Congenital long-QT syndromes - Who's at risk for sudden cardiac death?

These 2 clinical series highlight the importance of long-term surveillance and data collection. The identification of risk factors to improve stratification of patients with more precision is crucial, because not all patients with LQTS will clearly benefit from invasive treatments. It is clear from the present series and others that the majority of patients can be managed safely and effectively with medical therapies and trigger avoidance. The selection of the high-risk patient who may benefit from interventional therapies such as left cardiac sympathetic denervation or cardiac rhythm management devices must be balanced carefully with the risk of arrhythmic sudden death. Congenital LQTS is clearly not 1 homogeneous syndrome but rather related syndromes with clinical, genetic, and phenotypic heterogeneity. The International LQTS Registry has proved invaluable for the rich data set that continues to provide interesting findings to help manage patients of all ages with LQTS. © 2008 American Heart Association.