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Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter–defibrillator (ICD) recipients. Indeed, sudden death of presumed arrhythmic etiology is the most frequent mode of demise in patients with surgically repaired tetralogy of Fallot, often in early-to-mid adulthood. Nevertheless, the overall annual incidence of sudden death is actually low. Therein lies the major challenge of risk stratification. This review highlights some of the complexities involved in risk stratifying patients with tetralogy of Fallot, underscoring the importance of avoiding oversimplification by means of a rigid algorithm that dictates therapy. To complement sound clinical judgment, a quantitative probabilistic approach is presented, which considers the body of literature from primary prevention ICD trials, risk factors identified by observational studies, the value of programmed ventricular stimulation and insights gleaned from studying ICD recipients with tetralogy of Fallot.  相似文献   
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Current medical guidelines have prompted implementation of increasing numbers of implantable rhythm devices, be they pacemakers, internal cardioverter–defibrillators or loop recorders. These devices rely on complex microcircuitry and use electromagnetic waves for communication. They are, therefore, susceptible to interference from surrounding electromagnetic radiation and magnetic energy. Hermetic shielding in metallic cases, filters, interference rejection circuits and bipolar sensing have contributed to their relative resistance to electromagnetic interference (EMI) in household and workplace environments. Device interactions have occurred in hospitals where EMI sources are ubiquitous, including radiation, electrocautery and MRI exposures. However, with rapidly evolving technology, devices and potential sources of EMI continue to change. This review provides a contemporary overview of the current state of knowledge regarding risks attributable to EMI; highlights current limitations of implantable rhythm devices; and attempts to distinguish myths from realities.  相似文献   
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Azimilide dihydrochloride is an antiarrhythmic drug with Vaughn Williams class III properties, which blocks both fast (IKr) and slow (IKs) components of the delayed rectifier cardiac potassium channel. The drug slows the heart rate slightly and, like other class III antiarrhythmic drugs, prolongs ventricular repolarization and thus, the QT interval. Unlike sotalol, another class III antiarrhythmic drug, azimilide does not exhibit reverse-use dependence, that is, its binding characteristics and effectiveness are not related to the heart rate. Azimilide is 85% bioavailable, reaches peak blood concentrations in 6–8 h and has a long elimination half-life of 114 h. Clinical trials have utilized once-daily dosing. These trials have tested the use of the drug for patients with supraventricular and ventricular arrhythmias.  相似文献   
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Background

Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.

Objectives

The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.

Methods

A total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31).

Results

Patients’ median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents.

Conclusions

Young BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered.  相似文献   
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Background: The occurrence ICD undersensing of ventricular fibrillation due to the presence of a pacing stimulus artifact (PSA) is in part related to the amplitude of the artifact recorded on the ICD rate sensing circuit. There is little comparative data regarding PSA amplitude recorded by commercial ICD rate-sensing circuits.Purpose: To compare PSA amplitude recorded by commercial endovascular defibrillation leads utilizing integrated or true bipolar sensing circuits.Methods: Nineteen large (60–120 kg) pigs were utilized. Two different commercial endovascular defibrillation leads were evaluated, each with its distal tip located at the right ventricular apex: (1) Medtronic Transvene; and (2) CPI Endotak. Three different rate-sensing circuits were evaluated: (1) Transvene true bipolar (tip-ring); (2) Transvene integrated bipolar (tip-coil); and (3) Endotak integrated bipolar (tip-coil). Using a separate pacing lead located at the left ventricular apex (n = 19 animals) or right ventricular outflow tract (n = 10 animals), pacing was performed at a pulse width of 0.5 milliseconds at outputs of 1.5, 5 and 10 volts. PSA amplitude was recorded at each output by each circuit.Results: During pacing from the left ventricular apex, at each pacing output voltage the PSA amplitude recorded by the true bipolar circuit (0.6 ± 0.1 mV at 1.5 volts, 2.0 ± 0.5 mV at 5 volts, 3.7 ± 0.8 mV at 10 volts) was significantly smaller than recorded by the Transvene integrated circuit (1.4 ± 0.3 mV at 1.5 volts, 3.8 ± 0.7 mV at 5 volts, 4.1 ± 0.8 mV at 10 volts) or the Endotak integrated circuit (1.8 ± 0.4 mV at 1.5 volts, 4.2 ± 1.0 mV at 5 volts, 6.3 ± 1.8 mV at 10 volts). During pacing from the right ventricular outflow tract, at each pacing output voltage the PSA amplitude recorded by the true bipolar circuit (0.7 ± 0.1 mV at 1.5 volts, 1.7 ± 0.4 mV at 5 volts, 4.0 ± 0.7 mV at 10 volts) was significantly smaller than recorded by the Transvene integrated circuit (1.1 ± 0.4 mV at 1.5 volts, 3.9 ± 1.2 mV at 5 volts, 7.5 ± 1.8 mV at 10 volts) or the Endotak integrated circuit (1.6 ± 0.7 mV at 1.5 volts, 4.3 ± 1.7 mV at 5 volts, 7.5 ± 2.6 mV at 10 volts). For both pacing sites, the PSA amplitude recorded by the two integrated circuits was not significantly different.Conclusions: For a given pacing output voltage, PSA amplitude recorded by commercial endovascular rate sensing/defibrillation leads is greater when the sensing circuit is integrated than when it is true bipolar. These data may be helpful in planning ICD implantation in patients with previously implanted permanent pacemakers.  相似文献   
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