T‐Wave Morphology in Short QT Syndrome

Anttonen, Olli; Junttila, Juhani; Giustetto, Carla; Gaïta, Fiorenzo; Linna, Eeva; Karsikas, Mari; Seppänen, Tapio; Perkiömäki, Juha S.; Mäkikallio, Timo H.; Brugada, Ramón; Huikuri, Heikki V.

doi:10.1111/j.1542-474x.2009.00308.x

Cited by 27 publications

(18 citation statements)

References 14 publications

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“…However, the marked changes to T-wave morphology present in many patients means that there is an inherent risk in ICD use of inappropriate shock delivery, due to T-wave over-sensing [16,17,71,79]. Although this risk can be mitigated by ICD reprogramming [79], ICD use does not per se restore normal QT interval duration or rate dependence; also some patients (e.g., very young infants) may not be suitable for ICD implantation.…”

Section: Treatmentmentioning

confidence: 93%

The Short QT Syndrome

Hancox

McPate

Harchi

et al. 2011

Heart Rate and Rhythm

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Section: Treatmentmentioning

confidence: 93%

The Short QT Syndrome

Hancox

McPate

Harchi

et al. 2011

Heart Rate and Rhythm

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“…However, in contrast to measures of Jpoint-Tpeak, highamplitude T waves have not been shown to distinguish SQTS patients from healthy individuals with short QT intervals and no clinical events (43). Another morphological T-wave feature, the total cosine of the angle between the main vectors of the QRS and T-wave loops, has also been reported to be abnormal in SQTS patients (45). However, this parameter requires specific software for analysis, is not used routinely in clinical practice, and was not considered practical for use in diagnostic criteria.…”

Section: Figure 1 Cellular Mechanism Of Short Qt Intervalmentioning

confidence: 97%

The Short QT Syndrome

Gollob

Redpath

Roberts

2011

Journal of the American College of Cardiology

267

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“…Despite the success of this technique, there is an increased risk of an inappropriate shock discharged by the ICD [9, 11, 12, 14]. As the QT interval is not restored within the normal range over time, ICD is unsuitable for SQTS patients [12].…”

Section: Introductionmentioning

confidence: 99%

Effects of amiodarone on short QT syndrome variant 3 in human ventricles: a simulation study

2017

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BackgroundShort QT syndrome (SQTS) is a newly identified clinical disorder associated with atrial and/or ventricular arrhythmias and increased risk of sudden cardiac death (SCD). The SQTS variant 3 is linked to D172N mutation to the KCNJ2 gene that causes a gain-of-function to the inward rectifier potassium channel current (I K1), which shortens the ventricular action potential duration (APD) and effective refractory period (ERP). Pro-arrhythmogenic effects of SQTS have been characterized, but less is known about the possible pharmacological treatment of SQTS. Therefore, in this study, we used computational modeling to assess the effects of amiodarone, class III anti-arrhythmic agent, on human ventricular electrophysiology in SQT3.MethodsThe ten Tusscher et al. model for the human ventricular action potentials (APs) was modified to incorporate I K1 formulations based on experimental data of Kir2.1 channels (including WT, WT-D172N and D172N conditions). The modified cell model was then implemented to construct one-dimensional (1D) and 2D tissue models. The blocking effects of amiodarone on ionic currents were modeled using IC50 and Hill coefficient values from literatures. Effects of amiodarone on APD, ERP and pseudo-ECG traces were computed. Effects of the drug on the temporal and spatial vulnerability of ventricular tissue to genesis and maintenance of re-entry were measured, as well as on the dynamic behavior of re-entry.ResultsAmiodarone prolonged the ventricular cell APD and decreased the maximal voltage heterogeneity (δV) among three difference cells types across transmural ventricular wall, leading to a decreased transmural heterogeneity of APD along a 1D model of ventricular transmural strand. Amiodarone increased cellular ERP, prolonged QT interval and decreased the T-wave amplitude. It reduced tissue’s temporal susceptibility to the initiation of re-entry and increased the minimum substrate size necessary to sustain re-entry in the 2D tissue.ConclusionsAt the therapeutic-relevant concentration of amiodarone, the APD and ERP at the single cell level were increased significantly. The QT interval in pseudo-ECG was prolonged and the re-entry in tissue was prevented. This study provides further evidence that amiodarone may be a potential pharmacological agent for preventing arrhythmogenesis for SQT3 patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s12938-017-0369-0) contains supplementary material, which is available to authorized users.

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T‐Wave Morphology in Short QT Syndrome

Cited by 27 publications

References 14 publications

The Short QT Syndrome

The Short QT Syndrome

The Short QT Syndrome

Effects of amiodarone on short QT syndrome variant 3 in human ventricles: a simulation study

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