Evidence of genetic heterogeneity in Romano-Ward long QT syndrome. Analysis of 23 families.

Evidence of genetic heterogeneity in Romano-Ward long QT syndrome. Analysis of 23 families.

BACKGROUND The Romano-Ward long-QT Syndrome (LQTS) is an autosomal dominant inherited trait characterized by prolonged QT interval on ECG, life-threatening arrhythmias, syncope, and sudden death in affected individuals. A gene responsible for this disorder has been shown to be linked to the Harvey ras-1 locus (H-ras-1) DNA marker on the short arm of chromosome 11 (11p) in 7 families. The purpos...

Romano-Ward Long QT Syndrome Analysis of 23 Families

Background The Romano-Ward long-QT Syndrome (LQTS) is an autosomal dominant inherited trait characterized by prolonged QT interval on ECG, life-threatening arrhythmias, syncope, and sudden death in affected individuals. A gene responsible for this disorder has been shown to be linked to the Harvey ras-1 locus (H-ras-1) DNA marker on the short arm of chromosome 11 (llp) in 7 families. The purpos...

A recessive variant of the Romano-Ward long-QT syndrome?

BACKGROUND The congenital long-QT syndrome (LQTS) is a genetically heterogeneous disease characterized by prolonged ventricular repolarization and life-threatening arrhythmias. Mutations of the KVLQT1 gene, a cardiac potassium channel, generate two allelic diseases: the Romano-Ward syndrome, inherited as a dominant trait, and the Jervell and Lange-Nielsen syndrome, inherited as an autosomal rec...

Evidence of genetic and phenotypic heterogeneity in the Romano-Ward syndrome.

We report two families with phenotypically different forms of Romano-Ward syndrome. In one family, only five of 18 affected subjects are symptomatic, whereas in the other the proportion is three out of five. The families show distinct ECG morphologies, in addition to QT prolongation. Previous reports have shown genetic linkage either to the HLA locus on chromosome 6 or the Harvey-ras oncogene o...

Evidence of genetic heterogeneity in the long QT syndrome.