Competitive Sport Participation Among Athletes With Heart Disease

More than 8 million young men and women participate in competitive high school or collegiate sports in the United States annually. For the vast majority of these young men and women, sport participation plays an invaluable role in promoting healthy physical, emotional, and cognitive development. In rare cases, however, seemingly healthy athletes with occult cardiovascular disease (CVD) die suddenly during sport participation. Strategies to reduce these highly visible tragedies, including preparticipation screening and medical oversight of training and competition, are well established and can lead to the identification of athletes with undiagnosed CVD. For the competitive athlete diagnosed with CVD, determination of future athletic eligibility is a critical step in a care continuum that begins with diagnosis and continues through long-term treatment. Fundamentally, eligibility determination is a quest to reduce the risk of future sudden death while simultaneously avoiding unnecessary sport restriction. This process may be challenging for the patient and family, clinicians, and schools or sporting organizations. Accordingly, the American College of Cardiology (ACC), the American Heart Association (AHA), and the Heart Rhythm Society (HRS) provide eligibility recommendations for competitive athletes with established CVD.

Until recently, the 2005 update of the 36th Bethesda Conference statement, an encyclopedic compendium of CVD diagnoses with binary yes-or-no eligibility recommendations for each diagnosis, served as the standard of care.¹ This binary approach to eligibility inherently facilitated paternalistic decision making, in which doctors, schools, and athletic organizations were encouraged/expected to permit or restrict sport participation with little or no input from the afflicted athlete (and parents). It is important to note that the right of a school to implement this guideline-centric model of paternalistic decision making was supported by the pivotal court ruling on Knapp versus Northwestern.

In December 2015, a competitive sport eligibility statement that was sponsored jointly by ACC/AHA/HRS updated and replaced the 2005 Bethesda Conference.² This new document moves away from a binary approach to athletic eligibility and adopts the more nuanced and contemporary Class and Level of Evidence approach. Now used universally in cardiology professional society guidelines, this approach designates clinical strategies, in this case competitive athletic eligibility, as class I, recommended; class IIA and IIB indicating eligibility is reasonable or may be considered; and class III, not recommended. It is important to note that a number of CVD conditions including long-QT syndrome and the presence of an implantable cardiac defibrillator, for which competitive sports were previously considered strictly restricted, now carry class II recommendations. By definition, class II recommendations acknowledge clinical and scientific uncertainty, thereby providing clinicians and patients with an opportunity for flexible and individualized decision making. The potential impact of newly introduced class II recommendations is not trivial, because the AHA/ACC/HRS eligibility document now contains 84 class II recommendations among the 253 diagnoses and clinical scenarios.

Three scientific and societal currents underlie this important paradigm shift in the clinical approach to sport eligibility following CVD diagnosis. First, prior guidelines were based entirely on expert opinion because, until recently, data defining outcomes among athletes with CVD were lacking. Emerging observational data now demonstrate that the risks of competitive sport participation in some conditions appear to be lower than previously projected, and thus probably nonprohibitive.^3,4 Second, the Class of Recommendation approach has been accepted universally across cardiology statements because it acknowledges that available data are often incomplete and imperfect, a reality that needs to be integrated into medical decision making and communicated to patients and other relevant stakeholders. Third, there is increasing recognition across the broader medical community that involving patients and, when appropriate, parents, in their own medical care, through shared decision making, is an ethical imperative. The shared decision-making model acknowledges the inherent limitations of paternalism while recognizing the fundamental role of the physician in guiding patient care decisions. Termed the “pinnacle of patient-centered care,”⁵ shared decision making requires the physician to explain the risks and benefits of options and to help patients understand how to reconcile these options with their personal preferences and values.

The 2015 ACC/AHA/HRS eligibility document does not contain the phrase “shared decision making.” However, it indirectly but explicitly encourages this approach by acknowledging the importance of counseling the athlete with newly diagnosed CVD throughout the document. Counseling athletes with newly diagnosed CVD is not simply about managing grief around their diagnosis, but rather an educational process including reviewing available clinical outcomes data, acknowledging areas of ongoing clinical and scientific uncertainty, and striving to understand their patient’s level of comprehension and underlying belief system. This counseling process should keep the patient in a central role while simultaneously engaging other key individuals including family members, other care providers with key roles in the athlete care team, and, when appropriate, sponsoring schools and sport organizations. It is noteworthy that shared decision making in the care of athletes with CVD began before the 2015 guideline update. Pivotal recent data describing sport safety among athletes with implantable cardiac defibrillators and optimally treated long-QT syndrome were obtained through the study of athletes who chose to participate in competitive sports against the rules and doctors who remain engaged in their care. Patients with long-QT syndrome were treated and studied by one of us (M.J.A.), whereas athletes with implantable cardiac defibrillators were studied prospectively through the Implantable Cardiac Defibrillator Sports Registry by two of us (R.L. and M.J.A.). Future work to define clinical outcomes among athletes with other forms of CVD during sport participation is of critical importance.

The full clinical and social implications of this potentially radical paradigm shift in decision making regarding competitive sport eligibility among athletes with CVD have yet to be defined. Since the publication of the 2015 guidelines, we have seen firsthand a marked increase in referrals to our sports-cardiology program (A.L.B.) and genetic heart rhythm clinic (M.J.A.) for discussions of eligibility. This trend suggests increasing uncertainty in the cardiology and sports medicine communities about how to make decisions now that guidelines no longer endorse a comparatively simpler yes-or-no approach. Furthermore, how the legal community will interpret the new guidelines when an athlete elects to pursue sport participation, but a doctor, school, or organization disagrees, has not been tested. In the Knapp versus Northwestern decision, the right of the school to exclude Nick Knapp, without violating the Rehabilitation Act, was grounded in the fact that the school had made their decision on sound medical evidence including the use of published guidelines. Now that eligibility guidelines use phrases such as “may be considered,” it remains to be seen whether it is the athlete, physician, or school/sporting organization who renders the final verdict. Although Knapp versus Northwestern supported guidelines-based decision making, the ruling also allows for individualization by stating, “Simply put, all universities need not evaluate risk the same way.” These medical and legal uncertainties represent opportunities, not obstacles. The time has come to acknowledge, with full transparency, that sport participation among athletes with CVD is a complex clinical topic that remains shrouded in persistent scientific and clinical uncertainty. Simplified yes-versus-no decisions are almost always suboptimal, in particular when made through the lens of paternalism. Our young athletes deserve more. The time has come to partner with these young men and women in health and athletic participation decision making with an ultimate goal of promoting safety without unnecessary risk aversion.

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