Idiopathic Long QT Syndrome and QT Duration in Congenital Sensori-Neural Hearing Loss Students of Van School for the Deaf.
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2009
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Gümrükçüoğlu H.A, Van yöresindeki doğumsal İşitme engelli çocuklarda QT süresi ve Uzun QT Sendromu.Giriş: Uzun QT sendromu (UQTS) nadir kalıtımsal bir hastalık olup doğumsal işitme engelli bireylerdeki sıklığı 0,21% olarak rapor edilmiştir. UQTS hastaları tekrarlayan bayılma ve ölümcül ventriküler aritmiler için yüksek risk altındadırlar. Yine, doğumsal işitme engelli bireylerde QT süresinin sağlıklı bireylere göre daha uzun olduğu rapor edilmiştir.Amaç: Van ve çevre illerdeki doğumsal işitme engelli çocukların QT süresi değerlendirmek, UQTS sıklığını araştırmak, klinik ve elektrokardiyografik olarak çocukların uzun dönem takibini yapmaktır.Metot: Doğumsal işitme engelli olan 127 çocuk ve 50 tane aynı yaş gurubunda sağlıklı çocuk kontrol grubu olarak çalışmaya dâhil edildi. Tüm çocuklar kardiyolog tarafından fizik muayeneden geçirildi. Elektrokardiyografileri (EKG) çekildi. Deneyimli bir kardiyolog tarafından D2 veya V5 derivasyonları kullanılarak QTc değeri hesaplandı. QTc değeri >430 msn olanlara doğrulamak için tekrar EKG çekildi. QTc dispersiyonu (QTcD) herhangi bir derivasyondaki maksimum ve mininmum QTc süresi arasındaki fark olarak alındı. UQTS tanısı için Schwartz kriterleri kullanıldı. UQTS şüphesi olan bireylere (QTc>430msn) ekokardiyografi ve 24 saat ambulatuvar EKG kaydı yapıldı. İşitme engelliler ile kontrol grubunun EKG bulguları (kalp hızı, QTc, QTcD) karşılaştırıldı.Bulgular: Schwartz kriterlerine göre 1 çocuğa UQTS tanısı konuldu. Toplam 16 çocuğun EKG'sinde QTc değeri >430 msn olduğu için UQTS şüphesisle tekrar EKG çekildi. Bu çocukların ekokardiyografilerinde yapısal veya fonksiyonel kap hastalığına rastlanmadı. 24 saat ambulatuvar EKG kaydında patolojik bulguya rastlanmadı. QTc değeri >430 msn olan çocukların, Schwart kriterlerine göre aldığı puanlar 1,5-4 arasındaydı. QTcmax ve QTcD değeri işitme engelliler grubunda kontrol grubuna göre anlamlı olarak daha uzundu (397,82±26,56 msn karşı 384,9±19,2 msn <0,001 ve 40,54±8,4msn ye karşı 33,54±4,6 msn <0,001). İki yıllık takipte anlamlı EKG değişikliği ve klinik olay gözlenmedi.Sonuç: İşitme engelli grubunda UQTS sıklığı düşüktü (0,8%). QTcmax ve QTcD değeri kontrol grubuna göre işitme engelli grubunda daha uzundu fakat bunun klinik önemi belirsizdi.
Gümrükçüoğlu H.A, İdiopathic long QT syndrome and QT duration in congenital sensori-neural hearing loss students of Van School for the Deaf.Background: Long QT syndrome (LQTS) is a rare inherited disease with a reported prevalence of 0.21 per cent in children with congenital deafness. These patients carry a high risk of recurrent syncope and fatal ventricular arrhythmia. Nevertheless long QT duration reported that in children with congenital deafness according to healthy children.Purpose: To evaluate the QT duration and search frequency of LQTS in children with congenital deafness and to the long term follow up of clinical and electrocardiographic (ECG) findings in deaf children at Van and near villages.Methods: The study included a total of 127 children with congenital sensori-neural hearing loss attending Van School for Deafs and 50 healthy children. A questionnaire was employed and all children were examined by a cardiologist, 12-lead ECGs were obtained and QTc intervals were measured using standard methods in leads D2 and V5. If QTcmax interval is prolonged (>430msn), an additional control ECG were obtained for confirmation. QT dispersion (QTD) was determined as the difference between the maximum and minimum QTc intervals. Schwartz criteria were used to identify index cases with LQTS. Patients with prolonged QTcmax were also evaluated by transthoracic echocardiographic examination and 24 hour ambulatory ECG holter monitoring.Results: LQTS was defined by Schwartz criteria in 1 (0.8%) child. QTcmax was prolonged in 16 children (QTcmax>430 msn). There were no abnormalities on echocardiography and 24-hour ambulatory ECG record. Compared to healthy control group QTcmax and QTcD were significantly longer in deaf children (397,82±26,56 vs. 384,9±19,2 ms, p<0,001 and 40,54±8,4 vs 33,54±4,6 ms, p<0,001, respectively). During two years of follow-up, we didn?t observe a clinical event and there were no significant changes in ECG findings.Conclusion: Frequency of LQTS is low in children with congenital deafness (0,8%). Although, QTcmax and QTcD were prolonged in children with congenital deafness the clinical relevance of this finding is not clear
Gümrükçüoğlu H.A, İdiopathic long QT syndrome and QT duration in congenital sensori-neural hearing loss students of Van School for the Deaf.Background: Long QT syndrome (LQTS) is a rare inherited disease with a reported prevalence of 0.21 per cent in children with congenital deafness. These patients carry a high risk of recurrent syncope and fatal ventricular arrhythmia. Nevertheless long QT duration reported that in children with congenital deafness according to healthy children.Purpose: To evaluate the QT duration and search frequency of LQTS in children with congenital deafness and to the long term follow up of clinical and electrocardiographic (ECG) findings in deaf children at Van and near villages.Methods: The study included a total of 127 children with congenital sensori-neural hearing loss attending Van School for Deafs and 50 healthy children. A questionnaire was employed and all children were examined by a cardiologist, 12-lead ECGs were obtained and QTc intervals were measured using standard methods in leads D2 and V5. If QTcmax interval is prolonged (>430msn), an additional control ECG were obtained for confirmation. QT dispersion (QTD) was determined as the difference between the maximum and minimum QTc intervals. Schwartz criteria were used to identify index cases with LQTS. Patients with prolonged QTcmax were also evaluated by transthoracic echocardiographic examination and 24 hour ambulatory ECG holter monitoring.Results: LQTS was defined by Schwartz criteria in 1 (0.8%) child. QTcmax was prolonged in 16 children (QTcmax>430 msn). There were no abnormalities on echocardiography and 24-hour ambulatory ECG record. Compared to healthy control group QTcmax and QTcD were significantly longer in deaf children (397,82±26,56 vs. 384,9±19,2 ms, p<0,001 and 40,54±8,4 vs 33,54±4,6 ms, p<0,001, respectively). During two years of follow-up, we didn?t observe a clinical event and there were no significant changes in ECG findings.Conclusion: Frequency of LQTS is low in children with congenital deafness (0,8%). Although, QTcmax and QTcD were prolonged in children with congenital deafness the clinical relevance of this finding is not clear
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Kardiyoloji, Kulak Burun ve Boğaz, Qt, Işitme Engelliler, Cardiology, Otorhinolaryngology (ear-Nose-Throat), Qt, Hearing Impaired
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