Uzun QT sendromları

Gökhan Bektaşoğlu; Mehmet Yılmaz; Okan Turgut; İzzet Tandoğan

EN TR

Uzun QT sendromları

Abstract

Özet

Konjenital uzun QT sendromu (LQTS), membran iyon kanallarındaki yapısal ve fonksiyonel defektlere bağlı olduğunu göstermiştir. Günümüzde konjenital uzun QT sendromlarının 12 farklı alt tipi tanımlanmıştır.Konjenital LQTS' nin ana tipleri; otozomal dominant geçişli Romano-Ward ve otozomal resesif geçişli Jervell ve Lange-Nielsen sendromlarıdır. Akkiz LQTS konjenital forma kıyasla daha sık görülmektedir. Sendromun akkiz formu genellikle ilaçlara, elektrolit bozukluklarına, bradikardiye, toxinlere ve miyokardiyal iskemi gibi faktörlere bağlıdır. Ani kardiyak ölüm, senkop ve presenkop, sendromun Torsade de pointes nedeniyle gelişen klinik özelliklerini oluşturmaktadır. Akkiz formda tedavi sorumlu ajanın kesilmesi, elektrolit bozukluklarının giderilmesi gibi nedene yöneliktir. Konjenital LQTS de ise beta adrenoreseptör blokerleri tedavideki ilk basamaktır. Beta-blokerlere cevap alınamayan hastalarda servikal torasik sempatektomi, kalp pili ve ICD alternatif tedavi yöntemleridir.

Anahtar sözcükler: Uzun QT sendromu, Romano Ward sendromu, Jervell ve Lange-Nielsen sendromu, torsades de pointes, iyon kanalları.

Abstract

Long QT syndrome (LQTS) is related to the functional and structural defects in the membrane ion channels. Untill so far, 12 subtype of LQT syndrome was defined. The main types of congenital LQTS are Romano-Ward syndrome and Jervell and Lange-Nielsen syndrome. Acquired form of LQTS is more frequent according to the congenital LQTS. Acquire form of the syndrome is mainly secondary to drugs, electrolyte imbalances, bradycardia, toxins and myocardial ischemia. Sudden cardiac death, syncope and presyncope are the clinical features which are caused by Torsade de pointes. Treatment in acquired form should focus on the reason: removal of the causative agent, correcting electrolyte imbalance and so forth. However beta adrenoreceptor blockers is the first step in treatment of congenital LQTS. In patients with no response to beta-blockers; cervical thoracic sympathectomy, cardiac pacing and ICDs are alternative treatment modalities.

Keywords: Long QT syndrome, Romano Ward syndrome, Jervell and Lange-Nielsen syndrome, torsades de pointes, ionic channels

Keywords

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March 24, 2009

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March 24, 2009

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Year 1970 Volume: 31 Number: 4

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1.Bektaşoğlu G, Yılmaz M, Turgut O, Tandoğan İ. Uzun QT sendromları. CMJ. 2009;31(4):487-501. https://izlik.org/JA73FT94KF

Uzun QT sendromları

Abstract

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Uzun QT sendromları

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Anahtar Kelimeler

References

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