Genetic Testing in the Long QT Syndrome

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Abstract
The long QT syndrome (LQTS) is an inherited disease predisposing to cardiac arrhythmias and sudden death in young individuals. Two phenotypic variants have been described: the autosomal dominant Romano-Ward syndrome and the autosomal recessive Jervell and Lange-Nielsen syndrome.1-4 More recently, 2 additional uncommon variants presenting with prolonged QT interval and extracardiac manifestations were reported.5,6

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