Risk of Aborted Cardiac Arrest or Sudden Cardiac Death During Adolescence in the Long-QT Syndrome

Abstract

The hereditary long-QT syndrome (LQTS) is characterized by prolonged ventricular repolarization and an increased risk for ventricular tachyarrhythmias (torsades de pointes) and sudden cardiac death.¹ The clinical course of LQTS is influenced by many factors, including sex,^2-4 congenital deafness,⁵ prior cardiac events,^1,6 family history,⁷ QT-interval length,^1,2,4,7,8  and genotype.^3,4,9,10 Previous investigations have evaluated the effect of these factors on cardiac events (syncope, aborted cardiac arrest, and sudden cardiac death), with syncope the predominant end point in almost all of the prior studies.