Long QT Syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Natural History

About half the patients will have an arrhythmia that degenerates into Torsade de Pointes.

Complications

Long QT syndrome can result in fatal heart arrhythmias and death.

Prognosis

The Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive form of LQTS with associated congenital deafness. It is caused specifically by mutation of the KCNE1 and KCNQ1 genes

In untreated individuals with JLNS, about 50 percent die by the age of 15 years due to ventricular arrhythmias.