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• W1992387760 abstract "It is well-known that the presence of an inherited or acquired long QT interval can be the cause of lifethreatening ventricular arrhythmias, in particular torsade de pointes (TdP), which often spontaneously interrupts causing presyncope or syncope. However, sometimes it can degenerate in ventricular fibrillation (VF) causing sudden death. Recently, a new genetic syndrome associated to a high risk of sudden death has been described. These subjects have a short QT interval at electrocardiogram (ECG) (b300 ms) with only slight modifications of the QT interval with heart rate changes, the T waves are often tall, peaked, and narrow-based. The patients have structurally normal hearts. These patients generally suffer from palpitations and syncope and may die suddenly even during childhood. More recently, Maury et al reported a family with short QT syndrome (SQTS) and a not so short corrected QT (QTc) interval (up to 340 F 20 ms). These data suggest that the definition of SQTS should be less restrictive. The substrate of ventricular arrhythmias responsible for sudden death has been suggested to be the presence of very short ventricular refractory periods and the heterogeneous shortening of the action potential (AP) duration among the different layers of the ventricular wall. Until now, mutations in 3 different genes have been identified in this syndrome: 2 different missense mutations, which provoke an amino acid change (N588K) in the S5-P loop region of the cardiac Ikr channel HERG (KCNH2); 2 different mutations in the KCNQ1 gene, which encodes the slow component of the cardiac delayed rectifier K+ current; and a mutation in the KCNJ2 gene. In all cases, there is an increase in the repolarizing currents leading to a" @default.
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• W1992387760 date "2007-01-01" @default.
• W1992387760 modified "2023-10-16" @default.
• W1992387760 title "Is acquired short QT due to digitalis intoxication responsible for malignant venticular arrhythmias?" @default.
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• W1992387760 doi "https://doi.org/10.1016/j.jelectrocard.2006.07.003" @default.
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