Update on long QT syndrome

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Date
2019
Authors
Neira, Victor
Enriquez, Andrés
Simpson, Chris
Baranchuk, Adrian
Keyword
Electrocardiography , Long QT , QT Interval , Torsades de Pointes
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.