Key result
Updated review summarizes LQTS diagnosis, clinical management, and genetic testing across 16 linked genes.
Why the study?
Advances in molecular genetics have improved understanding of LQTS, warranting an updated review of its genetic basis, diagnosis, and clinical management.
This review summarizes the genetic basis, diagnosis, and clinical management of long QT syndrome, highlighting the role of genetic testing in risk stratification.
Supports genetic testing for LQTS diagnosis and risk stratification; leaves open need for prospective outcome studies.
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.
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Neira et al. (2019) conducted a review in Long QT syndrome (LQTS). This updated review summarizes the genetic basis, diagnosis, and clinical management of long QT syndrome, highlighting the 16 genes linked to the disorder and the role of genetic testing.
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