Increased awareness and understanding of the clinical presentations and electrocardiographic features of Long QT Syndrome are essential for physicians to identify this potentially fatal condition.
The LQTS is no longer the rare "zebra" whose purpose is to ensure that trainees recall that deafness and sudden cardiac death may be related (Jervell and Lange-Nielsen syndrome). Over the past 10 to 20 years, the number of cases of inherited LQTS (Romano-Ward syndrome) has increased dramatically. It is doubtful that this reflects a true increase in incidence of disease due to a greater rate of sporadic gene mutations occurring in the heart or because of a rising incidence of consanguinity. Rather, the "incidence" of LQTS has risen because of the emerging awareness of and respect for this electrical malady in the heart. Understanding the principal elements of the LQTS, knowing the types of presentations, and being able to identify its presence electrocardiographically will allow the astute physician to expose this silent killer.
Michael J. Ackerman (Wed,) conducted a review in Long QT Syndrome. Increased awareness and understanding of the clinical presentations and electrocardiographic features of Long QT Syndrome are essential for physicians to identify this potentially fatal condition.