Implantable cardioverter-defibrillators in patients with long QT syndrome: a multicentre study.
Agnieszka Zienciuk-Krajka,Maciej Sterliński,Artur Filipecki,Radosław Owczuk,Jacek Bednarek,Maciej Kempa,Sławomir Sielski,Marcin Dziduszko,Przemysław Mitkowski,Jarosław Kaźmierczak,Jacek Kuśnierz,Dariusz Michałkiewicz,Sebastian Stec,Marcin Gułaj,Ryszard Majcherek,Andrzej Lubiński,Grzegorz Raczak +16 more
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TLDR
ICD therapy is effective in nearly half the patient population; however, the rates of early and late complica-tions are high and the number of unnecessary ICD shocks and reimplantation procedures may be lowered by modern programming and increased longevity of newer ICD generators.Abstract:
Background: Implantable cardioverter-defibrillator (ICD) therapy has been proven effective in the prevention of sudden cardiac death, but data on outcomes of ICD therapy in the young and otherwise healthy patients with long QT syndrome (LQTS) are limited. Aim: We sought to collect data on appropriate and inappropriate ICD discharges, risk factors, and ICD-related complications. Methods: All LQTS patients implanted with an ICD in 14 centres were investigated. Demographic, clinical, and ICD therapy data were collected. Results: The study included 67 patients (88% female). Median age at ICD implantation was 31 years (12–77 years). ICD indication was based on resuscitated cardiac arrest in 46 patients, syncope in 18 patients, and malignant family history in three patients. During a median follow-up of 48 months, 39 (58%) patients received one or more ICD therapies. Time to first appropriate discharge was up to 55 months. Inappropriate therapies were triggered by fast sinus rhythm, atrial fibrillation, and T-wave oversensing. No predictors of inappropriate shocks were identified. Risk factors for appropriate ICD therapy were: (1) recurrent syncope despite b-blocker treatment before ICD implantation, (2) pacemaker therapy before ICD implantation, (3) single-chamber ICD, and (4) noncompliance to b-blockers. In 38 (57%) patients, at least one complication occurred. Conclusions: ICD therapy is effective in nearly half the patient population; however, the rates of early and late complications are high. Although the number of unnecessary ICD shocks and reimplantation procedures may be lowered by modern programming and increased longevity of newer ICD generators, other adverse events are less likely to be reduced.read more
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[Subcutaneous implantable cardioverter-defibrillator in prevention of sudden cardiac death in Poland - opinion paper endorsed by the Polish Cardiac Society Working Group on Heart Rhythm].
Paweł Ptaszyński,Marcin Grabowski,Oskar Kowalski,Maciej Kempa,Przemysław Mitkowski,Andrzej Przybylski,Maciej Sterliński +6 more
TL;DR: The subcutaneous defibrillator (S-ICD) as discussed by the authors is an ICD with an electrode system that is placed entirely subcutaneously, outside the thoracic cavity.
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Children and young adults treated with transvenous and subcutaneous implantable cardioverter-defibrillators: a 22-year single-center experience and new perspectives
TL;DR: Entirely subcutaneous ICD for SCD prevention is a feasible and safe therapy in young recipients and severe TR might be a serious clinical problem in some patients.
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A novel mutation in KCNH2 yields loss-of-function of hERG potassium channel in long QT syndrome 2
Kai Gu,Duoduo Qian,Huiyuan Qin,Chang Cui,W C Hewith A Fernando,Daowu Wang,Juejin Wang,Kejiang Cao,Minglong Chen +8 more
TL;DR: Wang et al. as discussed by the authors identified a novel KCNH2 mutation W410R in the patient with long QT syndrome 2 (LQT2), but the electrophysiological functions of this mutation remain unknown.
References
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Journal ArticleDOI
Proarrhythmic effect of pacing mode reprogramation in a patient with a congenital long-QT syndrome.
TL;DR: A 34-year-old woman with a congenital long-QT syndrome (LQTS) type 2, implanted 10 years before with a single chamber implantable defibrillator (ICD) for secondary prevention, was admitted to the authors' hospital to remove the malfunctioning ventricular ICD lead.
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Saving lives in congenital long QT syndrome: who benefits from implantable cardioverter defibrillator therapy?
TL;DR: In this issue of the Journal, Zareba et al. report on the efficacy of ICD therapy in a high-risk LQTS population and a retrospective comparison of patients in the International Long QT Registry with a history of cardiac sympathetic denervation.
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Proarrhythmic effect of "reverse mode switch" in a patient with long-QT syndrome.
TL;DR: A 44-year-old female admitted due to appropriate ICD shock delivery after ventricular fibrillation (VF) was diagnosed with long-QT syndrome previously and had received a dual-chamber ICD for secondary prevention.
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Ventricular Fibrillation due to Automated Atrial Threshold Testing in a Patient with an Implantable Cardioverter Defibrillator
TL;DR: This case describes why one should be careful using algorithms in implantable cardioverter defibrillators and why these algorithms are introduced in almost every new generation of ICDs.