The Automated Arrhythmia Origin Localization (AAOL) algorithm was developed for real-time prediction of early ventricular activation origins on a patient-specific electroanatomic (EAM) surface using a 3-lead electrocardiogram (AAOL-Surface). It has not been evaluated in 3-dimensional (3D) space (AAOL-3D), however, which may be important for predicting the arrhythmia origin from intramural or intracavity sites.

This study sought to assess the accuracy of AAOL for localizing earliest ventricular activation in 3D space.

This was a retrospective study of 3 datasets (BWH [Brigham and Women's Hospital], JHH [Johns Hopkins Hospital], and QEII [Queen Elizabeth II Health Sciences Centre]) involving 47 patients and 48 procedures, with an average of 19 ± 10 pacing sites each. In each patient, individual pacing sites were identified as target sites; the remaining pacing sites served as a training set (including QRS integrals from leads III, V2, and V6 with associated 3D coordinates). The AAOL-3D was then used to predict 3D coordinates of the pacing site. Localization error was assessed as the distance between known and predicted site coordinates, considering different EAM resolutions.

The AAOL-3D achieved a localization accuracy of 7.2 ± 3.1 mm, outperforming the AAOL-Surface (7.2 vs 7.8 mm; P < 0.05), with greater localization error for epicardial than endocardial pacing sites (8.7 vs 7.1 mm; P < 0.05). Cohort-specific analysis consistently favored AAOL-3D over AAOL-Surface in terms of accuracy. Exploration of AAOL-Surface accuracy across varying EAM resolutions showed optimal performance at the original and 75% resolution, with performance declining as resolution decreased.

The AAOL approach accurately identifies early ventricular activation origins in 3D and on EAM surfaces, potentially useful for identifying intramural arrhythmia origins.

The restriction of activation mapping to the ventricular surface of contemporary mapping systems often leads to failure to correctly identify the true sites of origin (SoOs) of intramural and/or subepicardial ventricular arrhythmias (VAs), reducing procedural success.

The aim of this study was to evaluate if noninvasive electromechanical wave imaging (EWI) can locate the SoOs of VAs along the endo-epicardial axis in patients with and without structural heart disease.

Patients with VAs requiring ablation underwent preprocedural transthoracic EWI to identify the SoOs and validate using contact mapping. Local electromechanical activation was defined as time point of the downward zero crossing on the incremental axial strain curve. The site of earliest activation on contact mapping and/or successful ablation was employed as ground truth for VA SoO.

Twenty-eight patients underwent EWI, and 25 proceeded to contact mapping (56% men, mean age 53 ± 16 years, mean left ventricular ejection fraction 47% ± 28%, 52% with late gadolinium enhancement (LGE) on magnetic resonance imaging). Five patients were excluded (insufficient or different ventricular tachycardia or ventricular ectopic activity at the time of EWI vs contact mapping). EWI mapping correctly localized the VA SoOs in 17 of 20 (85%) of the cases and mapped them to the adjacent segments in the remaining ones. In the presence of scar, EWI localized 81.8% of the VA SoOs (n = 9 of 11) correctly compared with 88.9% (n = 8 of 9) in patients without (P = NS). The transmural SoOs (endocardial, midmyocardial, or epicardial) were successfully identified in 18 of 20 cases (90%; P = NS for LGE-positive vs LGE-negative patients).

EWI correctly identified the transmural SoOs of focal VAs, including in the presence of scar, in the majority of cases using contact mapping as the gold standard and thus could support preprocedural planning, including requirement for epicardial access and/or ablation techniques. The absence of a true gold standard for clinical transmural mapping remains an important challenge for the validation of novel mapping technologies.

Stereotactic arrhythmia radioablation (STAR) is a novel, non-invasive, and promising treatment option for ventricular arrhythmias (VAs). It has been applied in highly selected patients mainly as bailout procedure, when (multiple) catheter ablations, together with anti-arrhythmic drugs, were unable to control the VAs. Despite the increasing clinical use, there is still limited knowledge of the acute and long-term response of normal and diseased myocardium to STAR. Acute toxicity appeared to be reasonably low, but potential late adverse effects may be underreported. Among published studies, the provided methodological information is often limited, and patient selection, target volume definition, methods for determination and transfer of target volume, and techniques for treatment planning and execution differ across studies, hampering the pooling of data and comparison across studies. In addition, STAR requires close and new collaboration between clinical electrophysiologists and radiation oncologists, which is facilitated by shared knowledge in each collaborator's area of expertise and a common language. This clinical consensus statement provides uniform definition of cardiac target volumes. It aims to provide advice in patient selection for STAR including aetiology-specific aspects and advice in optimal cardiac target volume identification based on available evidence. Safety concerns and the advice for acute and long-term monitoring including the importance of standardized reporting and follow-up are covered by this document. Areas of uncertainty are listed, which require high-quality, reliable pre-clinical and clinical evidence before the expansion of STAR beyond clinical scenarios in which proven therapies are ineffective or unavailable.

Substrate mapping is an important component of electrophysiological (EP) study for the treatment of reentrant ventricular tachycardia (VT). It is used to detect characteristics of the electrical circuit and, in particular, the location and properties of the central common pathway, aka the isthmus, where multiple circuit loops can coincide. Typically, reentrant circuits are single or double loop, but as the common pathway size increases, 4-loop patterns may emerge, consisting of 2 parallel isthmuses or a single isthmus with 4 loops. Arrhythmogenic substrate contains a mixture of scar, calcification, and fibrofatty regions blended with viable ventricular myocytes, which can slow conduction. It is identified in the EP laboratory in part by the presence of low-amplitude electrograms and a zone of uniform slow conduction resulting from a sparsity of remaining viable myocytes and molecular-level remodeling. The electrograms recorded near isthmus boundaries frequently exhibit an abnormal morphology, such as fractionation and late or split deflections, due to the separation of muscle fiber bundles by fibroadipose tissue or calcification, and due to other conduction impediments such as source-sink mismatch, wherein topographic changes to the viable myocardial structure occur. Substrate mapping facilitates the identification of arrhythmogenic regions during sinus rhythm, whereas inducible VT with periods of ongoing reentry, when recordable, can be used for further assessment. Substrate modeling augments substrate mapping by seeking to predict electrogram morphology and mapped features and properties to be encountered during EP study based on an accurate depiction of arrhythmogenic tissue. Herein, we elaborate on the details of VT substrate mapping and modeling to the present time.

Differentiating near-field (NF) and far-field (FF) electrograms (EGMs) is crucial in identifying critical arrhythmogenic substrate during ventricular tachycardia (VT) ablation. A novel algorithm annotates NF-fractionated signals enabling EGM peak frequency (PF) determination using wavelet transformation. This study evaluated the algorithms' effectiveness in identifying critical components of the VT circuit during substrate mapping.

A multicentre, international cohort undergoing VT ablation was investigated. VT activation maps were used to demarcate the isthmus zone (IZ). Offline analysis was performed to evaluate the diagnostic performance of low-voltage area (LVA) PF substrate mapping. A total of 30 patients encompassing 198 935 EGMs were included. The IZ PF was significantly higher in sinus rhythm (SR) compared to right ventricular paced (RVp) substrate maps (234 Hz (195-294) vs. 197 Hz (166-220); P = 0.010). Compared to LVA PF, the IZ PF was significantly higher in both SR and RVp substrate maps (area under curve, AUC: 0.74 and 0.70, respectively). The LVA PF threshold of ≥200 Hz was optimal in SR maps (sensitivity 69%; specificity 64%) and RVp maps (sensitivity 60%; specificity 64%) in identifying the VT isthmus. In amiodarone-treated patients (n = 20), the SR substrate map IZ PF was significantly lower (222 Hz (186-257) vs. 303 Hz (244-375), P = 0.009) compared to amiodarone-naïve patients (n = 10). The ≥200 Hz LVA PF threshold resulted in an 80% freedom from VT with a trend towards reduced ablation lesions and radiofrequency times.

LVA PF substrate mapping identifies critical components of the VT circuit with an optimal threshold of ≥200 Hz. Isthmus PF is influenced by chronic amiodarone therapy with lower values observed during RV pacing.

Voltage mapping could identify the conducting channels potentially responsible for ventricular tachycardia (VT). Standard thresholds (0.5-1.5 mV) were established using bipolar catheters. No thresholds have been analyzed with high-density mapping catheters. In addition, channels identified by cardiac magnetic resonance (CMR) has been proven to be related with VT.

The purpose of this study was to analyze the diagnostic yield of a personalized voltage map using CMR to guide the adjustment of voltage thresholds.

All consecutive patients with scar-related VT undergoing ablation after CMR (from October 2018 to December 2020) were included. First, personalized CMR-guided voltage thresholds were defined systematically according to the distribution of the scar and channels. Second, to validate these new thresholds, a comparison with standard thresholds (0.5-1.5 mV) was performed. Tissue characteristics of areas identified as deceleration zones (DZs) were recorded for each pair of thresholds. In addition, the relation of VT circuits with voltage channels was analyzed for both maps.

Thirty-two patients were included [mean age 66.6 ± 11.2 years; 25 (78.1%) ischemic cardiomyopathy]. Overall, 52 DZs were observed: 44.2% were identified as border zone tissue with standard cutoffs vs 75.0% using personalized voltage thresholds (P = .003). Of the 31 VT isthmuses detected, only 35.5% correlated with a voltage channel with standard thresholds vs 74.2% using adjusted thresholds (P = .005). Adjusted cutoff bipolar voltages that better matched CMR images were 0.51 ± 0.32 and 1.79 ± 0.71 mV with high interindividual variability (from 0.14-1.68 to 0.7-3.21 mV).

Personalized voltage CMR-guided personalized voltage maps enable a better identification of the substrate with a higher correlation with both DZs and VT isthmuses than do conventional voltage maps using fixed thresholds.

Idiopathic ventricular fibrillation (IVF) can be associated with undetected distinct conditions such as microstructural cardiomyopathic alterations (MiCM) or Purkinje (Purk) activities with structurally normal hearts.

This study sought to evaluate the characteristics of recurrent VF recorded on implantable defibrillator electrograms, associated with these substrates.

This was a multicenter collaboration study. At 32 centers, we selected patients with an initial diagnosis of IVF and recurrent arrhythmia at follow-up without antiarrhythmic drugs, in whom mapping demonstrated Purk or MiCM substrate. We analyzed variables related to previous ectopy, sinus rate preceding VF, trigger, and initial VF cycle lengths. Logistic regression with cross validation was used to evaluate the performance of criteria to discriminate Purk or MiCM substrates.

Among 95 patients (35 women, age 35 ± 11 years) meeting the inclusion criteria, IVF was associated with MiCM in 41 and Purk in 54 patients. A total of 117 arrhythmia recurrences including 91% VF were recorded on defibrillator. Three variables were mostly discriminant. Sinus tachycardia (≤570 ms) was more frequent in MiCM (35.9% vs 13.4%, P = 0.014) whereas short-coupled (<350 ms) triggers were most frequent in Purk-related VF (95.5% vs 23.1%, P = 0.001), which also had shorter VFCLs (182 ± 15 ms vs 215 ± 24 ms, P < 0.001).The multivariable combination provided the highest prediction (accuracy = 0.93 ± 0.05, range 0.833-1.000), discriminating 81% of IVF substrates with a high probability (>80%). Ectopy were inconsistently present before VF.

Characteristics of arrhythmia recurrences on implantable cardioverter- defibrillator provide phenotypic markers of the distinct and hidden substrates underlying IVF. These findings have significant clinical and genetic implications.

Electrophysiologic testing with programmed ventricular stimulation (PVS) has been utilized to induce ventricular tachycardia (VT), thereby improving risk stratification for patients with ischemic and nonischemic cardiomyopathies and determining the effectiveness of antiarrhythmic therapies, especially catheter ablation. A variety of procedural aspects can be modified during PVS in order to alter the sensitivity and specificity of the test including the addition of multiple baseline pacing cycle lengths, extrastimuli, and pacing locations. The definition of a positive result is also critically important, which has varied from exclusively sustained monomorphic VT (>30 seconds) to any ventricular arrhythmia regardless of morphology. In this review, we discuss the history of PVS and evaluate its role in sudden cardiac death risk stratification in a variety of patient populations. We propose an approach to future investigations that will capitalize on the unique ability to vary the sensitivity and specificity of this test. We then discuss the application of PVS during and following catheter ablation. The strategies that have been utilized to improve the efficacy of intraprocedural PVS are highlighted during a discussion of the limitations of this probabilistic strategy. The role of noninvasive programmed stimulation is also reviewed in predicting recurrent VT and informing management decisions including repeat ablations, modifications in antiarrhythmic drugs, and implantable cardioverter-defibrillator programming. Based on the available evidence and guidelines, we propose an approach to future investigations that will allow clinicians to optimize the use of PVS for risk stratification and assessment of therapeutic efficacy.

Although the epicardial predominance of substrate abnormalities has been well demonstrated in early stages of arrhythmogenic right ventricular cardiomyopathy (ARVC), endocardial (ENDO) ablation may suffice to eliminate ventricular tachycardia (VT) in some patients.

This study aimed to report the long-term outcomes of ENDO-only ablation in ARVC patients and factors that predict VT-free survival.

We included consecutive patients with Task Force Criteria diagnosis of ARVC undergoing a first ENDO-only VT ablation between 1998 and 2020. Ablation was predominantly guided by activation/entrainment mapping for mappable VTs and pace mapping/targeting abnormal electrograms for unmappable VTs. The primary endpoint was freedom from any recurrent sustained VT after the last ENDO-only ablation.

Seventy-four ARVC patients underwent ENDO-only VT ablation. VT noninducibility was achieved in 49 (66%) patients. During median follow-up of 6.6 years (Q1-Q3: 3.4-11.2 years), 40 (54.1%) patients remained free from any VT recurrence with rare VT ≤2 episodes in additional 12.2%. Among patients with noninducibility, VT-free survival was 75.5% during long-term follow-up. In multivariable analysis, >45 y of age at diagnosis (HR: 0.41; 95% CI: 0.17-0.98) and VT noninducibility (HR: 0.36; 95% CI: 0.16-0.80) were predictors of VT-free survival.

Long-term VT-free survival can be achieved in over half of ARVC patients following ENDO-only VT ablation, increasing to over 75% if VT noninducibility is achieved. Our results support consideration of a stepwise ENDO-only approach before proceeding to epicardial ablation if VT noninducibility can be achieved particularly in older patients.

This review aims to evaluate current evidence regarding ventricular tachycardia ablation in patients with ischemic heart disease and explore novel approaches currently developing to improve procedural and long-term outcomes.

Recently published trials (PARTITA, PAUSE-SCD, and SURVIVE-VT) have demonstrated the prognostic benefit of prophylactic ventricular tachycardia ablation compared to current clinical practice. Advanced cardiac imaging provides a valuable pre-procedural evaluation of the arrhythmogenic substrate, identifying ablation targets non-invasively. Advanced cardiac mapping techniques allow to better characterize arrhythmogenic substrate during ablation procedure. Emerging technologies like pulsed field ablation and ultra-low temperature cryoablation show promise in ventricular tachycardia ablation. Advancements in mapping techniques, ablation technologies, and pre-procedural cardiac imaging offer promise for improving ventricular tachycardia ablation outcomes in ischemic heart disease.

Signal-averaged electrocardiography (SAECG) provides diagnostic and prognostic information regarding cardiac diseases. However, its value in other nonischemic cardiomyopathies (NICMs) remains unclear. This study aimed to investigate the role of SAECG in patients with NICM.

This retrospective study included consecutive patients with NICM who underwent SAECG, biventricular substrate mapping, and ablation for ventricular arrhythmia (VA). Patients with baseline ventricular conduction disturbances were excluded. Patients who fulfilled at least one SAECG criterion were categorized into Group 1, and the other patients were categorized into Group 2. Baseline and ventricular substrate characteristics were compared between the two groups. The study included 58 patients (39 men, mean age 50.4 ± 15.5 years), with 34 and 24 patients in Groups 1 and 2, respectively. Epicardial mapping was performed in eight (23.5%) and six patients (25.0%) in Groups 1 and 2 (p = 0.897), respectively. Patients in Group 1 had a more extensive right ventricular (RV) low-voltage zone (LVZ) and scar area than those in Group 2. Group 1 had a larger epicardial LVZ than Group 2. Epicardial late potentials were more frequent in Group 1 than in Group 2. There were more arrhythmogenic foci within the RV outflow tract in Group 1 than in Group 2. There was no significant difference in long-term VA recurrence.

In our NICM population, a positive SAECG was associated with a larger RV endocardial scar, epicardial scar/late potentials, and a higher incidence of arrhythmogenic foci in the RV outflow tract.

Cardiac fibrosis stands as one of the most critical conditions leading to lethal cardiac arrhythmias. Identifying the precise location of cardiac fibrosis is crucial for planning clinical interventions in patients with various forms of ventricular and atrial arrhythmias. As fibrosis impedes and alters the path of electrical waves, detecting fibrosis in the heart can be achieved through analyzing electrical signals recorded from its surface. In current clinical practices, it has become feasible to record electrical activity from both the endocardial and epicardial surfaces of the heart. This paper presents a computational method for reconstructing 3D fibrosis using unipolar electrograms obtained from both surfaces of the ventricles. The proposed method calculates the percentage of fibrosis in various ventricular segments by analyzing the local activation times and peak-to-peak amplitudes of the electrograms. Initially, the method was tested using simulated data representing idealized fibrosis in a heart segment; subsequently, it was validated in the left ventricle with fibrosis obtained from a patient with nonischemic cardiomyopathy. The method successfully determined the location and extent of fibrosis in 204 segments of the left ventricle model with an average error of 0.0±4.3% (N = 204). Moreover, the method effectively detected fibrotic scars in the mid-myocardial region, a region known to present challenges in accurate detection using electrogram amplitude as the primary criterion.

Introduction: Patients with non-ischemic cardiomyopathy (NICM) are at risk for ventricular arrhythmias, but diagnosis and treatment planning remain a serious clinical challenge. Although computational modeling has provided valuable insight into arrhythmic mechanisms, the optimal method for simulating reentry in NICM patients with structural disease is unknown. Methods: Here, we compare the effects of fibrotic representation on both reentry initiation and reentry morphology in patient-specific cardiac models. We investigate models with heterogeneous networks of non-conducting structures (cleft models) and models where fibrosis is represented as a dense core with a surrounding border zone (non-cleft models). Using segmented cardiac magnetic resonance with late gadolinium enhancement (LGE) of five NICM patients, we created 185 3D ventricular electrophysiological models with different fibrotic representations (clefts, reduced conductivity and ionic remodeling). Results: Reentry was induced by electrical pacing in 647 out of 3,145 simulations. Both cleft and non-cleft models can give rise to double-loop reentries meandering through fibrotic regions (Type 1-reentry). When accounting for fibrotic volume, the initiation sites of these reentries are associated with high local fibrotic density (mean LGE in cleft models: p< 0.001, core volume in non-cleft models: p = 0.018, negative binomial regression). In non-cleft models, Type 1-reentries required slow conduction in core tissue (non-cleftsc models) as opposed to total conduction block. Incorporating ionic remodeling in fibrotic regions can give rise to single- or double-loop rotors close to healthy-fibrotic interfaces (Type 2-reentry). Increasing the cleft density or core-to-border zone ratio in cleft and non-cleftc models, respectively, leads to increased inducibility and a change in reentry morphology from Type 2 to Type 1. Conclusions: By demonstrating how fibrotic representation affects reentry morphology and location, our findings can aid model selection for simulating arrhythmogenesis in NICM.

Ventricular tachycardia is a potentially life-threatening arrhythmia associated with an overall high morbi-mortality, particularly in patients with structural heart disease. Despite their pivotal role in preventing sudden cardiac death, implantable cardioverter-defibrillators, although a guideline-based class I recommendation, are unable to prevent arrhythmic episodes and significantly alter the quality of life by delivering recurrent therapies. From open-heart surgical ablation to the currently widely used percutaneous approach, catheter ablation is a safe and effective procedure able to target the responsible re-entry myocardial circuit from both the endocardium and the epicardium. There are four main mapping strategies, activation, entrainment, pace, and substrate mapping, each of them with their own advantages and limitations. The contemporary guideline-based recommendations for VT ablation primarily apply to patients experiencing antiarrhythmic drug ineffectiveness or those intolerant to the pharmacological treatment. Although highly effective in most cases of scar-related VTs, the traditional approach may sometimes be insufficient, especially in patients with nonischemic cardiomyopathies, where circuits may be unmappable using the classic techniques. Alternative methods have been proposed, such as stereotactic arrhythmia radioablation or radiotherapy ablation, surgical ablation, needle ablation, transarterial coronary ethanol ablation, and retrograde coronary venous ethanol ablation, with promising results. Further studies are needed in order to prove the overall efficacy of these methods in comparison to standard radiofrequency delivery. Nevertheless, as the field of cardiac electrophysiology continues to evolve, it is important to acknowledge the role of artificial intelligence in both the pre-procedural planning and the intervention itself.

The use of catheter-based irreversible electroporation in clinical cardiac laboratories, termed pulsed-field ablation (PFA), is gaining international momentum among cardiac electrophysiology proceduralists for the non-thermal management of both atrial and ventricular tachyrhythmogenic substrates. One area of potential application for PFA is in the mitigation of ventricular tachycardia (VT) risk in the setting of ischemia-mediated myocardial fibrosis, as evidenced by recently published clinical case reports. The efficacy of tissue electroporation has been documented in other branches of science and medicine; however, ventricular PFA's potential advantages and pitfalls are less understood. This comprehensive review will briefly summarize the pathophysiological mechanisms underlying VT and then summarize the pre-clinical and adult clinical data published to date on PFA's effectiveness in treating monomorphic VT. These data will be contrasted with the effectiveness ascribed to thermal cardiac ablation modalities to treat VT, namely radiofrequency energy and liquid nitrogen-based cryoablation.

Bilateral cardiac sympathetic denervation (BCSD) for refractory life-threatening ventricular arrhythmias is a neuromodulatory intervention targeting sympathetically driven focal or re-entrant ventricular arrhythmias.

This study sought to provide a more complete and successful option for intervention in patients in whom premature ventricular contraction (PVC) ablation is not feasible or has been unsuccessful.

A total of 43 patients with >5% PVC burden and concomitant nonischemic cardiomyopathy (NICM) who previously failed medical and ablation therapies were referred for BCSD. All patients underwent bilateral video-assisted thoracoscopic surgical approach with T1-T4 sympathectomy. Primary effectiveness endpoints were postprocedural PVC burden resolution, improvement in left ventricular ejection fraction (LVEF), and cessation of antiarrhythmic drugs (AADs). Safety endpoints included peri- and postprocedural complications. Outcomes were assessed over a 1-year follow-up period.

Among the 43 patients who underwent BCSD, the mean age was 52.3 ± 14.7 years, 69.8% of whom were male patients. Presenting mean LVEF was 38.7% ± 7.8%, and PVC burden was 23.7% ± 9.9%. There were significant reductions in PVC burden postprocedurally (1.3% ± 1.1% post-BCSD, compared with 23.7% ± 9.9% pre-BCSD, P < 0.001) and improvements in LVEF (46.3% ± 9.5% post-BCSD, compared with 38.7% ± 7.8% pre-BCSD, P < 0.001). The rate of ICD therapies decreased from 81.4% (n = 35) to 11.6% (n = 5) (P < 0.001), leading to a significant reduction in use of AADs (100.0% to 11.6%, P < 0.001) and improvement in mean NYHA functional class (2.5 ± 0.5 to 1.4 ± 0.2, P < 0.001). Major intraoperative complications were seen in 4.7% of patients (hemothorax and chylothorax). Of the patients, 81.4% (n = 35) experienced no mortality or major complications over a 1-year follow-up period, with the remaining still within their first year postprocedure.

BCSD is effective for the management of refractory PVCs and ventricular tachycardia who have failed previous ablation therapy.

Catheter ablation of ventricular tachycardia (VTs) has emerged as an effective treatment modality. Ablation procedures for idiopathic VTs depends on the anatomical origin of the arrhythmias, is highly effective in certain cases, and has been implemented as a first-line therapy in recent European guidelines. In contrast, catheter ablation of VTs in patients with structural heart disease has a significant risk of arrhythmia recurrence. Interventional treatment for patients with ischemic cardiomyopathy was studied in multiple randomized multicenter trials and it was shown that catheter ablation was more effective in arrhythmia suppression compared to conservative treatment modalities. Catheter ablation of nonischemic cardiomyopathy suffers from far higher rates of arrhythmia recurrences as documented in several long-term studies and often needs complex procedures with or without epicardial mapping and ablation. There is still no clear proof of a mortality benefit from catheter ablation of VTs in patients with or without structural heart disease. Nevertheless, recent guidelines recommend catheter ablation as an alternative to implantation of cardioverter-defibrillators (ICD) in selected cases.

Three-dimensional electroanatomical mapping (EAM) has the potential to identify the pathological substrate underlying ventricular arrhythmias (VAs) in different clinical settings by detecting myocardial areas with abnormally low voltages, which reflect the presence of different cardiomyopathic substrates. In athletes, the added value of EAM may be to enhance the efficacy of third-level diagnostic tests and cardiac magnetic resonance (CMR) in detecting concealed arrhythmogenic cardiomyopathies. Additional benefits of EAM in the athlete include the potential impact on disease risk stratification and the consequent implications for eligibility to competitive sports. This opinion paper of the Italian Society of Sports Cardiology aims to guide general sports medicine physicians and cardiologists on the clinical decision when to eventually perform an EAM study in the athlete, highlighting strengths and weaknesses for each cardiovascular disease at risk of sudden cardiac death during sport. The importance of early (preclinical) diagnosis to prevent the negative effects of exercise on phenotypic expression, disease progression, and worsening of the arrhythmogenic substrate is also addressed.

Sudden cardiac death represents the leading cause of death worldwide; although the majority of sudden deaths occur in an elderly population with coronary artery disease, some occur in young and otherwise healthy individuals, as is the case of cardiomyopathies. The aim of the present review is to provide a stepwise hierarchical approach for the global sudden death risk estimation in primary cardiomyopathies. Each individual risk factor is analyzed for its contribution to the overall risk of sudden death for each specific cardiomyopathy as well as across all primary myocardial diseases. This stepwise hierarchical and personalized approach starts from the clinical evaluation, subsequently passes through the role of electrocardiographic monitoring and multimodality imaging, and finally concludes with genetic evaluation and electro-anatomical mapping. In fact, the sudden cardiac death risk assessment in cardiomyopathies depends on a multiparametric approach. Moreover, current indications for ventricular arrhythmia ablation and defibrillator implantation are discussed.

Reduced systolic myocardial function in the inferior region of the left ventricle has been suggested to be associated with malignant arrhythmias. We tested this hypothesis in patients with non-ischemic heart failure.

Patients with non-ischemic heart failure (left ventricular ejection fraction [LVEF] < 35%) were evaluated by 2D-speckle-tracking echocardiography. The regional longitudinal strain was calculated for each of the six left ventricular walls. The reduced regional function was defined as strain below the median. The outcome was a composite of sudden cardiac death, admission with sustained ventricular arrhythmia, resuscitated cardiac arrest, and appropriate therapy from a primary prophylactic implantable cardioverter defibrillator. Time-to-first-event analysis was performed using a Cox model.

From two centers, 401 patients were included (median age: 63 years, 72% male) with a median LVEF of 25% (interquartile range [IQR] 20;30), and a median inferior wall strain of -9.0% (-12.5; -5.4). During a median follow-up of 4.0 years, 52 outcomes occurred. After multivariate adjustment for clinical and electrocardiographic parameters, inferior wall strain was independently associated with the outcome (HR 2.50 [1.35; 4.62], p = .003). No independent association was found between the composite outcome and reduced strain in any of the other left ventricular walls, Global Longitudinal Strain (HR 1.66 [0.93; 2.98], p = .09), or LVEF (HR 1.33 [0.75; 2.33], p = .33).

Below median strain in the left ventricular inferior region was independently associated with a 2.5-fold increase in the risk of malignant arrhythmias and sudden cardiac death in patients with non-ischemic heart failure.

Ventricular fibrillation (VF) can be initiated by ventricular premature depolarizations (VPDs) in the absence of obvious structural abnormalities.

To determine the prevalence of 12-lead ECG sinus rhythm reduced QRS amplitude, QRS fractionation (QRSf) and early repolarization (ER) pattern, and the outcome of catheter ablation and VPD anatomic distribution in patients with VPDs initiating VF.

We compared a cohort with no apparent structural heart disease and VPDs initiating VF (Group 1, n=42) to a reference cohort (Group 2, n=61) of patients with no structural heart disease and symptomatic unifocal VPDs.

A reduced QRS amplitude (<.55 mV) in aVF (59 % vs 10%, p<0.001), QRSf in ≥2 contiguous leads (50% vs 16%, p<0.001) and early repolarization pattern (21.4% vs 1.6%, p=0.01) were more common in Group 1 vs Group 2. At least one abnormal ECG finding was present in 34 (81%) Group 1 vs 17 (28%) Group 2 patients, (p<0.001). VPD origin included RV and LV distal Purkinje system and moderator band/ papillary muscles, in 83% Group 1 vs 18% Group 2 patients, p<0.001. VF was eliminated with single ablation procedure in 77% of Group 1 patients with at least 2 years of follow-up.

A reduced QRS amplitude (<.55 mV) in aVF, QRS fractionation in ≥2 contiguous leads and/or an early repolarization pattern are frequently observed in patients with VPDs initiating VF. VPDs initiating VF typically originate from the distal Purkinje system and papillary muscles and can be successfully eliminated with catheter ablation.

The relations between endocardial voltage mapping and the genetic background of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been investigated so far. A total of 97 patients with proved or suspected ARVC who underwent 3-dimensional endocardial mapping and genetic testing have been retrospectively included. Presence, localization, and size of scar areas were correlated to ARVC diagnosis and the presence of a pathogenic variant. A total of 78 patients (80%) presented with some bipolar or unipolar scar on endocardial voltage mapping, whereas 43 carried pathogenic variants (44%). Significant associations were observed between presence of endocardial scars on voltage mapping and previous or inducible ventricular tachycardia, right ventricular function and dimensions, or electrocardiogram features of ARVC. A total of 60 of the 78 patients (77%) with an endocardial scar fulfilled the criteria for a definitive arrhythmogenic right ventricular dysplasia diagnosis versus 8 of 19 patients (42%) without scar (p = 0.003). Patients with a definitive diagnosis of ARVC had more scars from any location and the scars were larger in patients with ARVC. In the 68 patients with a definitive diagnosis of ARVC, the presence of any endocardial scar was similar whether an ARVC-causal mutation was present or not. Only scar extent was significantly greater in patients with pathogenic variants. There was no difference in the presence and characteristics of scars in PKP2 mutated versus other mutated patients. The 3-dimensional endocardial mapping could have an important role for refining ARVC diagnosis and may be able to detect minor forms with otherwise insufficient criteria for diagnosis. The trend for larger scar extent were observed in mutated patients, without any difference according to the mutated genes.

Ventricular tachycardia (VT) is caused by the presence of a slow conduction channel (CC) of border zone (BZ) tissue inside the scar-core tissue. Electroanatomic mapping can depict this tissue by voltage mapping. Areas of slow conduction can be detected as late potentials (LPs) and their abolition is the most accepted ablation endpoint. In the current guidelines, bipolar voltage thresholds for BZ and core scar are 1.5 and 0.5 mV respectively. The performance of these values is controversial. The aim of the study is to analyze the diagnostic yield of current amplitude thresholds in voltage map to define VT substrate in terms of CCs of LPs. Predictors of usefulness of current thresholds will be analyzed.

All patients with structural heart disease who underwent VT ablation in Hospital Clinic in 2016-2017 were included. Maps with delineation of CCs based on LPs were created with contact force sensor catheter. Thresholds were adjusted for every patient based on CCs. Diagnostic yield and predictors of performance of conventional thresholds were analyzed.

During study period, 57 consecutive patients were included (age: 60.4 ± 8.5; 50.2% ischemic cardiomyopathy, LVEF 39.8 ± 13.5%). Cutoff voltages that better identified the scar and BZ according to the LP channels were 0.32 (0.02-2 mV) and 1.84 (0.3-6 mV) respectively. Current voltage thresholds identified correctly core and BZ in 87.7% and 42.1% of the patients respectively. Accuracy was worse in non-ischemic cardiomyopathy (NICM) especially for BZ (28.6% vs 55.2%, p = 0.042).

Accuracy of standard voltage thresholds for scar and BZ is poor in terms of LPs detection. Diagnostic yield is worse in NICM patients specially for border zone.

Ventricular arrhythmias present with a wide spectrum of clinical manifestations, from mildly symptomatic frequent premature ventricular contractions to life-threatening events. Pathophysiologically, idiopathic ventricular arrhythmias occur in the absence of structural heart disease or ion channelopathies. Ventricular arrhythmias in the context of structural heart disease are usually determined by scar-related reentry and are associated with increased mortality. Catheter ablation is safe and highly effective in treating ventricular arrhythmias. The proper characterization of the arrhythmogenic substrate is essential for accurate procedural planning. We provide an overview on the main mechanisms of ventricular arrhythmias and their implications for catheter ablation.

Ablation of ventricular tachycardia (VT) is limited by the inability to create penetrating lesions to reach intramyocardial origins. Intramural needle ablation using in-catheter, heated saline-enhanced radio frequency (SERF) energy uses convective heating to increase heat transfer and produce deeper, controllable lesions at intramural targets. This first-in-human trial was designed to evaluate the safety and efficacy of SERF needle ablation in patients with refractory VT.

Thirty-two subjects from 6 centers underwent needle electrode ablation. Each had recurrent drug-refractory monomorphic VT after implantable cardioverter defibrillator implantation and prior standard ablation. During the SERF study procedure, one or more VTs were induced and mapped. The SERF needle catheter was used to create intramural lesions at targeted VT site(s). Acute procedural success was defined as noninducibility of the clinical VT after the procedure. Patients underwent follow-up at 30 days, and 3 and 6 months, with implantable cardioverter defibrillator interrogation at follow-up to determine VT recurrence.

These refractory VT patients (91% male, 66±10 years, ejection fraction 35±11%; 56% ischemic, and 44% nonischemic) had a median of 45 device therapies (shock/antitachycardia pacing) for VT in the 3 to 6 months pre-SERF ablation. The study catheter was used to deliver an average of 10±5 lesions per case, with an average of 430±295 seconds of radiofrequency time, 122±65 minute of catheter use time, and a procedural duration of 4.3±1.3 hours. Acute procedural success was 97% for eliminating the clinical VT. At average follow-up of 5 months (n=32), device therapies were reduced by 89%. Complications included 2 periprocedural deaths: an embolic mesenteric infarct and cardiogenic shock, 2 mild strokes, and a pericardial effusion treated with pericardiocentesis (n=1).

Intramural heated saline needle ablation showed complete acute and satisfactory mid-term control of difficult VTs failing 1 to 5 prior ablations and drug therapy. Further study is warranted to define safety and longer-term efficacy.

URL: https://www.

gov; Unique Identifier: NCT03628534 and NCT02994446.

Typical late gadolinium enhancement (LGE) patterns in dilated cardiomyopathy (DCM) include intramyocardial and subepicardial distribution. However, the ischemic pattern of LGE (subendocardial and transmural) has also been reported in DCM without coronary artery disease (CAD), but its correlates and prognostic significance are still not known. On these bases, this study sought to describe the prevalence and prognostic significance of the ischemic LGE pattern in DCM.

A total of 611 DCM patients with available cardiac magnetic resonance were retrospectively analyzed. A composite of all-cause-death, major ventricular arrhythmias (MVAs), heart transplantation (HTx) or ventricular assist device (VAD) implantation was the primary outcome of the study. Secondary outcomes were a composite of sudden cardiac death or MVAs and a composite of death for refractory heart failure, HTx or VAD implantation.

Ischemic LGE was found in 7% of DCM patients without significant CAD or history of myocardial infarction, most commonly inferior/inferolateral/anterolateral. Compared to patients with non-ischemic LGE, those with ischemic LGE had higher prevalence of hypertension and atrial fibrillation or flutter. Ischemic LGE was associated with worse long-term outcomes compared to non-ischemic LGE (36% vs 23% risk of primary outcome events at 5 years respectively, P = .006), and remained an independent predictor of primary outcome after adjustment for clinically and statistically significant variables (adjusted hazard ratio 2.059 [1.055-4.015], P = .034 with respect to non-ischemic LGE).

The ischemic pattern of LGE is not uncommon among DCM patients without CAD and is independently associated with worse long-term outcomes.

Mapping data of human ventricular fibrillation (VF) are limited. We performed detailed mapping of the activities underlying the onset of VF and targeted ablation in patients with structural cardiac abnormalities.

We evaluated 54 patients (50 ± 16 years) with VF in the setting of ischaemic (n = 15), hypertrophic (n = 8) or dilated cardiomyopathy (n = 12), or Brugada syndrome (n = 19). Ventricular fibrillation was mapped using body-surface mapping to identify driver (reentrant and focal) areas and invasive Purkinje mapping. Purkinje drivers were defined as Purkinje activities faster than the local ventricular rate. Structural substrate was delineated by electrogram criteria and by imaging. Catheter ablation was performed in 41 patients with recurrent VF. Sixty-one episodes of spontaneous (n = 10) or induced (n = 51) VF were mapped. Ventricular fibrillation was organized for the initial 5.0 ± 3.4 s, exhibiting large wavefronts with similar cycle lengths (CLs) across both ventricles (197 ± 23 vs. 196 ± 22 ms, P = 0.9). Most drivers (81%) originated from areas associated with the structural substrate. The Purkinje system was implicated as a trigger or driver in 43% of patients with cardiomyopathy. The transition to disorganized VF was associated with the acceleration of initial reentrant activities (CL shortening from 187 ± 17 to 175 ± 20 ms, P < 0.001), then spatial dissemination of drivers. Purkinje and substrate ablation resulted in the reduction of VF recurrences from a pre-procedural median of seven episodes [interquartile range (IQR) 4-16] to 0 episode (IQR 0-2) (P < 0.001) at 56 ± 30 months.

The onset of human VF is sustained by activities originating from Purkinje and structural substrate, before spreading throughout the ventricles to establish disorganized VF. Targeted ablation results in effective reduction of VF burden.

The initial phase of human ventricular fibrillation (VF) is critical as it involves the primary activities leading to sustained VF and arrhythmic sudden death. The origin of such activities is unknown.

Body-surface mapping shows that most drivers (≈80%) during the initial VF phase originate from electrophysiologically defined structural substrates. Repetitive Purkinje activities can be elicited by programmed stimulation and are implicated as drivers in 37% of cardiomyopathy patients.

The onset of human VF is mostly associated with activities from the Purkinje network and structural substrate, before spreading throughout the ventricles to establish sustained VF. Targeted ablation reduces or eliminates VF recurrence.