Purpose of Review <p>Sudden cardiac arrest (SCA) in a young athlete is a profoundly impactful and potentially tragic event. This review summarizes the epidemiology of SCA in athletes, evolving methods of carrying out the cardiac preparticipation evaluation (cPPE), and the appropriate identification and follow up care of athletes with positive cPPE screens. It also guides clinicians on decision making after a positive cPPE screen, provides an overview of shared decision-making following a new cardiac diagnosis, and emphasizes the importance of the emergency action plan (EAP).</p> Recent Findings <p>SCA is more common than historical estimates, and survival rates may be improving. cPPE strategies based on the history and physical exam alone have significant limitations. The resting electrocardiogram (ECG) is endorsed as an important component of the cPPE by some societies and leagues and is the most statistically powerful tool to achieve the goal of early detection of cardiac conditions. A comprehensive evaluation, inclusive of expert consultation and shared decision making, is recommended to determine participation status following a new cardiac diagnosis. Long-term follow up studies suggest athletic competition with a known cardiac diagnosis may be safe in some cases.</p> Summary <p>The optimal method of conducting the cPPE remains an area of intense debate. Regardless of method utilized, sports medicine clinicians should be able to accurately identify a positive screen based and understand the appropriate next steps based on modern ECG interpretation criteria and expert guidelines. No screening method is perfect, and the EAP will always remain a critical component of any safe athletic venture.</p>