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Long QT syndrome

Definition:

Long QT syndrome (LQTS) is a cardiovascular disorder resulting from mutations in cardiac ion channels. LQTS is characterized by prolongation of the QT interval in the electrocardiogram (ECG) and a propensity to torsades de pointes ventricular tachycardia frequently leading to syncope, cardiac arrest, or sudden death usually in young otherwise healthy individuals. Anesthesia in a patient with LQTS can trigger malignant arrhythmias and is therefore a high-risk procedure. There are two well-known syndromes with a long QT interval. The Romano-Ward syndrome is characterized by an autosomal-dominant inheritance without familial deafness. The Jervell and Lange-Nielsen syndrome (JLNS) has an autosomal-recessive pattern of inheritance and is associated with deafness.

References:

[1]. A Ilhan, et al. Jervell and Lange-Nielsen syndrome: neurologic and cardiologic evaluation. Pediatr Neurol. 1999 Nov;21(5):809-13. [Content Brief]

[2]. Ebru Aypar, et al. The effects of sevoflurane and desflurane anesthesia on QTc interval and cardiac rhythm in children. Paediatr Anaesth. 2007 Jun;17(6):563-7. [Content Brief]

[3]. Hyung Tae Kim, et al. Long QT syndrome provoked by induction of general anesthesia -A case report-. Korean J Anesthesiol. 2010 Dec;59 Suppl(Suppl):S114-8. [Content Brief]

[4]. Lia Crotti, et al. Calmodulin mutations associated with recurrent cardiac arrest in infants. Circulation. 2013 Mar 5;127(9):1009-17. [Content Brief]

[5]. Max Saussine, et al. Torsade de pointes during sevoflurane anesthesia in a child with congenital long QT syndrome. Paediatr Anaesth. 2006 Jan;16(1):63-5. [Content Brief]

[6]. Nicole J Boczek, et al. Spectrum and Prevalence of CALM1-, CALM2-, and CALM3-Encoded Calmodulin Variants in Long QT Syndrome and Functional Characterization of a Novel Long QT Syndrome-Associated Calmodulin Missense Variant, E141G. Circ Cardiovasc Genet. 2016 Apr;9(2):136-146. [Content Brief]

[7]. Wojciech Zareba, et al. Long QT syndrome and short QT syndrome. Prog Cardiovasc Dis. 2008 Nov-Dec;51(3):264-78. [Content Brief]

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