Short QT interval syndrome

[摘要] Every year, sudden cardiac death (SCD) takes lives of 400 000 American citizens, and in 10-20% ofthe cases, fatal outcome is explained by hereditary pathology. Short QT interval syndrome remains rarely diagnosed, and virtually unknown, as it was described just recently. Its clinical course is characterized by syncope or fainting episodes, and SCD in patients with corrected QT interval <320 ms. In patients with short QT syndrome, syncope can be caused by atrial fibrillation (AF) paroxysms or ventricular arrhythmias. AF episodes are typically the first manifestation ofthe disease, being registered more often in children and adolescents; some cases in new-born babies have been described. According to genetic examination results, short QT syndrome is a hereditary pathology, explained by various mutations of potassium channel gene. Genetic polymorphism in short QT syndrome manifests during antiarrhythmic treatment. At present, the single effective therapeutic method for such patients is cardioverter-defibrillator implantation.

[关键词] sudden death;short qt interval syndrome;cardioverter-defibrillator implantation