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Arthur J. Moss, MD

JAMA. 2003;289:2041-2044.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

INTRODUCTION

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).

Table appears in full text version. Table 1. Molecular Genetics of Long QT Syndrome (LQTS)*

LQTS Genotypes

Ventricular repolarization is determined by cardiac . . . [Full Text of this Article]

Clinical Diagnosis of LQTS

ECG Findings in LQTS

Triggers for Cardiac Events

Clinical Course of LQTS

Evaluation of Patients With Suspected or Definite LQTS

Therapy for LQTS

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

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