Diagnostic and management difficulties in congenitally long QT syndrome: a single centre experience

Abstract

Purpose: Long QT Syndrome (LQTS) is characterized by prolonged ventricular repolarization and tendency to malignant tachyarrhythmia. We reported 16 patient diagnosed congenitally LQTS as a tertiary centre's 12 years experience. Material and Methods: Patients whom diagnosed as congenitally LQTS in Cukurova University Divison of Pediatric Cardiology between years 2001 to 2013 were included the study.Results: Sixteen patients (6 female, 12 male) were diagnosed as congenitally LQTS. Mean age of patients was 10 years (2.6-20 years), mean follow up period was 35 months (11-120 months). Mean corrected QT interval was measured 520 ms (470-590 ms). At the diagnosis nine of sixteen patients (56%) had syncope, convulsion or cardiac arrest history, and three of them were misdiagnosed as epilepsy and were treated with antiepileptic drugs as well.Conclusion: We want to underline the importance of electrocardiography monitoring at all family members and some patients who misdiagnosed as recurrent seizures.