United States43 although the European Society of Cardiology (ESC) has indeed made that recommendation.44 Although multiple grass roots local screening organizations in the United States have undertaken ECG screening endeavors in order to identify at-risk individuals, this strategy has not yet been endorsed by the American Heart Association or other screening organizations in the United States as a mass screening strategy/plan Ongoing work is continuing in this area Further refinement of ECG criteria in this setting in order to define true “normals” in a multicultural society in the United States is ongoing in an effort to identify abnormalities and in order to minimize false positives, a variable but important issue in the setting of mass ECG screening.45 So although mass ECG screening has not been endorsed by all, as described above, there continues to be a refinement of ECG criteria and a collection of screening data by multiple organizations There are unquestionable examples of this strategy resulting in lives saved This strategy is still debated and discussed As these data are collected, debated, and discussed, this approach hopefully will be sorted out with perhaps more definitive data with regard to its efficacy In the meantime, the logistics and resources needed to adopt an effective strategy on a mass basis will ideally be worked out as we determine the optimal strategy for primary prevention of sudden cardiac arrest and sudden cardiac death Echocardiography as a primary screening tool has also been suggested as either an independent approach or in combination with ECG screening.46 Just as with ECG screening, it is important that the echocardiographic studies be performed by an experienced technician and that the studies are interpreted by an experienced echocardiographer It is unlikely that a screening echocardiogram will uncover all of the at-risk anatomic abnormalities including and especially congenital coronary artery abnormalities Certainly, an echocardiogram will not allow for the diagnosis of electrical abnormalities/channelopathies The resources necessary and the performance and interpretation of screening echocardiograms has not made this strategy one that is easy to carry out and is certainly not currently recommended as a mass screening approach in and of itself Finally, and beyond the scope of this chapter, it is important to consider the importance of secondary prevention studies with regard to sudden cardiac death Although it would be the hope that the above strategies could prevent sudden cardiac arrest in all cases, it is very unlikely that such will be the case Therefore it is critically important that we advocate for education in cardiopulmonary resuscitation and use automatic external defibrillator (CPR-AED) for as many people as possible, particularly high school students and staff Such a strategy will allow for a population that will be able to recognize cardiac arrest and intervene by doing CPR and using an AED Such a strategy has been shown to increase the disappointingly low incidence of lay-rescuer CPR, as well as the very poor outcomes associated with out-of-hospital cardiac arrest.47–50 Screening First-Degree Relatives After Sudden Cardiac Death in the Young The incidence of sudden death has been reported between 2.7 and 7.5 events per 100,000 patient-years among young people (age 1 to 40 years) who were previously thought to be well Exact numbers remain unknown, but these rates would result in 10,000 children and young adults experiencing sudden unexplained death in the United States every year Italy, France, the Netherlands, and international communities have rolled out screening programs and registries.51,52 Most of these deaths ultimately will be attributed to cardiac causes A large, prospective population-based study from Australia and New Zealand diagnosed cardiomyopathy on autopsy in 16% of cases.53 This correlates closely with a Danish population-based registry with autopsy-based cardiomyopathy in 17% of cases.54 Arrhythmogenic causes of sudden death are harder to quantify because the hearts tend to be structurally normal or nearnormal; a potentially arrhythmogenic cause was present in 45% of cases in the large Australia/New Zealand series In addition, pathogenic or likely pathogenic variants in genes associated with congenital arrhythmia susceptibility and familial cardiomyopathy have been demonstrated in 3% to 35% of postmortem samples from cases of sudden unexplained death (see Table 89.1).53,55–71 Because these diseases may be inherited, screening for affected family members is critical In work from several centers, approximately 15% of families are found to be affected with a heritable cardiac disease when first-degree relatives receive screening after sudden death in the family.53,72,73 Screening of first-degree relatives after sudden unexplained death is particularly important because therapy can alter the natural history of these diseases among surviving family members Long QT syndrome has become the prototypical channelopathy because it has the highest known incidence rate in the population and because β-blocker therapy for primary prevention is associated with a 42% to 78% reduction in risk of aborted cardiac arrest or sudden cardiac death.74–77 Among the remaining channelopathies and cardiomyopathies there have been successes with various combinations of medications, left cardiac sympathetic denervation, ablation therapy, lifestyle modification, and avoidance of triggering medications The most common channelopathy and cardiomyopathy diseases are long and short QT syndrome,