Background-Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of ventricular tachycardia (VT) and cardiac arrest in young patients. We hypothesized that treatment with implantable cardioverter/defibrillators (ICDs) is safe and improves the long-term prognosis of ARVC patients at high risk of sudden death. Methods and Results-Sixty patients with ARVC (aged 43Ϯ16 years) were treated with transvenous ICD systems. Despite a higher number of right ventricular sites tested for adequate lead positions (PϽ0.05), lower R-wave amplitudes (PϽ0.001) were achieved in ARVC patients compared with other entities. During follow-up of 80Ϯ43 months (396 patient-years), event-free survival was 49%, 30%, 26%, and 26% for appropriate ICD therapies and 79%, 64%, 59%, and 56% for potentially fatal VT (Ͼ240 bpm) after 1, 3, 5, and 7 years, respectively. Multivariate analysis identified extensive right ventricular dysfunction as an independent predictor of appropriate ICD discharge. Fifty-three adverse events occurred in 37 patients during the perioperative (nϭ10) or follow-up (nϭ43) period, mainly related to the leads (nϭ31 in 21 patients). No lead perforation was observed. Freedom from adverse events was 90%, 78%, 56%, and 42% and freedom from lead-related complications was 95%, 85%, 74%, and 63% after 1, 3, 5, and 7 years, respectively. Conclusions-These results strongly suggest an improvement in long-term prognosis by ICD therapy in high-risk patients with ARVC. However, meticulous placement and long-term observation of transvenous lead performance with focus on sensing function are required for the prevention and/or early recognition of disease progression and lead-related morbidity during long-term follow-up of ICD therapy in ARVC.

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BackgroundNo published data exist about the safety of diagnostic magnetic resonance (MR) of the heart performed in a larger series of patients implanted with MR conditional pacemakers (PM). The purpose of our study is to analyse safety and potential alterations of electrical lead parameters in patients implanted with the EnRhythm/Advisa MRI SureScan PM with 5086MRI leads (Medtronic Inc.) during and after MR of the heart at 1.5 Tesla.MethodsPatients enrolled in this single center pilot study who underwent non-clinically indicated diagnostic MR of the heart were included in this analysis. Heart MR was performed for analyses of potential changes in right and left ventricular functional parameters under right ventricular pacing at 80 and 110 bpm. Atrial/ventricular sensing, atrial/ventricular pacing capture threshold [PCT], and pacing impedances were assessed immediately before, during, and immediately after MR, as well at 3 and 15 months post MR.ResultsThirty-six patients (mean age 69 ± 13 years; high degree AV block 18 [50%]) underwent MR of the heart. No MR related adverse events occurred during MR or thereafter. Ventricular sensing differed significantly between the FU immediately after MR (10.3 ± 5.3 mV) and the baseline FU (9.8 ± 5.3 mV; p < 0.05). Despite PCT [V/0.4ms] was not significantly different between the FUs (baseline: 0.84 ± 0.27; in-between MR scans: 0.82 ± 0.27; immediately after MR: 0.84 ± 0.24; 3-month: 0.85 ± 0.23; 15-month: 0.90 ± 0.67; p = ns), 7 patients (19%) showed PCT increases by 100% (max. PCT measured: 1.0 V) at the 3-month FU compared to baseline. RV pacing impedance [Ω/5V] differed significantly at the FU in-between MR scans (516 ± 47), and at the 15-month FU (482 ± 58) compared to baseline (508 ± 75).ConclusionThe results of our study suggest MR of the heart to be safe in patients with the MR conditional EnRhythm/Advisa system, albeit although noticeable but clinically irrelevant ventricular PCT changes were observed.

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) typically present with ventricular tachyarrhythmias preferentially triggered by an elevated sympathetic tone. Previous studies demonstrated an impairment of the presynaptic catecholamine reuptake as assessed by 123 I-labeled norepinephrine analog on metaiodobenzylguanidine ( 123 I-MIBG) SPECT. Mutations in the gene encoding for plakophilin-2 (PKP-2) are the most common cause of autosomal dominant ARVC (ARVC-9). In this study, we investigated the potential role of adrenergic dysfunction on the arrhythmia profile in patients with ARVC and correlated these findings with the causative genotype. Methods: 123 I-MIBG SPECT was performed for 42 patients with definite ARVC (10 women, 32 men; mean age 6 SD, 43 6 14 y). Images were acquired at 4 h after injection and analyzed for regional 123 I-MIBG uptake in a standardized 33-segment polar map. Results were compared with those obtained from 10 control subjects (5 women, 5 men; mean age 6 SD, 43 6 12 y). Results: An abnormal tracer uptake was detected in 25 patients with ARVC (59%). The extents of right ventricular dilation and regional wall motion abnormalities as well as electrocardiographic markers of de-or repolarization were not significantly different between patients with normal and abnormal 123 I-MIBG SPECT findings. However, during long-term follow-up of 11.9 6 4.1 y, patients with abnormal 123 I-MIBG SPECT findings experienced life-threatening ventricular tachyarrhythmias significantly more often (22/25 patients [88%]) and independent of the extent of right ventricular dysfunction than those with a normal sympathetic innervation (6/17 patients [35%]; P , 0.0005). Mutations in PKP-2 were identified in 17 patients (40%) but were not correlated with the degree of adrenergic dysfunction. Conclusion: In patients with ARVC, an impairment of adrenergic innervation independent of the underlying genotype is associated with a higher incidence for future recurrences of ventricular tachyarrhythmias. This finding may suggest a potential role of 123 I-MIBG SPECT for individualized risk stratification in ARVC patients and asymptomatic PKP-2 mutation carriers alike.

BackgroundThe purpose of this study was to evaluate the feasibility of the magnetic resonance (MR) conditional pacemaker (PM) system (Evia SR-T and DR-T with Safio S leads) under MR conditions.MethodsPatients with standard PM indications and Evia PM were eligible for enrollment in this single center prospective non-randomized pilot study. Patients underwent MR of the brain and lower lumbar spine at 1.5 Tesla. Atrial (RA) und ventricular (RV) lead parameters (sensing, pacing threshold [PTH], pacing impedance) were assessed immediately before (baseline follow-up [FU]) and immediately after MRI (1st FU), after 1 month (2nd FU) and 3 months (3rd FU). The effect of MR on serious adverse device effect (SADE) free-rate, on atrial and ventricular sensing (AS/VS; mV) and atrial (RA) and ventricular (RV) pacing thresholds (PTH; V/0.4 ms) were investigated between baseline and 2nd FU. Continuous variables are expressed as mean ± SD and were compared using paired Student’s t-test. A p < 0.05 was considered significant.ResultsThirty-one patients were enrolled. One patient had to be excluded because of an enrollment violation. Therefore, data of 30 patients (female 12 [40%], age 73 ± 12 years, dual chamber PM 15 [50%]) were included in this analysis. No MR related SADE occurred. Lead measurements were not statistically different between the baseline FU and the 2nd FU (AS/VS at baseline 3.2 ± 2.1/15.0 ± 6.0, at 2nd FU 3.2 ± 2.1/14.9 ± 6.5; p = ns. RA-PTH/RV-PTH at baseline 0.68 ± 0.18/0.78 ± 0.22, at 2nd FU 0.71 ± 0.24/0.78 ± 0.22; p = ns). The presence of the permanent pacemakers led to MR imaging artifacts on diffusion weighted sequences of the brain, but did not affect other sequences (e.g. FLAIR and T2 weighted spin-echo images).ConclusionThe use of the MR conditional Evia PM in a MR environment under predefined conditions is feasible. No MR related SADEs nor clinically relevant changes in device functions occurred.

The HAWAI registry evaluated the role of heart rate variability in predicting the occurrence of ventricular tachycardia and fibrillation (VT/VF) and sinus tachycardia in patients with an implantable cardioverter-defibrillator (45 patients with 155 RR recordings). A significant decrease of the mean value of all RR intervals (MeanNN) was observed in the period starting 20 and 40 min prior to VT/VF and sinus tachycardia, respectively. The standard deviation of RR intervals (SDNN) and the power at low frequency (LF) were the only parameters with significant changes prior to VT/VF. For sinus tachycardia, the root mean square of successive differences of all successive RR intervals (r-MSSD) and the power at low and high frequency (HF) decreased, whereas SDNN and the power at very low frequency increased. Comparison of RR recordings preceding VT/VF and sinus tachycardia revealed significant differences of the MeanNN, SDNN, r-MSSD, LF and HF. Based on a classification and regression tree analysis, MeanNN, SDNN and r-MSSD showed a sensitivity of 94.4% and a specificity of 50.6% as predictors of VT/VF. Our results suggest that the temporal changes in heart rate before an arrhythmic event can be used to predict the occurrence of VT/VF. These parameters may be used to optimize pacing therapies designed to prevent VT/VF recurrences as well as for improving device-based discriminators for VT/VF and sinus tachycardia.

This is a report on a patient with an implanted cardioverter defibrillator (ICD) who intentionally underwent magnetic resonance imaging (MRI) of a malignant brain tumor. To avoid inadequate detection of ventricular tachycardia (VT) or ventricular fibrillation (VF), the ICD was inactivated by programming the VT-detection and VT/VF-therapy status off. The patient came through the protocol safely and without any difficulty or discomfort. There was no arrhythmic event. MRI affected neither programmed data nor the function of the ICD system.

Eighty-nine patients with a documented history of atrial tachyarrhythmias or fibrillation (AF) received a cardioverter defibrillator designed to selectively differentiate and treat atrial and ventricular arrhythmias. Twenty-two patients received a coronary sinus lead and, therefore, could use a separate shock vector for selective treatment of atrial tachyarrhythmias/AF. The device is designed to treat tachyarrhythmias with antitachycardia pacing (ATP) and/or shock therapy using an atrial and/or a ventricular shock vector. Patients underwent induction and shock termination of atrial or dual tachyarrhythmias (AF/VF) to verify proper device function and to measure the arrhythmia detection time with enhancements and preventive algorithms programmed On and Off, respectively. Detection time for 329 VF inductions was 2.41 +/- 0.64 seconds with enhancements On and 2.29 +/- 0.47 with enhancements Off (NS). At implant or predischarge, 283 AF and/or AF/VF (121 atrial and 162 atrial/ventricular fibrillation) were induced. Shock conversion efficacy was 89.8% with AF conversion energies ranging from 0.9 to 27 J. Thirteen of the 23 patients had atrial shock conversions using the separate shock vector with an average conversion energy of 1.9 +/- 1.4 J. (range 0.5-5 J). During follow-up the efficacy of ATP on atrial tachyarrhythmias was 59% and the efficacy of delivered shocks on AF was 85%. This new dual chamber cardioverter defibrillator appropriately detected and classified atrial arrhythmias, and shock therapy for AF was highly effective. The detection algorithm differentiated atrial tachyarrhythmia/AF and did not delay VF detection. The separate shock vector converted induced AF with energies ranging from 0.6 to 5 J.