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Long QT Syndrome 4

Presentation

This was initially shown with the H558R polymorphism of SCN5A, present in up to 30% of the population. [revespcardiol.org]

Education Plain Text - 2015 Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan 1999 - 2005 Hamamatsu University of Medicine, Hamamatsu, Japan Awards & Honors Plain Text Oct 2018 A novel CACNA1C mutation(R860Q) in a family presented [researchmap.jp]

Inappropriate pacing inhibition triggered by QT prolongation due to t wave oversensing in an icd recipient presenting with long QT syndrome. Internal medicine. 2011;50:1021-1024 34. [med.niigata-u.ac.jp]

Treatment

Although it is clear that treatment should be established when there are symptoms, the approach to use in asymptomatic patients is still under debate. [revespcardiol.org]

Prognosis

Two-to-one AV block is an infrequent manifestation with a poor prognosis that can be present since the fetal stage in the form of persistent bradycardia. [revespcardiol.org]

Etiology

It presents when the QT interval is prolonged, regardless of the etiology. It is a polymorphic ventricular tachycardia due to reentry, characterized electrocardiographically by continuous twisting of the QRS axis around an imaginary line. [revespcardiol.org]

Prevention

Frequencies programmed below 70 beats/min117 are not useful for preventing ventricular arrhythmia. [revespcardiol.org]

- prevention of vagal nerve activation-induced shortening of the atrial action potential duration. Circ J. 2010;74:895-902 8. Izumi D, Chinushi M, Iijima K, Ahara S, Komura S, Furushima H, Hosaka Y, Sanada A, Yagihara N, Aizawa Y. [med.niigata-u.ac.jp]

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