The long-QT syndrome (LQTS) is a familiar disease characterized by abnormal myocardial repolarization and a high risk of sudden cardiac death. As a hallmark of the disease, the heart-rate corrected QT interval is intrinsically prolonged. Recent advances in molecular genetics have elicited that various inborn defects in cardiac ion channel genes regulating cardiac ion currents underlie this propensity to develop malignant ventricular arrhythmias. Meanwhile, a widespread locus and allelic genetic heterogeneity in LQTS is evident, thus, complicating the power of DNA diagnostic tools. The following review will briefly summarize clinical and genetic aspects of LQTS.
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