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Long QT syndrome (LQTS) is an inherited primary arrhythmia syndrome associated with life-threatening ventricular arrhythmias and sudden death. This study aimed to report the clinical and genetic characteristics and outcomes of children diagnosed as having LQTS in a tertiary pediatric cardiology center in Turkey. This was a retrospective review of pediatric patients diagnosed as having LQTS at our center from January 2011 to April 2020. A total of 145 patients (76 males) were included, with a mean age of 9.2±4.5 years and a mean weight of