Perspective:

The highest risk of LAE occurs between the third and fourth decade of life and rarely before adolescence. Therefore, the authors believe that screening for ARVC around age 11 or 12 years is reasonable. Prior studies appear to have underestimated LAEs due to analysis of data based on clinical history rather than through prospective clinical observation. In this analysis, male sex, participation in strenuous exercise, history of atrial fibrillation, syncope, and hemodynamically tolerated VT all may be harbingers of adverse events. In the absence of more sophisticated risk stratification schemes, it seems reasonable to recommend implantable cardioverter-defibrillator implantation in the presence of one or more of these risk factors. Neither medical therapy nor ablation appear to reduce the risk of adverse events, although that does not necessarily mean that there is no impact on the natural history of ARVC.