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There is increasing evidence regarding the prevalence of genetic cardiomyopathies, for which arrhythmias may be the first presentation. Ventricular and atrial arrhythmias presenting in the absence of known myocardial disease are often labelled as idiopathic, or lone. While ventricular arrhythmias are well-recognized as presentation for arrhythmogenic cardiomyopathy in the right ventricle, the scope of arrhythmogenic cardiomyopathy has broadened to include those with dominant left ventricular involvement, usually with a phenotype of dilated cardiomyopathy. In addition, careful evaluation for genetic cardiomyopathy is also warranted for patients presenting with frequent premature ventricular contractions, conduction system disease, and early onset atrial fibrillation, in which most detected genes are in the cardiomyopathy panels. Sudden death can occur early in the course of these genetic cardiomyopathies, for which risk is not adequately tracked by left ventricular ejection fraction. Only a few of the cardiomyopathy genotypes implicated in early sudden death are recognized in current indications for implantable cardioverter defibrillators which otherwise rely upon a left ventricular ejection fraction ≤0.35 in dilated cardiomyopathy. The genetic diagnoses impact other aspects of clinical management such as exercise prescription and pharmacological therapy of arrhythmias, and new therapies are coming into clinical investigation for specific genetic cardiomyopathies. The expansion of available genetic information and implications raises new challenges for genetic counseling, particularly with the family member who has no evidence of a cardiomyopathy phenotype and may face a potentially negative impact of a genetic diagnosis. Discussions of risk for both probands and relatives need to be tailored to their numeric literacy during shared decision-making. For patients presenting with arrhythmias or cardiomyopathy, extension of genetic testing and its implications will enable cascade screening, intervention to change the trajectory for specific genotype-phenotype profiles, and enable further development and evaluation of emerging targeted therapies.

Develop a deflectable intracardiac MR imaging (ICMRI) guiding-sheath to accelerate imaging during MR-guided electrophysiological (EP) interventions for radiofrequency (500 kHz) ablation (RFA) of arrythmia. Requirements include imaging at three to five times surface-coil SNR in cardiac chambers, vascular insertion, steerable-active-navigation into cardiac chambers, operation with ablation catheters, and safe levels of MR-induced heating.ICMRI's 6 mm outer-diameter (OD) metallic-braided shaft had a 2.6 mm OD internal lumen for ablation-catheter insertion. Miniature-Baluns (MBaluns) on ICMRI's 1 m shaft reduced body-coil-induced heating. Distal section was a folded "star"-shaped imaging-coil mounted on an expandable frame, with an integrated miniature low-noise-amplifier overcoming cable losses. A handle-activated movable-shaft expanded imaging-coil to 35 mm OD for imaging within cardiac-chambers. Four MR-tracking micro-coils enabled navigation and motion-compensation, assuming a tetrahedron-shape when expanded. A second handle-lever enabled distal-tip deflection. ICMRI with a protruding deflectable EP catheter were used for MR-tracked navigation and RFA using a dedicated 3D-slicer user-interface. ICMRI was tested at 3T and 1.5T in swine to evaluate (a) heating, (b) cardiac-chamber access, (c) imaging field-of-view and SNR, and (d) intraprocedural RFA lesion monitoring.The 3T and 1.5T imaging SNR demonstrated400% SNR boost over a 4 × 4 × 4 cmICMRI assisted EP-catheter navigation to difficult targets and accelerated RFA monitoring.

Background: Initiation of ventricular tachycardia (VT) by programmed electrical stimulation (PES) has an important role to allow mapping and assess ablation end points. We hypothesized that substrate mapping may alter VT inducibility by mechanical bumping of critical sites. Methods: Subjects with left ventricular scar-related VT that was inducible by PES who were undergoing ablation were included. PES was repeated after substrate mapping (Group I) or after time under sedation/anesthesia during which additional imaging and transeptal puncture were performed without substrate mapping (Group II). The response to the second PES was categorized as type I if the same VT was induced, type II if a different VT was induced, and type III if VT was not inducible. Results: Twenty-eight patients (median age 66 years, 61% ischemic cardiomyopathy), 14 in Group I and 14 in Group II, were included. Age, time between initial and second PES, type of cardiomyopathy, ejection fraction, and anesthesia methods were not different between the 2 groups. Initial VT cycle length, however, was shorter in Group I (305 millisecond [range, 235–600] versus 350 millisecond [range, 235–600], P =0.016). Also, Group I required more extrastimuli to induce VT in PES 1 (2 [1–4] versus 2 [1–3], P =0.022). In Group I, following substrate mapping, the second PES induced the same VT in 3 patients (21%), a different VT in 9 (64%), and no VT in 2 (14%) patients. In contrast, in Group II the same VT was induced in 10 (71%) patients, a different VT in 3 (21%) and no VT in 1 (7%) patient ( P =0.017). Conclusions: Mechanical effects of substrate mapping commonly alter inducibility of VT. This has important implications for catheter ablation procedure planning and acute assessment of outcome and can potentially account for some recurrent VTs that are not recognized at the time of the procedure.

Background: Patients with dilated cardiomyopathy (DCM) who are undergoing catheter ablation of ventricular arrhythmias (VAs) are at risk of rapidly progressive heart failure (HF). Endocardial voltages decrease with loss of viable myocardium. Global left ventricular (LV) voltage as a surrogate for the amount of remaining viable myocardium may predict prognosis. Objectives: This study evaluated whether the newly proposed parameter volume-weighted (vw) unipolar voltage (UV) can predict HF-related adverse outcomes (HFOs), including death, heart transplantation, or ventricular assist device implantation, in DCM. Methods: In consecutive patients with DCM referred for VA ablation, vwUV was calculated by mathematically integrating UV over the left ventricle, divided by the endocardial LV surface area and wall thickness. Patients were followed for HFOs. Results: A total of 103 patients (57 ± 14 years of age; LV ejection fraction [LVEF], 39 ± 13%) were included. Median vwUV was 9.75 (IQR: 7.27-12.29). During a median follow-up of 24 months (IQR: 8-47 months), 25 patients (24%) died, and 16 had HFOs 7 months (IQR: 1-18 months) after ablation. Patients with HFOs had significantly lower LVEF (29% ± 10% vs 41% ± 12%), vw bipolar voltage (BV) (3.00 [IQR: 2.47-3.53] vs 5.00 [4.12-5.73]), and vwUV (5.94 [IQR: 5.28-6.55] vs 10.37 [IQR: 8.82-12.81]; all P < 0.001), than patients without HFOs. In Cox regression analysis and goodness-of-fit tests, vwUV was the strongest and independent predictor for HFOs (HR: 3.68; CI: 2.09-6.45; likelihood ratio chi-square, 33.05; P < 0.001). Conclusions: The novel parameter vwUV, as a surrogate for the amount of viable myocardium, identifies patients with DCM with VA who are at high risk for HF progression and mortality.

Aim To describe clinical characteristics, procedural details, specific challenges, and outcomes in patients with HeartMate3™ (HM3), a left ventricular assist device system with a magnetically levitated pump, undergoing ventricular tachycardia ablation (VTA). Methods and results Data were collected from patients with an HM3 system who underwent VTA in seven tertiary centres. Data included baseline patient characteristics, procedural data, mortality, and arrhythmia-free survival. The study cohort included 19 patients with cardiomyopathy presenting with ventricular tachycardia (VT) (53% with VT storm). Ventricular tachycardias were induced in 89% of patients and a total of 41 VTs were observed. Severe electromagnetic interference was present on the surface electrocardiogram. Hence, VT localization required analysis of intra-cardiac signals or the use of filter in the 40–20 Hz range. The large house pump HM3 design obscured the cannula inflow and therefore multi imaging modalities were necessary to avoid catheter entrapment in the cannula. A total of 32 VTs were mapped and were successfully ablated (31% to the anterior wall, 38% to the septum and only 9% to the inflow cannula region). Non-inducibility of any VT was reached in 11 patients (58%). Over a follow-up of 429 (interquartile range 101–692) days, 5 (26%) patients underwent a redo VT ablation due to recurrent VTA and 2 (11%) patients died. Conclusions Ventricular tachycardia ablation in patients with HM3 is feasible and safe when done in the appropriate setup. Long-term arrhythmia-free survival is acceptable but not well predicted by non-inducibility at the end of the procedure.

Objectives This study aimed to review the utility of quinidine in patients presenting with recurrent sustained ventricular arrhythmia (VA) and limited antiarrhythmic drug (AAD) options. Ba...

Objectives This study sought to investigate the factors associated with repeat infection following lead extraction procedures. Background Although lead extraction is an essential therapy for patients with cardiovascular implantable electronic device (CIED) infection, repeat infection still occurs in some patients. Methods The authors reviewed data for consecutive patients who underwent extraction of infected CIEDs from August 2003 to May 2019. Repeat infection was defined as infective endocarditis, sepsis with no alternative focus, or pocket infection after extraction of infected CIEDs. Results Extraction of infected CIEDs was performed in 496 patients. The most commonly implicated pathogen was Staphylococcus aureus (n = 188). In 449 patients (90.5%), all leads were completely extracted using only transvenous techniques. Thirty-three patients (6.7%) underwent surgical lead extraction, and 14 (2.8%) had retained leads or lead components. After a median follow-up of 352 [40 to 1,255] days after CIED extraction, 144 patients (29.0%) died. Repeat infection occurred in 47 patients (9.5%) with the median time from the extraction to repeat infection of 103 [45 to 214] days. In multivariable analysis, presence of a left ventricular assist device, younger age at extraction, and S. aureus infection were independent predictors of repeat infection. Additionally, chronic kidney disease, congestive heart failure, presence of septic emboli, S. aureus infection, and occurrence of major complications were independent predictors of increased mortality. Conclusions Patients with S. aureus infection have a high risk of repeat infection and poor prognosis after CIED extraction. Repeat infection is also predicted by younger age and the presence of a left ventricular assist device, whereas mortality was predicted by congestive heart failure, chronic kidney disease, and septic emboli.

Ventricular arrhythmias (VAs) can originate from different anatomical locations of the right ventricle. Ventricular arrhythmias originating from right ventricle have unique electrocardiographic (ECG) characteristics that can be utilized to localize the origin of the arrhythmia. This is crucial in pre-procedural planning particularly for ablation treatments. Moreover, non-ischaemic structural heart diseases, such as infiltrative and congenital heart diseases, are associated with the VAs that exhibit particular ECG findings. This article comprehensively reviews discriminatory ECG characteristics of VAs in the right ventricle with and without structural right ventricular diseases.

Catheter ablation can effectively reduce the frequency of ventricular tachycardia in ischemic cardiomyopathy by ablating sites of reentry within complex regions of myocardial scar. In cases of near transmural infarction, this arrhythmia substrate may be nearer the epicardium than the endocardium, and epicardial ablation may be necessary. An epicardial substrate location can potentially be predicted by imaging that suggests transmural infarction. Percutaneous epicardial ablation improves outcomes in selected patients, but is higher risk and avoided in patients with prior coronary artery bypass grafting.

Objectives This study sought to identify midmyocardial arrhythmogenic substrates by examining the frequency content of unipolar endocardial surface electrograms, comparing sites with transmural scar versus sites with intramural excitable substrate (IES) as identified during needle catheter ablation for ventricular tachycardia (VT). Background Midmyocardial arrhythmogenic substrates are a common reason catheter ablation for VT may fail. Methods A total of 659 intramural needle sites were studied in 26 patients (aged 61 ± 9 years, 85% male, 69% nonischemic cardiomyopathy) who underwent intramural needle catheter ablation for VT. Among 136 sites where endocardial pacing did not capture (threshold >10 mA), needle pacing captured at 29 indicating IES, and did not capture at 107 indicating transmural scar. Intramural needle ablation was performed at 21 of 29 IES sites. Analysis of voltage amplitude, duration, and power spectra of endocardial and intramural needle electrograms was performed. Results IES sites compared with transmural scar had higher endocardial unipolar electrogram voltage, 0.99 (Interquartile Range [IQR]: 0.69 to 1.62) mV versus 0.78 (IQR: 0.61 to 1.09) mV, p = 0.038; higher unipolar intramural needle electrogram voltage, 1.16 (0.8 to 1.69) mV versus 0.76 (0.6 to 1.12) mV, p = 0.003; higher endocardial unipolar frequency power particularly in the 5- to 20-Hz band, 1.97 (IQR: 0.93 to 3.89) mV2/s versus 1.03 (IQR: 0.63 to 2.22) mV2/s, p = 0.002; and higher unipolar intramural electrogram frequency particularly in the 0–10Hz range, 3.02 (IQR: 0.98 to 6.95) mV2/s versus 1.33 (IQR: 0.70 to 3.13) mV2/s, p = 0.018. Endocardial unipolar frequency in the 5- to 20-Hz band identified sites with IES, area under the curve of 0.676, p = 0.002; power frequency integral of >0.77 mV2/s provided a 90% sensitivity and 41% specificity. Conclusions The frequency content of unipolar electrograms may complement voltage in the detection of deep intramural substrates to facilitate VT catheter ablation. (Intramural Needle Ablation for Ablation of Recurrent Ventricular Tacchycardia [NCT01791543])

Background: Frequent premature ventricular contractions (PVCs) are often amenable to catheter ablation. However, a deep intramural focus may lead to failure due to inability of standard ablation techniques to penetrate the focus. We sought to assess the efficacy and safety of infusion needle ablation (INA) for PVCs that are refractory to standard radiofrequency ablation. Methods: Under 2 Food and Drug Administration approved protocols, INA was evaluated in patients with frequent PVCs that were refractory to standard ablation. Initial targets for ablation were selected by standard mapping techniques. INA was performed with a deflectable catheter equipped with an extendable/retractable needle at the tip that can be extended up to 12 mm into the myocardium and is capable of pacing and recording. After contrast injection for location assessment, radiofrequency ablation was performed with the needle tip using a temperature-controlled mode (maximum temperature 60 °C) with saline infusion from the needle. The primary end point was a decrease in PVC burden to Results: At 4 centers, 35 patients (age 55.3±16.9 years, 74.2% male) underwent INA. The baseline median PVC burden was 25.4% (interquartile range, 18.4%–33.9%) and mean left ventricular ejection fraction was 37.7±12.3%. Delivering 10.3±8.0 INA lesions/patient (91% had adjunctive standard radiofrequency ablation also) resulted in acute PVC elimination in 71.4%. After a mean follow-up of 156±109 days, the primary efficacy end point was met in 73.3%. The median PVC burden decreased to 0.8% (interquartile range, 0.1%–6.0%; P Conclusions: INA is effective for the elimination of frequent PVCs that are refractory to conventional ablation and is associated with an acceptable safety profile. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT01791543 and NCT03204981.

INTRODUCTION Ventricular tachycardia (VT) normally occurs from an abnormal structural substrate. CASE We report a case in which VT was caused by a large tumor in the interventricular septum. Surgical intervention was not an option due to the location of the tumor and its proximity to the coronary arteries. CONCLUSION The patient underwent ablation and upgrade to CRT before ultimately receiving a heart transplant.

Radiofrequency (RF) catheter ablation has long been an important therapy for ventricular tachycardia and frequent symptomatic premature ventricular beats and nonsustained arrhythmias when antiarrhythmic drugs fail to suppress the arrhythmias. It is increasingly used in preference to antiarrhythmic drugs, sparing the patient adverse effects of drugs. Success of ablation varies with the underlying heart disease and type of arrhythmia: very effective for patients without structural heart disease, less effective in structural heart disease. Failure occurs when a target for ablation cannot be identified or ablation lesions fail to reach and abolish the arrhythmia substrate that may be extensive, intramural, or subepicardial in location. Approaches to improving ablation lesion creation are modifications to RF ablation and emerging investigational techniques. Easily- implemented modifications to RF methods include manipulating the size and location of the cutaneous dispersive electrode, increasing duration of RF delivery, and use of lower-tonicity catheter irrigation (usually 0.45% saline). When catheters can be placed on either side of culprit substrate, RF can be delivered in a bipolar or simultaneous unipolar configuration that can be successful. Catheters with extendable and retractable irrigated needles for delivery of RF are under investigation in clinical trials. Cryoablation is potentially useful with specific situations in which maintaining contact is difficult. Transvascular ethanol ablation and stereotactic radioablation have both shown promise for arrhythmias that fail other ablation strategies. Although substantial clinical progress has been achieved, further improvement is clearly needed. With ability to increase ablation lesion size, continued careful evaluation of safety, which has been excellent for standard RF ablation, remains important.

Current guidelines recommend complete extraction of cardiovascular implantable electronic devices (CIEDs) in the case of persistent or recurrent fungemia without other identifiable sources, though supporting evidence is lacking. We sought to evaluate the prognosis of patients with candidemia and CIEDs. Twelve consecutive patients (54 ± 12 years, 8 male) with CIED and concurrent candidemia were reviewed. At the time of diagnosis with candidemia, seven patients were immunocompromised, six were on long-term antibacterial therapy, two were intravenous drug users, four were on chronic hemodialysis, and six had a central venous catheter. Four patients were confirmed as definite CIED infection as vegetation was visible on lead by echocardiogram. The other 8 patients were considered possible CIED infection with candidemia of unknown focus. All patients with visible vegetation underwent CIED removal without complications, and other patients were initially managed non-operatively. After 1 year of follow-up, 7 patients had died and at extended follow-up, all patients without lead removal died while 3 of 4 patients with lead extraction survived. Of note, 50% of deaths in the patients without lead removal were associated with fungal sepsis. Candida fungemia is associated with a high mortality. CIED removal should be an early consideration in these patients even if lead vegetations are not seen.

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and treat them. The field of catheter ablation has progressed with the development of new methods and tools, and with the publication of large clinical trials. Therefore, global cardiac electrophysiology professional societies undertook to outline recommendations and best practices for these procedures in a document that will update and replace the 2009 EHRA/HRS Expert Consensus on Catheter Ablation of Ventricular Arrhythmias. An expert writing group, after reviewing and discussing the literature, including a systematic review and meta-analysis published in conjunction with this document, and drawing on their own experience, drafted and voted on recommendations and summarized current knowledge and practice in the field. Each recommendation is presented in knowledge byte format and is accompanied by supportive text and references. Further sections provide a practical synopsis of the various techniques and of the specific ventricular arrhythmia sites and substrates encountered in the electrophysiology lab. The purpose of this document is to help electrophysiologists around the world to appropriately select patients for catheter ablation, to perform procedures in a safe and efficacious manner, and to provide follow-up and adjunctive care in order to obtain the best possible outcomes for patients with ventricular arrhythmias. Electronic supplementary material The online version of this article (10.1007/s10840-019-00664-2) contains supplementary material, which is available to authorized users.

A high rate of malfunction, predominantly intermittent high-frequency artifacts (HFAs), has been recently reported in Abbott Medical Tendril pacing leads.To investigate the factors associated with the occurrence of HFAs on Tendril leads using a commonly used comparator lead for a control.We reviewed institutional data for Medtronic CapSureFix 5076 and Abbott Medical Tendril pace-sense leads retrospectively. Recordings deemed to be due to electromagnetic interference and far-field oversensing were not included in the classification of HFAs.A total of 7673 leads were analyzed: 1628 Optim-insulated Tendril leads, 825 non-Optim Tendril leads, and 5220 CapSureFix 5076. HFAs were seen in 212 leads and were more frequently observed in Tendril compared to CapSureFix leads during a mean follow-up of 4.1 ± 3.6 years. Lower age at implant, defibrillator systems, atrial position, and connection to an Abbott Medical generator were associated with increased HFA. In multivariable analysis, only connection to Abbott Medical generators (odds ratio 7.686, P.001) and age (odds ratio 0.988 per year, P = .016) were independently associated with HFAs on pace-sense leads. In an Abbott-generator-only analysis, Optim-insulated Tendril leads were more likely to display HFAs than non-Optim Tendril leads but not Medtronic CapSureFix 5076 leads.Abbott Medical pulse generators independently predict HFA in Tendril and CapSureFix 5076 leads, likely the result of displaying short or low-amplitude noise episodes that other devices do not record. When restricted to Abbott generators only, Optim-insulated Tendril leads show an increased incidence of HFAs when compared to non-Optim Tendril leads but not CapSureFix 5076 leads.

Background Catheter ablation for polymorphic ventricular tachycardia and ventricular fibrillation (PMVT/VF) may target triggering premature ventricular contractions (PVCs). Targeting ventricular scar has also been suggested, but data are limited. Objective The purpose of this study was to characterize the electrophysiological findings and ablation outcomes for patients with PMVT/VF and structural heart disease (SHD) compared to those with idiopathic VF. Methods Data from 32 consecutive patients (13 idiopathic VF, 19 SHD) with recurrent PMVT/VF who underwent catheter ablation were reviewed. Results A low-voltage area of myocardial scar was present in 15 of 19 patients with SHD. Sustained monomorphic ventricular tachycardia (SMVT) associated with scar was inducible and targeted in 8, 3 of whom had previous SMVT episodes separate from PMVT/VF episodes and 5 had no history of SMVT. Triggering PVCs were identified in 11 patients and arose from an area of endocardial scar in 6. Only scar ablation was performed in 8 patients who did not have triggering PVCs. All idiopathic VF patients underwent PVC ablation only. During a median of 540 days, 74% of SHD patients and 77% of idiopathic VF patients were free of recurrence, including 75% of those with only PVC ablation, 86% of those with scar plus PVC ablation, and 63% of those with only scar ablation. Conclusion Patients with recurrent PMVT/VF and SHD often have a low-voltage scar associated with PVCs or inducible SMVT, which may also be the substrate for PMVT/VF. When present, substrate ablation targeting scar is a reasonable option for treatment of PMVT/VF even if PVCs are absent.