Clinical Aspects of Type 3 Long-QT Syndrome
نویسندگان
چکیده
منابع مشابه
Revealing the Concealed Nature of Long-QT Type 3 Syndrome.
BACKGROUND Gain-of-function mutations in the voltage-gated sodium channel (Nav1.5) are associated with the long-QT-3 (LQT3) syndrome. Nav1.5 is densely expressed at the intercalated disk, and narrow intercellular separation can modulate cell-to-cell coupling via extracellular electric fields and depletion of local sodium ion nanodomains. Models predict that significantly decreasing intercellula...
متن کاملThe congenital long QT syndrome Type 3: An update
Congenital long QT syndrome type 3 (LQT3) is the third in frequency compared to the 15 forms known currently of congenital long QT syndrome (LQTS). Cardiac events are less frequent in LQT3 when compared with LQT1 and LQT2, but more likely to be lethal; the likelihood of dying during a cardiac event is 20% in families with an LQT3 mutation and 4% with either an LQT1 or an LQT2 mutation. LQT3 is ...
متن کاملGenetic analysis of Brugada syndrome and congenital long-QT syndrome type 3 in the Chinese
BACKGROUND Brugada syndrome and congenital long-QT syndrome (LQTS) type 3 (LQT3) are 2 inherited conditions of abnormal cardiac excitability characterized clinically by an increased risk of ventricular tachyarrhythmias. SCN5A gene that encodes the cardiac sodium channel α subunit is responsible for the 2 diseases, and more work is needed to improve correlations between SCN5A genotypes and assoc...
متن کاملRanolazine for Congenital Long-QT Syndrome Type III: Experimental and Long-Term Clinical Data.
BACKGROUND The basic defect in long-QT syndrome type III (LQT3) is an excessive inflow of sodium current during phase 3 of the action potential caused by mutations in the SCN5A gene. Most sodium channel blockers reduce the early (peak) and late components of the sodium current (INa and INaL), but ranolazine preferentially reduces INaL. We, therefore, evaluated the effects of ranolazine in LQT3 ...
متن کاملLong QT Syndrome
Background. Erythromycin is known to prolong ventricular repolarization and has been associated with the occurrence of torsades de pointes. In this study, we have investigated potential mechanisms in vivo and in vitro for induction of an acquired long QT syndrome by erythromycin. Methods and Results. Ventricular electrograms and endocardial monophasic action potentials were recorded in anesthet...
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ژورنال
عنوان ژورنال: Circulation
سال: 2016
ISSN: 0009-7322,1524-4539
DOI: 10.1161/circulationaha.116.021823