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Update on long QT syndrome
Author(s) -
Neira Víctor,
Enriquez Andrés,
Simpson Chris,
Baranchuk Adrian
Publication year - 2019
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/jce.14227
Subject(s) - medicine , long qt syndrome , risk stratification , qt interval , genetic testing , disease , cardiology , intensive care medicine , bioinformatics , biology
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.