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Review
. 2019 Dec;30(12):3068-3078.
doi: 10.1111/jce.14227. Epub 2019 Oct 14.

Update on long QT syndrome

Víctor Neira  1 , Andrés Enriquez  1 , Chris Simpson  1 , Adrian Baranchuk  1
Affiliations
Review

Update on long QT syndrome

Víctor Neira et al. J Cardiovasc Electrophysiol. 2019 Dec.

Abstract

Long QT syndrome (LQTS) is an inherited disorder characterized by a prolonged QT interval in the 12-lead electrocardiogram and increased risk of malignant arrhythmias in patients with a structurally normal heart. Since its first description in the 1950s, advances in molecular genetics have greatly improved our understanding of the cause and mechanisms of this disease. Sixteen genes linked to LQTS have been described and genetic testing had become an integral part of the diagnosis and risk stratification. This article provides an updated review of the genetic basis, diagnosis, and clinical management of LQTS.

Keywords: QT interval; electrocardiography; long QT; torsades de pointes.

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References

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