INTRODUCTIONLong QT syndrome (LQT) is a pro‐arrhythmogenic condition that increases the risk of a unique life‐threatening polymorphic ventricular tachycardia known as “torsades de pointes,” and sudden cardiac death (SCD). Congenital LQTs are inherited disorders caused by mutations in cardiac conduction channels or associated proteins, and are estimated to affect 0.005% to 0.05% of the general population. LQT is also accounted for by QT prolonging drugs, electrolyte abnormalities, ischemic heart disease or structural heart disease; a condition that is often referred to as acquired LQT. With the advent of genome sequencing it is evident that genetic variants with small effects also account for some, if not all, subclinical acquired LQTs that manifest themselves in the presence of additional precipitating factors. It has also been estimated that 10% to 36% of patients with LQT genotypes are silent mutation carriers.To this date 15 different types of congenital LQT have been characterized and account for about 80% of inherited long QT cases. These correspond to mutations in genes encoding potassium channels, calcium channels, calcium signaling proteins, anchoring proteins, transport proteins, and voltage‐gated sodium channels. Most of these genes have been established as casual genes for LQT based on linkage or segregation analysis, or
Clinical Genetics – Wiley
Published: Jan 1, 2018
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