Are the Bethesda Guidelines Still Relevant for the Pediatric Athlete with Heart Disease

ACCEL | Back in July 2013, CardioSource WorldNews (and ACCEL) featured an interview with Michael John Ackerman, MD, PhD, who argued that current guidelines for determining eligibility for sports among young athletes with heart disease can be boiled down to "tell them all no." As a participant and co-author of the 2005 ACC Bethesda Conference Report on the topic,1 Dr. Ackerman said, both the US and European guidelines2 relating to sports participation say essentially: "Unless your heart is perfect or the syndrome is confined to just your genome…no competitive sports except perhaps class 1A sports."

Take-aways
  • There is a need to guide sports participation in the pediatric patient with heart disease, but in some settings the current guidelines for competitive athletic participation may be overly restrictive.
  • Far more youngsters with complex cardiovascular disease are growing into adulthood but high-profile cases of sudden cardiac death in young athletes has highlighted the awareness of medico-legal implications of clinical decision making.
  • Guidelines are still relevant, but it's important to individualize recommendations for a particular athlete based on sound peer-reviewed data, wisdom, and athlete/family input.
  • Examples of class 1A sports include billiards, bowling, cricket, curling, golf, and riflery.

    Using the recommendations relating to individuals with congenital long QT syndrome (LQTS) as an example, he said the guidelines advised disqualification from almost all sports (except those class 1A sports) for patients with:

    • history of symptoms
    • asymptomatic baseline ECG with QTc >470 ms (males) or >480 ms (females)
    • The exception: genotype-positive/phenotype-negative LQTS patients are OK to play (other than LQT1 individuals who should avoid swimming)

    The European Society of Cardiology (ESC) guidelines advise disqualification from all competitive sports for any LQTS patient, symptomatic or asymptomatic. As for exercise-positive/phenotype-negative patients, the ESC said it is not OK for them either.

    In other words, Dr. Ackerman said, according to the ESC, the appropriate message for sports participants determined to have congenital LQTS is "Forget about it! Manifest or concealed, you are done. Gene carriers, you are done."

    Still Relevant or Not?

    So, are current recommendations still relevant or not? According to Silvana Lawrence, MD, PhD, here's the reasoning behind the guidelines:

    • There is a need to guide sports participation in individuals with cardiovascular conditions.
    • The recommendations were based on sound published data available at the time, as well as expert opinion, and both individual and collective judgments.
    • There is an increased risk of sudden cardiac death during intense athletic participation and this is deemed a controllable risk factor.
    • High-profile cases have raised awareness of the medico-legal implications of decision making.

    However, even the 36th Bethesda Conference Report noted that the recommendations were "intended neither to be overly permissive nor restrictive" and "should not be regarded as absolutely rigid dictum." Dr. Lawrence acknowledges that sudden cardiac death is a devastating event—despite its rarity—and individuals with a cardiovascular condition are more likely to succumb during exertion. Advances in genomics, however, have helped our understanding of risk conditions. Unfortunately, risk stratification remains the Holy Grail to appropriately direct safe participation. While that's not possible yet, she said the decision to play no longer rests only with the physician.

    To make that point, she cited a paper by Dr. Ackerman demonstrating that athletes and their families are fully capable of self-disqualification.3 Records of all patients were evaluated in a 10-year period for documentation of athletic participation after LQTS diagnosis and LQTS-related events during an average follow-up of 5.5±3.4 years. The individuals included 353 patients with LQTS (199 females) and a mean age of 17±11 years.

    The majority of patients (63%) were either not involved in sports in the first place (88% of this group) or chose to discontinue sports (12%) following evaluation. Another 130 patients remained in competitive athletics, including 20 with implantable cardioverter-defibrillators (ICDs). Of these 130 patients, about half were genotype-positive/phenotype-negative and competing in athletics contrary to ESC guidelines, but within the Bethesda guidelines. None of these athletes had a sport-related event. Of the 60 LQTS athletes continuing in sports contrary to both the Bethesda and ESC guidelines (genotype-positive/phenotype-positive), only one had a sporting-related event with appropriate ICD shock.

    The one patient with an event was a known high-risk 9-year-old LQT1 male with a history of cardiac arrest, who received two appropriate ICD therapies, both while warming up prior to practices, and both during admitted non-compliance with ß-blocker medication.

    Dr. Ackerman and his co-author, Jonathan Johnson, MD, concluded that current guideline-based recommendations for disqualification may be excessive for this disease.

    More than LQTS

    Of course, LQTS is just one of many CV issues young athletes face that can impact the risk of sports participation. Dr. Lawrence said health care providers are being challenged by several factors:

    • Expanding knowledge in genetic expression of CV disease
    • Advances in medical and device therapy
    • Exponential increase in survival into adulthood of individuals with complex congenital heart disease
    • Widespread knowledge of scientific advances

    Given the pandemic of physical inactivity in the 21st century and epidemic of childhood obesity, Dr. Lawrence said, "It must be our mission as cardiovascular professionals to nurture physical activity and safe regular exercise participation for all."

    So, are the current guidelines still relevant? Yes, she said, they are. However, there is a pressing need for revisions given new advances in genomics, evidence of a natural history in patients that challenges the present guidelines, more data on exercise performance in patients with coronary heart disease, and an expanding population of adults with congenital heart defects.


    References

    1. Maron BJ, Zipes DP. J Am Coll Cardiol. 2005;45:1312-75.
    2. Pelliccia A, Fagard R, Bjørnstad HH, et al. Eur Heart J. 2005;26:1422-45.
    3. Johnson JN, Ackerman MJ. Br J Sports Med. 2013;47:28-33.

    Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Sports and Exercise Cardiology, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Congenital Heart Disease, CHD & Pediatrics and Arrhythmias, CHD & Pediatrics and Prevention, CHD & Pediatrics and Quality Improvement, Sports & Exercise and Congenital Heart Disease & Pediatric Cardiology

    Keywords: Athletes, Decision Making, Documentation, Long QT Syndrome, Pediatric Obesity, Risk Factors, Genomics, Electrocardiography, Awareness, Child, Judgment, Expert Testimony, Cardiology, Death, Sudden, Cardiac, ACC Publications, CardioSource WorldNews


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