Prevalence of the Congenital Long-QT Syndrome

Abstract

Background-The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1:20 000 to 1:5000 were published, but none was based on actual data. Our objective was to define the prevalence of LQTS. Methods and Results-In 18 maternity hospitals, an ECG was performed in 44 596 infants 15 to 25 days old (43 080 whites). In infants with a corrected QT interval (QTc) Ͼ450 ms, the ECG was repeated within 1 to 2 weeks. Genetic analysis, by screening 7 LQTS genes, was performed in 28 of 31 (90%) and in 14 of 28 infants (50%)

Mutations in voltage-gated L-type calcium channel: implications in cardiac arrhythmia

“…Long QT syndrome (LQTS) is one of the most common cardiac electrophysiological diseases with an estimated prevalence of 1 in every 2,534 persons [49]. It is a typical cardiac repolarization abnormality defined by heart rate-corrected QT interval (QTc) prolongation on resting electrocardiogram (ECG) [4,50], and characterized by an increased trend for ventricular tachycardia with torsade de pointes (TdP).…”

The role of genetic testing in unexplained sudden death

“…Congenital LQTS has a prevalence of approximately 1:2000 71 and is characterized by prolongation of the QT interval on the electrocardiogram (ECG) and increased risk of the characteristic ventricular arrhythmia, Torsades de Pointes, and SCD. Hundreds of mutations in 14 genes 25 predominantly encoding components and channel interacting proteins of repolarizing potassium currents and the sodium channel current (Nav1.5) have been identified (Table I).…”

Clinical utility gene card for: Long-QT Syndrome (types 1–13)

“…If known to be variable between ethnic groups, please report): 1:2,000 individuals in the general population. 4 It may be assumed that the prevalence is of comparable magnitude in different populations.…”

“…Beckmann and Kääb had received grants from ANR SCD Gene (01 KU 0907), M 4 Innovative Therapiestrategien für Herzrhythmusstörungen und Drug Safety (01 EX 1021 E) and NGFN Plus of the BMBF (01 GS 0838). Kääb is a Principal Investigator of the Munich Heart Alliance and receives grants from the DZHK (German Centre for Cardiovascular Research).…”