congenital long-QT syndrome

congenital long-QT syndrome

A rare autosomal dominant heart disorder causing prolongation of the QT interval in the ECG with ventricular arrhythmias, syncope, and the risk of sudden death. The most common forms, types 1 and 2, are caused by mutations respectively in the potassium channel genes KCNQ1 and KCNH2. Alleles for the syndrome are transmitted to daughters more often than to sons.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
References in periodicals archive ?
Crotti et al., "Prevalence of the congenital long-qt syndrome," Circulation, vol.
Diagnostic miscues in congenital long-QT syndrome. Circulation.
The warning advised against using ondansetron in patients with congenital long-QT syndrome, and recommended ECG monitoring for certain patients on ondansetron, including those with electrolyte abnormalities.
Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 2008;117:2184-91.
Crotti etal., "Prevalence of the congenital long-QT syndrome," Circulation, vol.
Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation 2000; 101:616-623.
Epinephrine QT stress testing in the evaluation of congenital long-QT syndrome: diagnostic accuracy of the paradoxical QT response.
Other areas investigated include the origin and role of N-homocysteinylated proteins in cardiovascular disease, the potential role of biomarkers in diagnosing the risk of sudden cardiac death, and pharmacological therapy in children with congenital long-QT syndrome. Information on the editor is not given.
Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome. Findings from the international LQTS registry.
Effectiveness and limitations of [Beta]-blocker therapy in congenital long-QT syndrome. Circulation.

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