Alev ARSLAN, Sevcan ERDEM, Nazan ÖZBARLAS, Osman KÜÇÜKOSMANOĞLU

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

Amaç: Uzun QT Sendromu (LQTS) uzamış ventriküler repolarizasyon zamanı ve malign taşikardiye eğilim ile karakterizedir. Bu yazıda doğumsal LQTS tanısı almış 16 hastamızda 12 yıllık deneyimimizi sunduk. Gereç ve Yöntem: Çalışmamızda Çukurova Üniversitesi Pediatrik kardiyoloji bölümünde 2001-2013 yılları arasında konjenital olarak LQTS tanısı alan hastalar dahil edildi. Bulgular: 16 hastaya (6 kadın, 12 erkek) konjenital LQTS tanısı konuldu. Hastaların ortalama yaşı 10 (2.6-20 yaş) ortalama takip süresi ise 35 ay idi (11-120 ay). Ortalama doğrulanmış QT intervali 520 ms (470-590 ms) olarak ölçüldü. Teşhis esnasında onaltı hastadan dokuzunda senkop, konvülsiyon veya kalp krizi hikayesi varken üç hastaya yanlış epilepsi teşhisi konarak antiepiletik ilaçlarla tedavi edilmiş.Sonuç: Özellikle tüm aile bireylerinde yüzeyel EKG taramasının önemini ve bazen hastaların yanlış tanı alarak tekrarlayan dirençli nöbet olarak tedavi edildiklerini vurgulamak istedik.

Konjenital uzun QT sendromunda tanısal ve yönetimsel zorluklar: tek merkez deneyim

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.

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