Romano-Ward Long QT Syndrome: Identification of the Gene Responsible for Cardiac Arrhythmia in the First Autosomal Dominant LQT Family, the Ward Kindred • 149

Abstract

Background: Long QT syndrome (LQT) is an inherited cardiac arrhythmia that causes sudden death in young, previously healthy individuals. In 1964, the first family of autosomal dominant LQT was reported by OC Ward. Since then, four genes for autosomal dominant LQT were mapped to chromosome 11p 15.5 (LQT1), 7q35-36 (LQT2), 3p21-24 (LQT4), and 4q25-27 (LQT4). Genes for LQT1, LQT2, and LQT3 have been identified as KVLQT1, HERG, SCN5A, respectively. However, the gene responsible for the first autosomal dominant LQT family is unknown. We hypothesized that a mutation in either KVLQT1, HERG, or SCN5A would be responsible for the cardiac arrhythmia in this family.