The Long QT Syndrome: A Review and Mortality Analysis

Objective .- To provide a recent review of literature pertinent to the assessment of life risk for individuals with a history of long QT syndrome (LQTS) and assess the mortality risk of a subset of these patients. Methods .- Standard comparative mortality techniques were employed to analyze relevant survival curves. Results .- Mortality risk in LQTS varies by age, sex, QTc interval length, genotype, and history of symptoms. A narrowly-defined subgroup of LQTS patients, those over age 20 with no history of syncope prior to age 20 and a QTc of less than 500ms, have an excess death rate of 0.5 - 2.0 per thousand per year. Conclusions .- Though the risk of sudden cardiac death in those with a history of long QT syndrome persists, there are subgroups of patients for whom the excess risk is lower than has been reported for this group of patients as a whole.