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April 23/30, 2003

Long QT Syndrome

Author Affiliations

Author Affiliation: Cardiology Unit, Department of Medicine, University of Rochester Medical Center, Rochester, NY.

JAMA. 2003;289(16):2041-2044. doi:10.1001/jama.289.16.2041

The long QT syndrome (LQTS) was first described in 1957 in a family in which several children with congenital bilateral neural deafness and QT prolongation on electrocardiogram (ECG) experienced recurrent syncope and sudden death, with a family pattern that suggested autosomal recessive inheritance (Jervell and Lange-Nielsen syndrome).1 A similar and much more common familial disorder with QT prolongation but without deafness was described a few years later, with family patterns that suggested autosomal dominant inheritance (Romano-Ward syndrome). These reports highlighted the familial nature of this QT prolongation disorder, and subsequent studies identified malignant ventricular arrhythmias as the cause of syncope and sudden death in patients with LQTS. The molecular-genetic basis of LQTS was discovered in the 1990s and, currently, mutations have been identified in 7 LQTS genes (Table 1).

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