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Long QT Syndrome

Arthur J. Moss, MD
JAMA. 2003;289(16):2041-2044. doi:10.1001/jama.289.16.2041.
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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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Figure. Twelve-Lead Electrocardiogram From a Patient With the LQT3 Genotype of Long QT Syndrome
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The electrocardiogram shows sinus bradycardia with a heart rate of 48/min, a QT interval of 0.68 seconds, and a corrected QT interval of 0.61 seconds. The long ST-segment interval before the onset of a late, prominent T wave is characteristic of the LQT3 genotype. The patient was not receiving β-blocker medication at the time this tracing was obtained.

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