Shared decision-making is a process in which a medical decision is made for a patient that actively involves the physician, patient, and at times other health care professionals and family members.¹ In a shared decision patient care model, all parties involved share ideas and information. The healthcare provider describes different approaches with their potential risks and benefits. The patient, equipped with this information, shares their preferences. Overall, in a shared decision model both parties share responsibility for the ultimate decision reached.

An area of medicine that is ideal for shared decision-making is that of sports participation in an athlete who also has a clear or unclear increased risk of sudden death due to the presence of a heart disorder. In 2005, The 36th Bethesda Conference of the American College of Cardiology published recommendations for competitive athletes with cardiovascular abnormalities in the United States. In this document, symptomatic long QT, asymptomatic long QT (males >470 ms, females >480 ms), short QT, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia (CPVT), and probable or unequivocal hypertrophic cardiomyopathy were restricted to class 1A sport activities.² Class 1A activities have a low static (maximal voluntary contraction) and a low dynamic component (maximal oxygen uptake).³ The listed sports in this category were billiards, bowling, cricket, curling, golf, and riflery. Other disorders were presented in these recommendations, but for this article we will focus on channelopathies and hypertrophic cardiomyopathy as they illustrate well the challenges faced by clinicians and our evolving understanding of risks.

From a practice guidelines standpoint, it is relatively easy to promote a conservative management approach as cardiovascular intensity in the setting of a predisposition may promote arrhythmia. However, in clinical practice, when talking to competitive and trained athletes, active adolescents, and sports enthusiasts, these guidelines can be life altering. In some cases adherence to low intensity activities and a less active lifestyle can result in weight gain, depression, and ultimately other acquired cardiovascular disease states. In other cases, recommendations are not followed by patients as they pursue higher degree athletic activities as a means to improve quality of life, minimize mood disturbances, and promote health. For example, in our heritable heart disease clinic we care for a woman with long QT1 syndrome (>500 ms) manifest with a syncopal episode while waterskiing. She is on beta blockade therapy and has not had another event. However, despite our recommendations she actively competes in swim meets, triathlons, and other endurance sports. She understands the potential risks and swims with a capable partner, but learning of these activities creates angst in her healthcare providers.

Recently, sport participation guidelines were updated by the American College of Cardiology and American Heart Association.^4,5 What has changed since 2005 is that there are many patients, like ours, who choose to participate in athletics despite risk. Providers prescribe beta blockers and avoid QT prolonging drugs and recommend careful attention to electrolyte replacement, minimizing prolonged periods of pyrexia, having an automated external defibrillator (AED), and discourage swimming. Observational experiences with long QT syndrome patients who return to athletics have been very good with very few adverse events.^6,7 In fact, in these studies only one event was observed in a patient who had beta blocker noncompliance and received appropriate ICD shocks (estimated event rate of 1 event in 331 athlete-years). The newer guidelines reflect these experiences with support for return to athletic activities in those patients with guideline-based therapies used with compliance, an asymptomatic period of at least 3 months, and other disease-based precautionary measures.⁵ Unfortunately for patients with hypertrophic cardiomyopathy, recommendations remain similar in that once cardiac manifestations of the disease are present by echocardiogram or MRI, only class 1A activities are recommended. This partially reflects a cardiac disease state that is less predictable and also subject to physiologic stresses and influences common in athletics such as dehydration, electrolyte shifts, catecholamine surges, etc.⁴

Returning to our model of shared decision making, let's illustrate the process for both cardiac channelopathies and hypertrophic cardiomyopathy. In cardiac channelopathies that are well managed with medical therapy, if patients are asymptomatic and other disease-specific precautions are in place, including the availability of an AED, returning to sports is a viable option. It is not risk free, but neither is confinement to activities that do not allow people to expend calories, maintain a healthy weight, lower blood pressure, and maintain muscle and bone integrity. Right now there are no data to support the use of an ICD as a means to allow these patients to increase their athletic participation with a potentially even lower risk.⁸ However, in patients with an ICD who are free of symptoms for at least 3 months and who are on appropriate therapy, athletic participation can be considered beyond class 1A activities. To some patients and their families the event rate of one in 331 athlete-years without any observed deaths is too high.^6,7 In others, the risk is acceptable when considered against the quality of life and potential health benefit gained with athletic participation beyond class 1A.

In asymptomatic hypertrophic cardiomyopathy patients who are genotype positive and have no structural changes on echocardiogram or MRI, cardiovascular risks are low and athletic activities are not restricted. For hypertrophic cardiomyopathy patients with structural changes, physicians have no guideline statements recommending the utilization of shared decision-making model to expand activities beyond those considered class 1A. Similar to other cardiac disorders, guideline statements have not advocated prophylactic ICDs to allow greater athletic participation.⁴ In patients who want to consider more athletic participation, we discuss the uncertainty of the disease and our limited metrics of risk prediction. If they do participate, we use beta blockers to lower peak heart rate and modify catecholamine influence on the heart, set discrete heart rate goals, aggressively promote hydration, minimize electrolyte loss, and avoid pyrexia that can lead to dehydration. This strategy is complicated because beta blockers can impair performance and athletes may be disinclined to take them. In addition, we recommend that they carry an AED as part of their safety athletic gear. This strategy is also very complex with issues including availability of the AED, someone designated to use it, keeping the battery charged and maintained, and the bulkiness of the device, which can be limiting to athletes exercising independently. College, national team, and professional athletes often have knowledgeable training staff that makes this more feasible, but those organizations may be very uncomfortable assuming risk. Unlike long QT syndrome patients, we cannot tell patients these steps result in a very low risk of cardiovascular events and death. However, we emphasize to these patients that if they do develop other diseases, such as coronary disease, obesity, or sleep apnea, the hypertrophic cardiomyopathy disease risk increases and to this end we need to find ways to keep them active and maintain a healthy lifestyle.^9,10 Some patients are comfortable with the disease uncertainty and these prophylactic risk modifications and continue to participate in athletic activities beyond class 1A. Others are less comfortable and make different lifestyle choices. In those that significantly reduce their activity to a class 1A level, we focus our discussion on becoming less sedentary in general day-to-day activities, increasing walking volume, and dietary approaches to weight loss if needed. Management of hypertrophic cardiomyopathy patients will continue to evolve as observational studies may validate or question consensus recommendations. For example, at the recent European Society of Cardiology Scientific Sessions, Finocchiaro and colleagues reported in a post-mortem study of 148 hypertrophic cardiomyopathy patients that the majority died at rest (81%) not during exertion.¹¹ However, few of these people were considered recreational or competitive athletes (11%) before their event. These data raise important questions into mechanisms of arrest in the community and should cause physicians to question if severely limiting exercise activities and athletic participation are the only options that should be considered.

Scenarios in which patients choose activities that may increase cardiac event rates are never comfortable for a physician. However, athletic activity participation is a lifestyle choice, like many others, with potential risks and benefits that are amplified in patients with a cardiac risk predisposition. When these riskier lifestyle choices are made in a shared decision model where all data are presented, our job as healthcare providers is to try to reduce risk wherever possible in the confines of the patient's informed choices.

References

1. Charles C, Gafni A, Whelan T. Shared decision-making in the medical encounter: what does it mean? (or it takes at least two to tango). Soc Sci Med 1997;44:681-92.
2. Zipes DP, Ackerman MJ, Estes NA, Grant AO, Myerburg RJ, Van Hare G. Task force 7: arrhythmias. J Am Coll Cardiol 2005;45:1354-63.
3. Mitchell JH, Haskell W, Snell P, Van Camp SP. Task force 8: classification of sports. J Am Coll Cardiol 2005;45:1364-7.
4. Maron BJ, Udelson JE, Bonow RO, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2362-71.
5. Ackerman MJ, Zipes DP, Kovacks RJ, Maron BJ. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 10: the cardiac channelopathies: a scientific statement from the American Heart Assocation and the American College of Cardiology. J Am Coll Cardiol 2015;66:2424-8.
6. Johnson JN, Ackerman MJ. Competitive sports participation in athletes with congenital long QT syndrome. JAMA 2012;308:764-5.
7. Johnson JN, Ackerman MJ. Return to play? Athletes with congenital long QT syndrome. Br J Sports Med 2013;47:28-33.
8. Zipes DP, Link MS, Ackerman MJ, Kovacks RJ, Myerburg RJ, Estes NA. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 9: arrhythmias and conduction defects: a Scientific Statement from the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015;66:2412-23.
9. Sengupta PP, Sorajja D, Eleid MF, et al. Hypertrophic obstructive cardiomyopathy and sleep-disordered breathing: an unfavorable combination. Nat Clin Pract Cardiovasc Med 2009;6:14-5.
10. Sorajja P, Ommen SR, Nishimura RA, Gersh BJ, Berger PB, Tajik AJ. Adverse progniosis of patients with hypertrophic cardiomyopathy who have epicardial coronary artery disease. Circulation 2003;108:2342-8.
11. Finocchiaro G, Papadakis M, Dhutia H, et al. Circumstances of sudden death in hypertrophic cardiomyopathy: data from a large pathology registry. Eur Heart J 2016;37:1226.

Keywords: Athletes, Blood Pressure, Body Weight, Brugada Syndrome, Cardiomyopathy, Hypertrophic, Cardiovascular Abnormalities, Catecholamines, Coronary Disease, Defibrillators, Dehydration, Electrolytes, Genotype, Gryllidae, Heart Rate, Long QT Syndrome, Obesity, Patient Care, Quality of Life, Risk Assessment, Risk Factors, Sleep Apnea Syndromes, Syncope, Tachycardia, Ventricular, Walking, Weight Gain, Weight Loss

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