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Published By Oxford University Press

1532-2092, 1099-5129
Updated Sunday, 17 October 2021

EP Europace ◽  
2021 ◽  
Author(s):  
Meor Azraai ◽  
Daniel D’Souza ◽  
Yuan-Hong Lin ◽  
Voltaire Nadurata

Abstract Patients with cardiac implantable electronic devices (CIED) undergoing radiotherapy (RT) are more common due to the ageing of the population. With newer CIEDs’ implementing the complementary metal-oxide semiconductor (CMOS) technology which allows the miniaturization of CIED, it is also more susceptible to RT. Effects of RT on CIED ranges from device interference, device operational/memory errors of permanent damage. These malfunctions can cause life-threatening clinical effects. Cumulative dose is not the only component of RT that causes CIED malfunction, as neutron use and dose rate effect also affects CIEDs. The management of this patient cohort in clinical practice is inconsistent due to the lack of a consistent guideline from manufacturers and physician specialty societies. Our review will focus on the current clinical practice and the recently updated guidelines of managing patients with CIED undergoing RT. We aim to simplify the evidence and provide a simple and easy to use guide based on the recent guidelines.


EP Europace ◽  
2021 ◽  
Author(s):  
Xuehui Fan ◽  
Guoqiang Yang ◽  
Jacqueline Kowitz ◽  
Firat Duru ◽  
Ardan M Saguner ◽  
...  

Abstract Cardiovascular diseases are the main cause of sudden cardiac death (SCD) in developed and developing countries. Inherited cardiac channelopathies are linked to 5–10% of SCDs, mainly in the young. Short QT syndrome (SQTS) is a rare inherited channelopathy, which leads to both atrial and ventricular tachyarrhythmias, syncope, and even SCD. International European Society of Cardiology guidelines include as diagnostic criteria: (i) QTc ≤ 340 ms on electrocardiogram, (ii) QTc ≤ 360 ms plus one of the follwing, an affected short QT syndrome pathogenic gene mutation, or family history of SQTS, or aborted cardiac arrest, or family history of cardiac arrest in the young. However, further evaluation of the QTc ranges seems to be required, which might be possible by assembling large short QT cohorts and considering genetic screening of the newly described pathogenic mutations. Since the mechanisms underlying the arrhythmogenesis of SQTS is unclear, optimal therapy for SQTS is still lacking. The disease is rare, unclear genotype–phenotype correlations exist in a bevy of cases and the absence of an international short QT registry limit studies on the pathophysiological mechanisms of arrhythmogenesis and therapy of SQTS. This leads to the necessity of experimental models or platforms for studying SQTS. Here, we focus on reviewing preclinical SQTS models and platforms such as animal models, heterologous expression systems, human-induced pluripotent stem cell-derived cardiomyocyte models and computer models as well as three-dimensional engineered heart tissues. We discuss their usefulness for SQTS studies to examine genotype–phenotype associations, uncover disease mechanisms and test drugs. These models might be helpful for providing novel insights into the exact pathophysiological mechanisms of this channelopathy and may offer opportunities to improve the diagnosis and treatment of patients with SQT syndrome.


EP Europace ◽  
2021 ◽  
Author(s):  
Harshil Dhutia ◽  
Aneil Malhotra ◽  
Gherardo Finocchiaro ◽  
Sameer Parpia ◽  
Raghav Bhatia ◽  
...  

EP Europace ◽  
2021 ◽  
Author(s):  
Andrea Mazzanti ◽  
Alessandro Trancuccio ◽  
Deni Kukavica ◽  
Eleonora Pagan ◽  
Meng Wang ◽  
...  

Abstract Aims Risk stratification of patients with long QT syndrome (LQTS) represents a difficult task. In 2018, we proposed a granular estimate of the baseline 5-year risk of life-threatening arrhythmias (LAE) for patients with LQTS, based on the genotype (long QT syndrome Type 1, long QT syndrome Type 2, and long QT syndrome Type 3) and the duration of the QTc interval. We sought to externally validate a novel risk score model (1-2-3-LQTS-Risk model) in a geographically diverse cohort from the USA and to evaluate its performance and assess potential clinical implication of this novel model. Methods and results The prognostic model (1-2-3-LQTS-Risk model) was derived using data from a prospective, single-centre longitudinal cohort study published in 2018 (discovery cohort) and was validated using an independent cohort of 1689 patients enrolled in the International LQTS Registry (Rochester NY, USA). The validation study revealed a C-index of 0.69 [95% confidence interval (CI): 0.61–0.77] in the validation cohort, when compared with C-index of 0.79 (95% CI: 0.70–0.88) in the discovery cohort. Adopting a 5-year risk ≥5%, as suggested by the ROC curve analysis as the most balanced threshold for implantable cardioverter-defibrillator (ICD) implantation, would result in a number needed to treat (NNT) of nine (NNT = 9; 95% CI: 6.3–13.6). Conclusion The 1-2-3-LQTS-Risk model, the first validated 5-year risk score model for patients with LQTS, can be used to aid clinicians to identify patients at the highest risk of LAE who could benefit most from an ICD implant and avoid unnecessary implants.


EP Europace ◽  
2021 ◽  
Author(s):  
Lukoye Atwoli ◽  
Abdullah H Baqui ◽  
Thomas Benfield ◽  
Raffaella Bosurgi ◽  
Fiona Godlee ◽  
...  
Keyword(s):  

EP Europace ◽  
2021 ◽  
Author(s):  
Michael Glikson ◽  
Jens Cosedis Nielsen ◽  
Mads Brix Kronborg ◽  
Yoav Michowitz ◽  
Angelo Auricchio ◽  
...  

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