New Insights Into the Arrhythmogenic Substrate of the Long QT Syndrome

A lot has transpired in our understanding of the inherited Long QT syndrome (LQTS) since the first description of a family with prolonged QT interval, deafness, and sudden death by Jervell and Lange-Nielsen in 19571 and a similar family reported in 1958 by Levine and Woodworth.2 Several children with LQTS and sudden death without deafness were reported by Romano et al in 19633 and Ward in 1964.4 Numerous LQTS case reports were published during the next several years, and the first specific therapy for this disorder involving antiadrenergic left cervicothoracic sympathetic ganglionectomy was reported in 1971,5 with β-blocker therapy introduced a few years thereafter. The Rochester-based International LQTS Registry was initiated in 1979. Using some patients from the LQTS Registry, Keating et al6 reported linkage of LQTS to the Harvey ras-1 locus on chromosome 11 in 1991, and within a few years Keating and associates identified the genes for LQT1, 2, and 3, which ushered in the extensive LQTS genotype-phenotype studies during the last 20 years. Various clinical studies identified the duration of the …