An estimated 1 to 3/100,000 apparently healthy young athletes develop abrupt-onset ventricular tachycardia or ventricular fibrillation and die suddenly during exercise. Males are affected up to 10 times more often than females. Basketball and football players in the US and soccer players in Europe may be at highest risk (1, 2).
In young athletes, sudden cardiac death has many causes (see table Causes of Sudden Cardiovascular Death in Young Athletes), but the most common is
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Undetected hypertrophic cardiomyopathy
Commotio cordis (sudden ventricular tachycardia or fibrillation after a blow to the precordium) is a risk in athletes with thin, compliant chest walls even when no cardiovascular disorder is present. The blow may involve a moderate-force projectile (eg, baseball, hockey puck, lacrosse ball) or impact with another player during a vulnerable phase of myocardial repolarization.
Other causes include inherited arrhythmia syndromes (eg, long QT syndrome, Brugada syndrome). Some young athletes die of aortic aneurysm rupture (in Marfan syndrome).
In older athletes, sudden cardiac death is typically caused by
Occasionally, hypertrophic cardiomyopathy or valvular disease is involved.
In other conditions underlying sudden death in athletes (eg, asthma, heatstroke, illicit or performance-enhancing drug-related complications), ventricular tachycardia or fibrillation is a terminal, not a primary, event.
Symptoms and signs are those of cardiovascular collapse; diagnosis is obvious.
Immediate treatment with advanced cardiac life support is successful in < 20%; the percentage may increase as distribution of community-based, automated external defibrillators (AEDs) expands. In fact, studies have demonstrated that the presence of AEDs can increase the neurologically intact survival rates to over 80% (3). For survivors, treatment is management of the underlying condition. In some cases, an implanted cardioverter-defibrillator may ultimately be required.
Causes of Sudden Cardiovascular Death in Young Athletes
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Commotio cordis |
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Coronary artery anomalies (eg, anomalous left main coronary artery origin, anomalous right coronary artery origin, coronary arterial hypoplasia) |
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Ruptured aortic aneurysm |
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Arrhythmogenic right ventricular dysplasia |
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Tunneled left anterior descending coronary artery |
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Premature atherosclerotic coronary artery disease |
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Myxomatous degeneration of mitral valve |
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Wolff-Parkinson-White syndrome (anterograde conduction only) |
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Catecholaminergic polymorphic tachycardia |
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Right ventricular outflow tract tachycardia |
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Cardiac sarcoidosis |
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Cardiac trauma |
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Ruptured cerebral artery aneurysm |
General references
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1. Maron BJ, Shirani J, Poliac LC, et al: Sudden death in young competitive athletes: Clinical, demographic, and pathological profiles. JAMA 276(3):199–204, 1996.
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2. Maron BJ, Haas TJ, Ahluwalia A, et al: Demographics and epidemiology of sudden deaths in young competitive athletes: From the United States National Registry. Am J Med 129:1170–1177, 2016. doi: 10.1016/j.amjmed.2016.02.031
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3. Johri AM, Poirier P, Dorian P, et al: Canadian Cardiovascular Society/Canadian Heart Rhythm Society joint position statement on the cardiovascular screening of competitive athletes. Can J Cardiol 35:1-11, 2019. doi: 10.1016/j.cjca.2018.10.016
Cardiovascular Screening for Sports Participation
Athletes are commonly screened to identify risk before participation in sports. In the United States, they are reevaluated every 2 years (if high school age) or every 4 years (if college age or older). In Europe, screening is repeated every 2 years regardless of age.
Screening recommendations in the US for college-age young adults—as well as for children and adolescents—include the following:
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Medical, family, and drug history (including use of performance-enhancing drugs and drugs that predispose to long QT syndrome)
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Physical examination (including blood pressure and supine and standing cardiac auscultation)
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Selected testing based on findings on history and physical examination
European guidelines differ from American guidelines in that a screening electrocardiogram (ECG) is recommended for all children, adolescents, and college-age athletes.
Screening for older adults (35 years or older) with risk factors may include incremental symptom-limited exercise testing, especially if they have been sedentary for a number of years.
History and examination are neither sensitive nor specific; false-negative and false-positive findings are common because prevalence of cardiac disorders in an apparently healthy population is very low. Use of screening ECG or echocardiography would improve disease detection but would produce even more false-positive diagnoses and is impractical at a population level.
Genetic testing for hypertrophic cardiomyopathy or long QT syndrome is not recommended or even feasible for the screening of athletes.
Selected testing
Athletes with a family history or symptoms or signs of hypertrophic cardiomyopathy, long QT syndrome, or Marfan syndrome require further evaluation, typically with ECG, echocardiography, or both. Confirmation of any of these disorders may preclude sports participation.
Athletes with presyncope or syncope can also be evaluated for anomalous coronary arteries (eg, by cardiac catheterization).
If ECG reveals Mobitz type II heart block, complete heart block, true right bundle branch block, or left bundle branch block, or there is clinical or electrocardiographic evidence of supraventricular or ventricular rhythm disorders, a search for cardiac disease is required.
If an enlarged aorta is detected on echocardiography (or incidentally), further assessment is needed.
Recommendations
Athletes should be counseled against use of illicit and performance-enhancing drugs. Patients with mild or moderate valvular heart disease may participate in vigorous activity; however, patients with severe valvular heart disease, particularly of the stenotic variety, should not participate in competitive sports or high-intensity recreational sports. Patients with most structural or arrhythmogenic heart disorders (eg, hypertrophic cardiomyopathy, coronary artery anomalies, arrhythmogenic right ventricular dysplasia) should not participate in competitive sports or high-intensity recreational sports.
Key Points
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Sudden cardiac death during exercise is rare and is most commonly due to hypertrophic cardiomyopathy (younger athletes) and coronary artery disease (older athletes).
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Screen younger participants (children through young adults) with history and physical examination; those with abnormal findings or positive family history typically have ECG and/or echocardiography.
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Screen older participants with risk factors (particularly if they have been sedentary for a number of years) with history, physical examination, and usually an exercise stress test.
More Information
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Johri AM, Poirier P, Dorian P, et al: Canadian Cardiovascular Society/Canadian Heart Rhythm Society joint position statement on the cardiovascular screening of competitive athletes. Can J Cardiol 35:1-11, 2019. doi: 10.1016/j.cjca.2018.10.016
