Long QT Syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]
Overview
About half the patients with long QT syndrome will have an arrhythmia that degenerates into Torsade de Pointes that may terminate spontaneously or may end in sudden cardiac death. Nevertheless, some subjects with congenital long QT syndrome can remain asymptomatic throughout their lives.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of long QT syndrome typically develop after exposure to some medication including some anti-arrhythmic drugs, some antipsychotic drugs, such as Haloperidol and Ziprasidone.
Complications
- Long QT syndrome can result in the fatal heart arrhythmias and death. Certain medications can increase the risk of fatal arrhythmias and death in persons with long QT syndrome.[1]
Prognosis
- People who are treated with lifestyle modifications and medications live longer than those who are not. For people who are not treated, half of them, mostly those with the inherited form of long QT syndrome, will die within 10 years.[2]
- 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
- ↑ Crotti L, Celano G, Dagradi F, Schwartz PJ (July 2008). "Congenital long QT syndrome". Orphanet J Rare Dis. 3: 18. doi:10.1186/1750-1172-3-18. PMC 2474834. PMID 18606002.
- ↑ Schwartz PJ, Crotti L, Insolia R (August 2012). "Long-QT syndrome: from genetics to management". Circ Arrhythm Electrophysiol. 5 (4): 868–77. doi:10.1161/CIRCEP.111.962019. PMC 3461497. PMID 22895603.