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Background: The long-QT syndromes (LQTS) are inherited electrical cardiomyopathies characterized by prolonged ventricular repolarization and ventricular arrhythmias. Several genetic reports have associated defects in LQTS-causing genes with atrial fibrillation (AF). We therefore studied whether atrial arrhythmias occur in patients with LQTS under daily-life conditions., Methods: We systematically assessed atrial arrhythmias in LQTS patients and matched controls using implanted defibrillators or pacemakers as monitors of atrial rhythm in a nested case-control study. Twenty-one LQTS patients (3 male; 39 +/- 18 years old; 18 on beta blocker, ICD therapy duration 6.3 +/- 2.7 years; 4 LQT1, 6 LQT2, 2 LQT3) were matched to 21 control subjects (13 male; 50 +/- 19 years old; 3 on beta blocker; pacemaker therapy duration 8.5 +/- 5.5 years; 19 higher-degree AV block, 2 others). LQTS patients were identified by a systematic search of the LQTS patient databases in Münster and Munich., Results: One-third (7 of 21) of the LQTS patients developed self-terminating atrial arrhythmias (atrial cycle lengths <250 ms). Only one control patient developed a single episode of postoperative AF (P < 0.05 vs LQTS)., Conclusions: LQTS patients at high risk for ventricular arrhythmias may develop short-lasting atrial arrhythmias under daily-life conditions, suggesting that prolonged atrial repolarization may contribute to the initiation of AF.

Objectives: Implantation of an additional HV-P/S lead versus extraction of the defective HV-P/S lead and implantation of a new one is one possible therapeutic approach in cases of a defective high-voltage pace/sense lead (HV-P/S). No information is available on potential differences in clinical outcome in these different approaches., Methods: Between January 2000 and February 2006, 86 patients with HV-P/S lead defect received either an additional transvenous HV-P/S lead (n = 33, group 1) or the HV-P/S lead was replaced (n = 53, group 2). The duration of the initially implanted leads was significantly different in the two groups (7.4 +/- 2.9; group 1 and 4.1 +/- 3.4 years; group 2). The outcome of these two groups of patients was retrospectively analyzed., Results: Seventy-three patients [85%] survived until the end of follow-up of 29 +/- 15 (group 1) and 33 +/- 21 (group 2) months (P = ns), respectively. Thirteen patients died: six in group 1 and seven in group 2 (P = ns). Fourteen patients experienced perioperative complications (group 1: six; group 2: eight; P = ns). ICD system-related complications occurred in 22 patients (group 1: seven; group two: 15; P = ns). The event-free cumulative survival of patients with additional and replaced HV-P/S lead for postoperative events (including death) after 1, 2, and 3 years was 82%, 70%, 70%, and 86%, 81%, 66%, respectively (P = 0.93)., Conclusions: Implantation of an additional HV-P/S lead or replacement of the HV-P/S lead in case of HV-P/S lead failure is statistically not different concerning mortality and morbidity. There are no predictors for further lead defects. Implantation of an additional HV-P/S lead should not be recommended in young patients or patients with greater likelihood of living many years. Predictors for death were an age over 70 years and renal insufficiency.

Sudden death among implantable cardioverter defibrillator (ICD) recipients is well known. The underlying mechanism remains unclear in most cases. One possible mechanism is an accidentally induced arrhythmia by the ICD itself. This report describes for the first time a case of a life-threatening ventricular arrhythmia induced by a specific algorithm for ventricular arrhythmia reconfirmation before shock delivery in a fourth-generation ICD.

Introduction: This retrospective study investigated whether induced episodes could be used to predict the morphology of future spontaneous atrial episodes., Methods: Eighty-two patients (64 +/- 12 years; 77% male; CAD in 60%; left ventricular ejection fraction 45 +/- 16%) with a history of atrial tachycardia or atrial fibrillation (AT/AF) were implanted with a dual-chamber implantable cardioverter defibrillator (ICD) and followed for 6 months. A total of 224 episodes of induced and spontaneous AT/AF were classified into type I, II, and III according to the method of Israel et al. and then compared based on average cycle length (CL) and atrial amplitude. Episodes were also grouped as "pace-terminable" or "nonpace-terminable" based on the CL definition of Gillis et al., Results: The analysis of 121 induced episodes (from 80 patients) and 103 spontaneous episodes (from 43 patients) showed that within each arrhythmia type, there were no significant differences in CL or mean amplitude between induced and spontaneous episodes. Additional analysis of patients that had both induced and spontaneous episodes (n = 41) showed 78% had at least one spontaneous episode that matched the induced episode. Fifty-seven percent of spontaneous episodes were considered to be pace-terminable based on CL., Conclusions: Our data suggest that there is no significant difference between induced and spontaneous episodes of AT/AF of the same type. The majority of patients had at least one spontaneous episode of the same type as the induced episode, showing that induced atrial arrhythmias may be useful in predicting the morphology of future spontaneous episodes and in identifying patients potentially benefiting from atrial antitachycardia pacing.

Introduction: The need to access the right ventricle might preclude transvenous placement of a defibrillation lead at implantable cardioverter defibrillator (ICD) placement, especially in small children or children with complex congenital heart defects. We investigated a subcutaneous array lead in addition to an abdominally placed "active can" ICD device in two children to avoid a thoracotomy., Methods and Results: The first child (age 12 years, 138 cm, 41 kg) had transposition of the great arteries with a subsequent surgical intra-atrial correction by the Mustard technique. The second child (age 14 years, 161 cm, 54 kg) had a single atrium and a single ventricle, d-transposition of the aorta, and atresia of the main pulmonary artery with a surgical anastomosis between the aorta and the right pulmonary artery by the Cooley technique. The defibrillation threshold was 18 J and <20 J at initial implantation and at generator replacement in the first patient and 20 J in the second patient. During follow-up of 6 years and 1 month, respectively, no ICD-related complications occurred., Conclusion: In children in whom endocardial, right ventricular placement of a defibrillation lead is precluded, defibrillation is possible and safe between an abdominally placed "active can" ICD device and a subcutaneous array lead. This approach may avoid a thoracotomy in children with no possibility for transvenous ICD placement.

About one half of deaths in patients with heart failure are sudden, mostly due to ventricular tachycardia (VT) degenerating to ventricular fibrillation or immediate ventricular fibrillation. In severe heart failure, sudden cardiac death also may occur due to bradyarrhythmias. Other dysrhythmias complicating heart failure include atrial and ventricular extrasystoles, atrial fibrillation (AF), and sustained and nonsustained ventricular tachyarrhythmias. The exact mechanism of the increased vulnerability to arrhythmias is not known. Depending on the etiology of heart failure, different preconditions, including ischemia or structural alterations such as fibrosis or myocardial scarring, may be prominent. Reentrant mechanisms around scar tissue, afterdepolarizations, and triggered activity due to changes in calcium metabolism significantly contribute to arrhythmogenesis. Furthermore, alterations in potassium currents leading to action potential prolongation and an increase in dispersion of repolarization play a significant role. Treatment of arrhythmias is necessary either because patients are symptomatic or to reduce the risk for sudden cardiac death. The individual history, left ventricular function, electrophysiologic testing, and the signal-averaged ECG give useful information for identifying patients at risk for sudden cardiac death. The implantable cardioverter defibrillator (ICD) has evolved as a promising therapy for life-threatening arrhythmias. A potential role may exist for antiarrhythmic drugs, mainly amiodarone. There is growing evidence that patients with sustained VT or a history of resuscitation have the best outcome with ICD therapy regardless of the degree of heart failure. Many of these patients require additional antiarrhythmic therapy because of AF or nonsustained VTs that may activate the device. Catheter ablation or map-guided endocardial resection are additional options in selected patients but seldom represent the only therapeutic strategy.

Introduction: Inappropriate therapies are the most frequent adverse event in patients with implantable cardioverter defibrillators (ICDs). Most ICDs offer a stability criterion to discriminate ventricular tachycardia (VT) from atrial fibrillation and an onset criterion to discriminate VT from sinus tachycardia. The efficacy and safety of these criteria, if used immediately after implantation, is unknown., Methods and Results: In a case control study, 87 patients in whom stability and onset criteria had been activated immediately after ICD implantation were matched to 87 patients in whom these criteria had not been activated. The groups were matched for known predictors of inappropriate therapies. With stability and onset criteria off, 24 patients (28%) received inappropriate therapies due to atrial fibrillation (n = 14) or sinus tachycardia (n = 11); with stability and onset on, only 11 patients (13%) were treated by the ICD due to atrial fibrillation (n = 5) or sinus tachycardia (n = 7) (log rank: P = 0.029). Five patients suffered inappropriate therapies despite the fact that onset (n = 4) or stability (n = 1) criteria were not fulfilled once tachycardias continued for a prespecified duration. Only one patient experienced a failure to detect VT due to the onset criterion; none because of stability., Conclusion: The immediate use of stability and onset criteria after ICD implantation reduces inappropriate therapies due to atrial fibrillation and sinus tachycardia. Because of the potential for underdetection of VT, this approach should be limited to tachycardia rates hemodynamically tolerated by the patient.

Introduction: The size of current implantable cardioverter defibrillators (ICD) is still large in comparison to pacemakers and thus not convenient for pectoral implantation. One way to reduce ICD size is to defibrillate with smaller capacitors. A trade-off exists, however, since smaller capacitors may generate a lower maximum energy output., Methods and Results: In a prospective randomized cross-over study, the step-down defibrillation threshold (DFT) of an experimental 90-microF biphasic waveform was compared to a standard 125-microF biphasic waveform. The 90-microF capacitor delivered the same energy faster and with a higher peak voltage but provided only a maximum energy output of 20 instead of 34 J. DFTs were determined intraoperatively in 30 patients randomized to receive either an endocardial (n = 15) or an endocardial-subcutaneous array (n = 15) defibrillation lead system. Independent of the lead system used, energy requirements did not differ at DFT for the experimental and the standard waveforms (10.3 +/- 4.1 and 9.5 +/- 4.9 J, respectively), but peak voltages were higher for the experimental waveform than for the standard waveform (411 +/- 80 and 325 +/- 81 V, respectively). For the experimental waveform the DFT w as 10 J or less using an endocardial lead-alone system in 10 (67%) of 15 patients and in 12 (80%) of 15 patients using an endocardial-subcutaneous array lead system., Conclusions: A shorter duration waveform delivered by smaller capacitors does not increase defibrillation energy requirements and might reduce device size. However, the smaller capacitance reduces the maximum energy output. If a 10-J safety margin between DFT and maximum energy output of the ICD is required, only a subgroup of patients will benefit from 90-microF ICDs with DFTs feasible using current defibrillation lead systems.

Introduction: The defibrillation threshold (DFT) of a transvenous-subcutaneous electrode configuration is sometimes unacceptably high. To obtain a DFT with a sufficient safety margin, the defibrillation field can be modified by repositioning the electrodes or more easily by a change of electrode polarity. In a prospective randomized cross-over study, the effect of transvenous electrode polarity on DFT was evaluated., Methods and Results: In 21 patients receiving transvenous-subcutaneous defibrillation leads, the DFT was determined intraoperatively for two electrode configurations. Two monophasic defibrillation pulses were delivered in sequential mode between either the right ventricular (RV) electrode as common cathode and the superior vena cava (SVC) and subcutaneous electrodes as anodes (configuration I) or the SVC electrode as common cathode and the RV and subcutaneous electrodes as anodes (configuration II). In each patient, both electrode configurations were used alternately with declining energies (25, 15, 10, 5, 2 J) until failure of defibrillation occurred. The DFT did not differ between both configurations (18.3 +/- 8.2 J vs 18.9 +/- 8.9 J; P = 0.72). Eleven patients had the same DFT with both electrode configurations, 5 patients a lower DFT with the RV electrode as cathode, and 5 patients a lower DFT with the SVC as cathode. Four patients had a sufficiently low DFT (< or = 25 J) with only 1 of the 2 configurations., Conclusion: A change of electrode polarity of transvenous-subcutaneous defibrillation electrodes may result in effective defibrillation if the first electrode polarity tested fails to defibrillate. In general, neither the RV electrode nor the SVC electrode is superior if used as a common cathode in combination with a subcutaneous anodal chest patch.

Introduction: For current implantable defibrillators, the nonthoracotomy approach to implantation fails in a substantial number of patients. In a prospective randomized cross-over study the defibrillation efficacy of a standard monophasic and a new biphasic waveform was compared for different lead configurations., Methods and Results: Intraoperatively, in 79 patients receiving nonthoracotomy defibrillation leads, the defibrillation threshold was determined in the initial lead configuration for the mono- and biphasic waveform. In each patient, both waveforms were used alternately with declining energies (20, 15, 10, 5 J) until failure of defibrillation occurred. Three different initial lead configurations were tested in different, consecutive, nonrandomized patients using a bipolar endocardial defibrillation lead alone (A; n = 36) or in combination with a subcutaneous defibrillation patch (B; n = 24) or array (C; n = 19) lead. The lowest successful defibrillation energy with the biphasic waveform was less than, equal to, or higher than with the monophasic waveform in 64%, 28%, and 8% of patients, respectively, and on average significantly lower with the biphasic waveform for all three lead configurations (A: 11.3 +/- 4.4 J vs 14.5 +/- 4.5 J; B: 9.7 +/- 4.7 J vs 15.1 +/- 4.5 J; C: 7.9 +/- 4.5 J vs 12.4 +/- 4.9 J). Defibrillation efficacy at 20 J was significantly improved by the biphasic waveform (91% vs 76%)., Conclusion: In combination with nonthoracotomy defibrillation leads, the biphasic waveform of a new implantable cardioverter defibrillator showed superior defibrillation efficacy in comparison to the standard monophasic waveform. Defibrillation thresholds were improved for lead systems with and without a subcutaneous patch or array lead.