Long QT Syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]
Overview
Natural History, Complications and Prognosis
Risk stratification
The risk for untreated LQTS patients having events (syncopes or cardiac arrest) can be predicted from their genotype (LQT1-8), gender and corrected QT interval.[1]
- High risk (>50%)
QTc>500 msec LQT1 & LQT2 & LQT3(males)
- Intermediate risk (30-50%)
QTc>500 msec LQT3(females)
QTc<500 msec LQT2(females)& LQT3
- Low risk (<30%)
QTc<500 msec LQT1 & LQT2 (males)
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.
References
- ↑ Risk Stratification in the Long-QT Syndrome: N Engl J Med 2003; 349:908-909, Aug 28, 2003. PMID 12944579.