Until recently, sudden cardiac death in a young person often remained an unexplained tragedy. However, in the last decade there have been dramatic advances in medical knowledge regarding inheritable dysrhythmias that increase the risk of SCD in otherwise healthy young individuals. The primary mechanism in this group of dysrhythmias appears to be an alteration of cardiac repolarization. In some diseases, the specific genes affected and even precise cellular mechanisms have been identified. The information about these diseases is often complex and rapidly evolving, challenging both healthcare providers and the families who must make important decisions based on emerging and incomplete information. The purpose of this article is to describe current understanding of the repolarization-related dysrhythmias and discuss the clinical implications for advanced practice nurses.