations from the 2005 Bethesda guidelines for sports eligibility, which focused on athletes of all ages. Those guidelines listed each heart disease, from systemic hypertension to hypertrophic cardiomyopathy, and a “yes” or “no” answer as to whether a patient can return to play after they have already been diagnosed with a particular CVD, and a health care professional would then consult them accordingly. The recommendations featured in the document were based on expert opinion, current knowledge about disease processes in nonathletic individuals and available evidence from limited registries on sudden cardiac death in sports.

Until several years ago, if there was uncertainty about whether to disqualify a patient from sports activity, it was common for the patient to not return to play. Regardless of whether the cardiologist had doubt, the decision was traditionally approached in a conservative, risk-averse manner. Risks include sudden cardiac death from conditions such as long QT syndrome and hypertrophic cardiomyopathy, though it is important to distinguish nonpathological changes in cardiac morphology associated with athletic training from cardiac diseases.

Dermot M.J. Phelan, MD, PhD, FACC
Dermot M.J. Phelan

“Doctors have traditionally focused on the disease process and the management of the disease. They’ve done an excellent job doing that, but they have not focused at all on the impact of the disease on athletic performance,” Dermot M.J. Phelan, MD, PhD, FACC, staff cardiologist in the section of cardiovascular imaging in the Robert and Suzanne Tomsich Department of Cardiovascular Medicine at Cleveland Clinic; medical director of the Sports Cardiology Center in the Sydell and Arnold Miller Family Heart and Vascular Institute at Cleveland Clinic; and assistant professor at the Cleveland Clinic Lerner College of Medicine at Case Western Reserve University, told Cardiology Today. “There may be a risk involved with permitting a young athlete with heart disease to participate in sports, and if something bad happens to them, then ... the blame is often laid at the doctor’s feet. Traditionally, doctors have not wanted to take on that risk and as a result have been very conservative.”

PAGE BREAK

Updated guidelines

Since the 2005 Bethesda guidelines, updated guidelines have been released, including the 2015 scientific statement by the American Heart Association and the American College of Cardiology. The AHA/ACC scientific statement provided a more tailored approach to deciding whether patients should participate in sports or not based on their CV condition and its severity (see Sidebar below).

The idea of participation in patients who are asymptomatic even in the presence of a CV condition for some conditions was introduced in this set of guidelines. In this patient population, health care professionals were encouraged to counsel on the risks and benefits of continued participation and what to be aware of if symptoms begin to worsen.

The 2015 scientific statement also provided each recommendation within a class and level structure based on whether a patient could be considered to return to play or be disqualified.

“The inclusion of class II recommendations really changes the landscape of counseling and decision-making because now it’s not binary — ‘yes’ or ‘no’, ‘you’re in’ or ‘you’re out’ — but rather it’s now really a decision that needs to be considered,” Lampert told Cardiology Today.

The focus on arrhythmias and other CV conditions in guidelines for the treatment of athletes stems from the awareness of sudden cardiac death, although its incidence has not increased over the past 10 years and some would argue that it is rare. According to a database analysis published in the Mayo Clinic Proceedings in 2016, sudden cardiac death occurs between 1 in 43,000 athlete-years and 67,000 athlete-years in college athletes; for high school athletes, sudden cardiac death occurs between 1 in 46,000 athlete-years and 919,000 athlete-years. The rate of sudden cardiac death varies from 1 in 3,000 athlete-years in NCAA Division I male basketball athletes to 1 in 917,000 athlete-years in high school athletes in Minnesota, according to an analysis published in Circulation in 2015. This has led health care professionals to concentrate more on the athlete and ensure that the screening process is done properly and to enforce a shared decision-making approach.

Updates to athletic participation guidelines
Sources: Baggish AL, et al. Circulation. 2017;in press.
Maron BJ, et al. J Am Coll Cardiol. 2015;doi:10.1016/j.jacc.2015.09.032.

Debate over ECG screening

The question of whether to perform an ECG for preparticipation screening has been a focus of ongoing debate among sports cardiologists. The AHA and ACC do not currently recommend universal ECG screening, whereas the European Society for Cardiology strongly supports screening. Available research is also conflicting. A study from Italy published in JAMA in 2006 showed that instituting ECG screenings decreased the number of young athletes who died, but a study in Israel published in the Journal of the American College of Cardiology in 2011 did not show a change in overall mortality.

In a recent study published in the Journal of the American Heart Association, Harshil Dhutia, BSc, from the cardiology clinical and academic group of St. George’s University of London, and colleagues used the new international criteria, which reduced the number of ECGs that were originally read as abnormal.

Health care professionals who favor ECG screenings generally understand that the data aren’t perfect, but support that screening improves the yield of CV diagnoses in the absence of physical findings and sometimes unreliable family histories.

Although ECG screening may be helpful, a number of changes are needed to properly utilize the screening method, according to some experts. ECGs require careful interpretation, because athletes have different ECG patterns than older adults. An educated workforce can prevent false positives and negatives.

PAGE BREAK

In a debate on ECG screening in athletes at the American College of Cardiology Scientific Session in March, Francesco Fedele, MD, professor in the department of cardiovascular, respiratory, nephrology, anesthesia and geriatric science at Sapienza University of Rome and president of the Italian Federation of Cardiology, said according to one study, adding ECGs to the family history and physical examination portions of screening “would save 2.06 life-years per 1,000 young athletes.”

“The benefit of screening goes beyond the identification of the student’s risk of [sudden cardiac death] because it often triggers the evaluation of first-degree relatives who may also be at risk from inherited cardiac diseases.” Fedele said. “Cardiac screening with ECG has the potential of saving additional lives.”

During that same ACC session, Bryan C. Cannon, MD, FACC, FHRS, pediatric cardiology fellowship director at Mayo Clinic, said during a presentation on the disadvantages of ECG screening that while the cost of saving per human life cannot be valued, “there are too many issues right now to implement universal ECG screening.”

ECG screening does not immediately help diagnose a patient with sudden cardiac arrest, as it sometimes develops over time, Cannon said. “There was a study of 61 family members with a genetic defect,” he said. “Thirty-four percent had a normal echo and ECG when they were first screened, including nine children and 12 adults. When they followed serial echocardiography over the next 6 years, 60% developed new hypertrophy. So if you screen somebody at one point, it may not mean that they don’t have the underlying condition or disease.”

Alfred Bove, MD, PhD
Alfred Bove

If an arrhythmia is detected, its significance must be characterized. It is important to determine whether an arrhythmia that a patient may be experiencing is caused by something like too much coffee, not enough sleep or another factor that would cause it to be more serious, Alfred Bove, MD, PhD, emeritus professor of medicine at Temple University Lewis Katz School of Medicine, told Cardiology Today.

John P. Higgins, MD, MPhil, MBA, FACC, FACP, FAHA, FACSM
John P. Higgins

“Right now, we’ve gotten pretty good at identifying those at high risk with screening by augmenting history and physical with ECGs and ultrasounds,” John P. Higgins, MD, MPhil, MBA, FACC, FACP, FAHA, FACSM, professor of medicine at McGovern Medical School at UTHealth in Houston, chief of cardiology at Lyndon B. Johnson General Hospital, and director of exercise physiology at the Institute for Sports Medicine and Human Performance at the Memorial Hermann Health System, said in an interview. “We’re getting better at finding the conditions, but once we’ve found them ... we don’t want to say, ‘You can’t work out.’ Rather, we should tailor an approach where they can still do most of what they were doing before, but make minor changes ... modifying the exercise(s) that we think might be putting them at high risk during the activity and/or managing the condition better. In 2017, more and more athletes with properly managed cardiac conditions are playing sports than in the past.”

Different levels of athletics

There are different levels of screening throughout the various levels of athletes, from school-age to collegiate to elite, but the advice given to athletes after they are evaluated generally remains the same, Phelan told Cardiology Today.

Michael S. Emery, MD
Michael S. Emery

The current recommendation is for competitive athletes to at least undergo the AHA 14-point history and physical or the 4th edition of the Preparticipation Physical Evaluation, which have been endorsed by the American Medical Society of Sports Medicine and American Academy of Pediatrics, according to Emery.

However, rules and guidelines for evaluations are not as strict for the estimated 8 million school-age students participating in sports, Richard J. Kovacs, MD, the Q.E. and Sally Russell Professor of Cardiology, clinical director of the Krannert Institute of Cardiology, Indiana University School of Medicine, told Cardiology Today. School-aged athletes often visit a family doctor for a physical and a questionnaire and receive a signed form stating whether he or she can return to sports.

“That is not a detailed screening process, and yet it’s appropriate because there are so many athletes and you can’t start doing detailed technical screening on them,” Bove said.

PAGE BREAK

Decision-making for athletes at the school level involves the parents and the doctor. When a decision is made, communication with the school is important regarding safety drills involving automatic external defibrillators and other methods necessary for the athlete to participate.

Although it is important to begin screening for athletic participation at an early age, some cardiac conditions do not manifest until after adolescence, Higgins said.

“If you do one-time screening when a child is 12 or 13 years of age, it’s possible that everything may come back reasonably normal, but then if you were to screen them a few years later, after they have been engaging in a lot more sports and competitive activities, you may find a condition that manifests itself only under extreme activity,” Higgins told Cardiology Today. “That is why most large boards worldwide recommend some sort of screening every 1 or 2 years for very competitive athletes.”

Evaluations for college-age and elite athletes are more detailed than for school-age students. Motivations to continue to play are comparable in both college and elite athletes, as livelihood and scholarships depend on the athlete’s participation.

After an elite athlete is screened, results of the evaluation are reported back to the sports team. Sometimes the team may be risk-averse and go against a doctor’s recommendations if they suggest that the athlete is able to play, especially if there is institutional memory regarding an on-the-field tragedy.

Michael J. Ackerman, MD, PhD
Michael J. Ackerman

For college athletes, universities across the country require different levels of screening. Some schools may require an ECG, physical and medical history, while others perform fewer screening tests than that. Regardless, some schools aim to screen, identify, treat and keep athletes, while others screen, identify, disqualify and eliminate athletes, Ackerman said.

No matter what level of sports an athlete is participating in, there are differing opinions as to how to screen the patients and the recommendations that follow. In a research letter published in Circulation in June, Lampert and colleagues reported that in a series of 440 patients with implantable cardioverter defibrillators who participated in rigorous competitive sports, none experienced failure-to-terminate arrhythmia or personal injury.

Appropriateness of participation

A patient’s specific heart condition is a crucial component of deciding whether they can return to play sports or be disqualified.

“There will be some cardiac conditions where continuing to participate in sport will make the cardiac condition worse or will accelerate the progression of the cardiac condition,” Phelan told Cardiology Today. “In those instances, it’s very important that the athletes discontinue the sport because otherwise they are absolutely accelerating the progression of their disease.”

Although there are not data on every heart condition, there is some critical information on some conditions and what level of sports participation, if any, is best for the patient. For example, patients with long QT syndrome who are properly risk-assessed and treated have low risk for disease-triggered events if they return to play in sports, Ackerman said.

Patients with arrhythmogenic right ventricular cardiomyopathy are recommended not to play sports based on an increasing amount of data. Vigorous exercise in patients with this condition leads to worsening of the underlying cardiomyopathy, which may lead to HF and sudden death, Lampert said.

Patients with the same disorder may have different outcomes. Recommendations for athletes with hypertrophic cardiomyopathy should be dependent on the patient’s level of risk, experts told Cardiology Today.

The type of sport an athlete plays is also an important part of the decision-making process. The Mitchell classification of sports (see Table below) characterizes sports by the amount of static and dynamic activity, or strength vs. endurance, Emery said. This scale helps health care professionals determine the optimal activity level for an athlete based on the condition he or she has.

Table. Mitchell classification of intensity of sports
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Continued monitoring

Once an athlete has been screened and cleared to play, a team-based approach to caring for and monitoring the patient is important, experts said.

Richard J. Kovacs, MD
Richard J. Kovacs

“That is a challenge for the cardiovascular team, and should involve the cardiologist, the team physician, the athletic trainers, the parents and the coaches in order to minimize the risk of those patients’ participation,” Kovacs said. “That means not only adherence to the drug or device therapy that’s been recommended, if there has been a recommendation, but also, even with the best of drug and device therapy, there can be cardiac events, and ... everybody involved in the care of the athlete should be familiar with emergency action plans with use of automatic external defibrillators and availability of those devices to resuscitate athletes with known or unknown cardiovascular abnormalities on the field of play.”

Ackerman said he asks school-age patients who have been cleared to play sports to maintain several conditions to be able to continue participation. Some of the conditions include full agreement with the parents, the school-age athlete and the doctor. If one of these people disagree with the patient’s participation, then the athlete is disqualified. The coach, team officials and other entities are contacted about the patient’s condition and necessary safety drills, which includes the athlete having his/her own AED as part of his/her own sports safety gear. and a 911 protocol if an event occurs.

“Akin to an athlete with a life-threatening peanut allergy who is equipped with his/her own EpiPen, our athletes should have their own AED,” Ackerman said.

Ackerman said that despite insisting on this piece of safety gear, he has never had one of his more than 200 athletes with some form of genetic heart disease require an AED rescue in more than 17 years of observation.

Research continues

Much progress has been made over the years to improve the decision-making process of participation or disqualification for sports participation depending on a patient’s CV condition. However, experts Cardiology Today spoke with said more information is needed to make the decision process as informed as possible for health care professionals, athletes, families, teams and others who may be involved.

Quantifying risk for adverse outcomes for each CV condition an athlete can be a valuable tool in making a decision. Patients with the same CV condition sometimes do not have consistent events such as sudden cardiac arrest or death; thus, determining predictors of events would be helpful in assessing patients.

Aaron L. Baggish, MD, FACC, FACSM
Aaron L. Baggish

“Before we can help patients and their families and their teams and their schools make decisions about participation, we really need to better understand in a quantitative fashion what the risk of bad things happening is,” Baggish told Cardiology Today. “We simply can’t educate people and help them make decisions effectively now because we don’t understand the risk calculus.”

One area of further research and awareness is on specific CV conditions, as many current recommendations are based on expert opinion. For example, evidence-based data are available on patients with long QT syndrome and those with ICDs. However, arrhythmogenic right ventricular cardiomyopathy is another CV condition in which more data are needed on the effects of endurance training and disease acceleration, and whether CV health is replenished once training stops.

Further, quality of life is an oft-overlooked topic when disqualifying an athlete from sports participation. Disqualification from sports can affect patients’ lives, including depression, social isolation, suicidal ideation and attempts, weight gain and other risks common with a sedentary lifestyle, experts told Cardiology Today.

Other anticipated research findings should come from a current NIH-funded study (LIVE-HCM/LIVE-LQTS) on patients with either hypertrophic cardiomyopathy or cardiomyopathies and long QT syndrome, with a goal to analyze activity levels and the association with quality of life as well as the disease-triggered event rates in these two specific disease populations.

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Research is also moving toward the molecular level to understand how the heart functions and using that information to identify subclinical abnormalities, Bove said. Molecular data may help health care professionals correct various cardiac conditions in athletes, he said.

Experts Cardiology Today interviewed also called for a strategy to educate health care professionals on how to treat this specific patient population.

“There is knowledge required to understand the safety and levels of participation in sports,” Bove said. “We don’t have a board exam or anything in sports cardiology. We’re trying to work toward programs where you can go and spend a week learning sports cardiology.”

In October of this year, a sports cardiology curriculum was published in JACC, which details the formation of a council to define skills that are required to practice sports cardiology effectively. The ACC also runs an annual program called Care of the Athletic Heart, which highlights clinical care of the athlete.

Well-informed decisions

The ACC and AHA recognized sports cardiology as a separate subspecialty in 2011, and more cardiologists are now qualified to help make decisions about athletes with CVD.

“Recognition of the subspecialty of sports cardiology has helped athletes who have heart disease,” Phelan said. “The advice that you will get from someone with a special interest in this area may be very different than what you would get from other cardiologists who do not have a specified interest in sports cardiology.”

Although there are more data needed to treat school-age, collegiate and elite athletes, the movement toward shared decision-making is helping both health care professionals and patients make the right decision regarding return to play.

“Maybe our role is to inform, educate and empower athletes and their families to make a well-informed decision,” Ackerman told Cardiology Today. “With that approach of shared decision-making, we were willing to honor or respect an athlete’s and their family’s right to stay engaged in competitive sport or return to competitive sport, despite being contrary to the previous guidelines.”

Shared decision-making has been involved in the care of athletes for years, but newer statements have accentuated its importance.

“This concept of shared decision-making between athletes, doctors and schools ... isn’t brand new since the 2015 guidelines,” Lampert said. “Presumably all of the patients in our ICD Sports registry have had this type of discussion as they were returning to play, but particularly now with the 2015 guidelines emphasizing class II recommendations, we really need to encourage and be talking about the concept of shared decision-making.” – by Darlene Dobkowski

Disclosures: Baggish, Bove, Emery, Higgins, Kovacs and Phelan report no relevant financial disclosures. Lampert reports she received research funding from Boston Scientific, Medtronic and St. Jude Medical/Abbott, received honoraria from Medtronic and served on an advisory board for Medtronic. Ackerman reports he consults for Audentes Therapeutics, Boston Scientific, Gilead Sciences, Invitae, Medtronic, MyoKardia and St. Jude Medical and may receive potential equity/royalties from AliveCor, Blue Ox Health Corporation and StemoniX.