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

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References in periodicals archive

Crotti et al., "Prevalence of the congenital long-qt syndrome," Circulation, vol.

A Hiccup in Hiccup Management: Cardiac Arrest from Previously Undiagnosed Congenital Long QT Syndrome

Diagnostic miscues in congenital long-QT syndrome. Circulation.

4 EKG abnormalities: what are the lifesaving diagnoses?

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.

Drugs, pregnancy, and lactation: ondansetron--troubling data

Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome. Circulation 2008;117:2184-91.

JERVELL AND LANGE-NIELSEN SYNDROME IN DEAF SCHOOL CHILDREN POPULATION

Crotti etal., "Prevalence of the congenital long-QT syndrome," Circulation, vol.

Modelling human channelopathies using induced pluripotent stem cells: a comprehensive review

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.

Normal QT response during exercise testing and hyperventilation in children

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.

Horizons in world cardiovascular research; v.1

Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome. Findings from the international LQTS registry.

The importance of routine QT interval measurement in rhythm interpretation

Effectiveness and limitations of [Beta]-blocker therapy in congenital long-QT syndrome. Circulation.

Genotype and Severity of Long QT Syndrome