Sudden death in adult congenital heart disease: Risk stratification in 2014

Arrhythmias and sudden death continue to plague a subset of adult patients with congenital heart disease. Despite investigative efforts spanning many decades, accurate identification of the high-risk patient remains challenging owing to a limited population size, relatively low event rate, and constantly evolving surgical approaches to the various malformations. Furthermore, until recently, most studies of the subject involved single-center formats with limited statistical power. The number of adult survivors has now reached a critical size where larger collaborative projects are beginning to generate more objective criteria for assessing risk. This review will provide an update on risk stratification for several of the major congenital cardiac lesions and outline the current recommendations for surveillance and management.