Sudden death (SD) in individuals between the ages of 12 and 25 years (particularly those participating in organized sports activities) may not be a common occurrence, but it is a particularly devastating event for families and communities.1 Even though SD can occur in any population, most discussions of SD focus on young athletes. Although a mandatory reporting system for SD in young people is lacking, SD in young athletes arouses considerable public interest. Vigorous physical activity in young persons with undetected cardiovascular risk factors increases their risk for SD.1 The harsh reality is that SD occurs in 1 of every 53,703 college athletes yearly in the United States, with the majority of these deaths occurring in male athletes of Afro-Caribbean descent.1-3 The most common cause of SD in young people is hypertrophic cardiomyopathy (HCM), although congenital coronary anomalies, myocarditis, valvular heart disease, dilated cardiomyopathy, Wolff-Parkinson-White (WPW) syndrome, and ion channelopathies are also seen in a small number of cases of SD.1,2 Table 1 lists the pathophysiology, risk factors, and presentation of some of the common causes of SD in young athletes. However, it is important to keep in mind that this number of deaths is relatively low compared with the number of deaths from other causes in the same age group.1
It is a common practice in the United States for individuals participating in sanctioned sports in high school and college to undergo some type of preparticipation screening to detect factors associated with a high risk for injury and/or death; however, this screening process has yet to be standardized.1 In 2014, the American Heart Association (AHA) and the American College of Cardiology (ACC) published recommendations for the screening of young people to detect cardiovascular disease, along with the role of 12-lead electrocardiography (ECG) in such screening. To assist in the detection of the genetic and congenital cardiac conditions that are largely responsible for SD, the recommendations include 14 elements that should be evaluated during the preparticipation history and physical examination that are mandatory for all athletes wishing to participate in competitive sports. In the position paper, the AHA and ACC make it clear that they do not currently support universal 12-lead ECG testing in asymptomatic individuals.1 However, the importance of automated external defibrillators (AEDs) in responding to cardiac emergencies is stressed, and AEDs are considered an invaluable tool in the prevention of death.2
The 14 elements that the AHA and ACC recommend be included in the preparticipation screening of competitive athletes cover personal history, family history, and a physical examination. The personal history should include information regarding the following: exertional chest pain or discomfort; unexplained syncopal or near-syncopal episodes; exertional dyspnea, fatigue, or palpitations; prior history of a heart murmur; hypertension; prior restrictions on sports activities; and prior cardiovascular evaluation. The family history should be evaluated for the following: death of a first-degree relative before the age of 50 years that was attributed to heart disease; disability of a close relative before the age of 50 years due to heart disease; and a family history of HCM, dilated cardiomyopathy, long-QT syndrome or other ion channelopathies, Marfan syndrome, clinically significant arrhythmias, or other genetic cardiac conditions. A positive response to even one of these questions can be sufficient to require a referral for a more comprehensive cardiovascular evaluation. The examiner should take care to ensure the accuracy of the responses when appropriate. Finally, the physical examination should at minimum include the following: an assessment for organic murmurs with the patient in the supine and standing positions, an assessment of the femoral pulses and blood pressure, and a search for the physical signs and symptoms of Marfan syndrome.1,4 A screening tool based on these 14 elements is shown in Figure 1 that can assist examiners in completing the preparticipation screening of young athletes for cardiovascular risks.