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Competing Interests: Funding Support and Author Disclosures This work was supported by the Leducq International Network on Bioelectronics for Neurocardiology.

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: 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., Objectives: This study sought to evaluate the characteristics of recurrent VF recorded on implantable defibrillator electrograms, associated with these substrates., Methods: 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., Results: 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., Conclusions: 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., Competing Interests: Funding Support and Author Disclosures Dr Haissaguerre has received grant support from Biosense Webster. Dr Marijon has received grant support and consulting fees from Medtronic, Boston Scientific, Abbott, Microport, Biotronik, and Zoll. Pr Gandjbakhch has received lecture fees from Biotronik; and consulting fees from Medtronic, Microport, and Abbott. Dr Tilz is a consultant for Boston Scientific, Biotronik, Biosense Webster, and Abbott Medical; has received speaker honoraria from Boston Scientific, Biotronik, Biosense Webster, Abbott Medical, and Lifetech; and has received research grants from Abbott, Biosense Webster, and Lifetech. Dr Roten has received research grants from Medtronic, the Swiss National Foundation, the Swiss Heart Foundation, the Immanuel and Ilse Straub Foundation, and the Sitem Insel Support Fund, all for work outside the submitted study; and has received speaker/consulting honoraria from Abbott and Medtronic. Dr Reichlin has receivedresearch grants from the Swiss National Science Foundation, the Swiss Heart Foundation, the Sitem Insel support funds, Biotronik, Boston-Scientific, and Medtronic, all for work outside the submitted study; and has received speaker/consulting honoraria or travel support from Abbott/SJM, Biosense-Webster, Biotronik, Boston-Scientific, and Medtronic, all for work outside the submitted study. Support for his institution’s fellowship program from Abbott/SJM, Biosense-Webster, Biotronik, Boston-Scientific, and Medtronic for work outside the submitted study. Dr Sacher has receivedspeaking honorarium from Abbott, Boston Scientific, and Biosense Webster; and equity from InHeart. Dr Nogami has received lecture fees from Abbott; and endowments from Medtronic. Dr Massoullié has received lecture fees from Boston Scientific, Biosense Webster, and Abbot. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Desmoplakin (DSP) pathogenic variants are rare causes of arrhythmogenic cardiomyopathy and often involve the right and left ventricles. Ventricular tachycardia (VT) ablations may be required in these patients, but procedural characteristics have not been reported., Objectives: In this study, the authors sought to report a multicenter experience of VT ablation in patients with DSP pathogenic variants., Methods: VT ablations performed in patients with known DSP pathogenic variants were analyzed across 6 centers in 3 countries. Patient characteristics and acute and long-term procedural outcomes were reported., Results: A total of 20 patients (13 men, median age 43 years [Q1-Q3: 41.5-53.0 years], left ventricular ejection fraction 43.0% [Q1-Q3: 41.5%-53.0%], 11 previous failed ablations) were referred for VT ablation procedures. All patients had symptomatic VTs, with ICD therapy in 19 patients. Epicardial procedures were performed in 16 of the 20 patients. VT target sites were located in the right ventricular (RV) endocardium (n = 11), the RV epicardium (n = 4), the left ventricular (LV) endocardium (n = 2) and the LV epicardium (n = 7). In 3 patients, the VT target sites were in close proximity to coronary arteries, limiting ablation. During follow-up, VTs recurred in 11 patients, and repeated ablations were performed in 9 patients. Allowing for multiple procedures, 19 of the 20 patients remained free of VT recurrence after a median follow-up of 18 months [Q1-Q3: 5-60 months]., Conclusions: Patients with DSP cardiomyopathy often have biventricular involvement, and ablation procedures often require ablation in both ventricles and the epicardium. Recurrences are not uncommon, and the pathologic substrate can be located in close proximity to epicardial coronary arteries, limiting the success rate of ablations., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024. Published by Elsevier Inc.)

Background: Electrophysiological mapping of ventricular tachycardia (VT) is tedious and poorly reproducible. Substrate analysis on imaging cannot explicitly display VT circuits., Objectives: This study sought to introduce a computed tomography-based model personalization approach, allowing for the simulation of postinfarction VT in a clinically compatible time frame., Methods: In 10 patients (age 65 ± 11 years, 9 male) referred for post-VT ablation, computed tomography-derived wall thickness maps were registered to 25 electroanatomical maps (sinus rhythm, paced, and VT). The relationship between wall thickness and electrophysiological characteristics (activation-recovery interval) was analyzed. Wall thickness was then employed to parameterize a fast and tractable organ-scale wave propagation model. Pacing protocols were simulated from multiple sites to test VT induction in silico. In silico VTs were compared to VT circuits mapped clinically., Results: Clinically, 6 different VTs could be induced with detailed maps in 9 patients. The proposed model allowed for fast simulation (median: 6 min/pacing site). Simulations of steady pacing (600 milliseconds) from 100 different sites/patient never triggered any arrhythmia. Applying S1-S2 or S1-S2-S3 induction schemes allowed for the induction of in silico VTs in the 9 of 10 patients who were clinically inducible. The patient who was not inducible clinically was also noninducible in silico. A total of 42 different VTs were simulated (4.2 ± 2 per patient). Six in silico VTs matched a VT circuit mapped clinically., Conclusions: The proposed framework allows for personalized simulations in a matter of hours. In 6 of 9 patients, simulations show re-entrant patterns matching intracardiac recordings., Competing Interests: Funding Support and Author Disclosures This study received financial support from the French government as part of the “Investments of the Future” program managed by the National Research Agency (ANR-10-IAHU-04). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Substrate abnormalities can alter atrial activation during atrial tachycardias (ATs) thereby influencing AT-wave morphology on the surface electrocardiogram., Objectives: This study sought to identify determinants of isoelectric intervals during ATs with complex atrial activation patterns., Methods: High-density activation maps of 126 ATs were studied. To assess the impact of the activated atrial surface on the presence of isoelectric intervals, this study measured the minimum activated area throughout the AT cycle, defined as the smallest activated area within a 50-millisecond period, by using signal processing algorithms (LUMIPOINT)., Results: ATs with isoelectric intervals (P-wave ATs) included 23 macro-re-entrant ATs (40%), 26 localized-re-entrant ATs (46%), and 8 focal ATs (14%), whereas those without included 46 macro-re-entrant ATs (67%), 21 localized-re-entrant ATs (30%), and 2 focal ATs (3%). Multivariable regression identified smaller minimum activated area and larger very low voltage area as independent predictors of P-wave ATs (OR: 0.732; 95% CI: 0.644-0.831; P < 0.001; and OR: 1.042; 95% CI: 1.006-1.080; P = 0.023, respectively). The minimum activated area with the cutoff value of 10 cm 2 provided the highest predictive accuracy for P-wave ATs with sensitivity, specificity, and positive and negative predictive values of 96%, 97%, 97%, and 95%, respectively. In re-entrant ATs, smaller minimum activated area was associated with lower minimum conduction velocity within the circuit and fewer areas of delayed conduction outside of the circuit (standardized β: 0.524; 95% CI: 0.373-0.675; P < 0.001; and standardized β: 0.353; 95% CI: 0.198-0.508; P < 0.001, respectively)., Conclusions: Reduced atrial activation area and voltage were associated with isoelectric intervals during ATs., Competing Interests: Funding Support and Author Disclosures This research was partly funded by a grant from Investissement d’avenir: IHU LIRYC ANR-10-IAHU-04. Ms Carapezzi is an employee of Boston Scientific. Dr Jaïs has received speaking honoraria and consulting fees from Boston Scientific. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Brugada syndrome is a significant cause of sudden cardiac death (SCD), but the underlying mechanisms remain hypothetical., Objectives: This study aimed to elucidate this knowledge gap through detailed ex vivo human heart studies., Methods: A heart was obtained from a 15-year-old adolescent boy with normal electrocardiogram who experienced SCD. Postmortem genotyping was performed, and clinical examinations were done on first-degree relatives. The right ventricle was optically mapped, followed by high-field magnetic resonance imaging and histology. Connexin-43 and Na V 1.5 were localized by immunofluorescence, and RNA and protein expression levels were studied. HEK-293 cell surface biotinylation assays were performed to examine Na V 1.5 trafficking., Results: A Brugada-related SCD diagnosis was established for the donor because of a SCN5A Brugada-related variant (p.D356N) inherited from his mother, together with a concomitant NKX2.5 variant of unknown significance. Optical mapping demonstrated a localized epicardial region of impaired conduction near the outflow tract, in the absence of repolarization alterations and microstructural defects, leading to conduction blocks and figure-of-8 patterns. Na V 1.5 and connexin-43 localizations were normal in this region, consistent with the finding that the p.D356N variant does not affect the trafficking, nor the expression of Na V 1.5. Trends of decreased Na V 1.5, connexin-43, and desmoglein-2 protein levels were noted; however, the RT-qPCR results suggested that the NKX2-5 variant was unlikely to be involved., Conclusions: This study demonstrates for the first time that SCD associated with a Brugada-SCN5A variant can be caused by localized functionally, not structurally, impaired conduction., Competing Interests: Funding Support and Author Disclosures This work received financial support from the French Government as part of the “Investments of the Future” program managed by the National Research Agency (ANR-10-IAHU04-LIRYC), the Leducq-Foundation (RHYTHM network, 16CVD02), and the Fondation Coeur et Artères (FC17T2). Dr Barc is supported by the ANR JCJC LEARN (R21006NN, RPV21014NNA). Dr Schott is supported by IRP-, an I-SITE NExT health and engineering initiative (Ecole Centrale & Nantes University) and by the IRP- GAINES funded by INSERM and CNRS. Dr Marionneau is supported by the ANR-16-CE92-0013-01 and the National Institutes of Health (R01-HL148803). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Ultra-low temperature cryoablation (ULTC) using near-critical nitrogen (-196ºC) has been shown to produce durable, contiguous, transmural lesions in ventricles of animal models. This report summarizes acute experience with ULTC in the first-ever 13 patients with recurrent monomorphic ventricular tachycardias (VTs) of both ischemic cardiomyopathy and nonischemic etiologies enrolled in the CryoCure-VT (Cryoablation for Monomorphic Ventricular Tachycardia; NCT04893317) clinical trial. After an average of 9.6 ± 4.6 endocardial ULTC lesions per patient, no clinical ventricular tachycardias were inducible in 91% of patients. Two procedure-related serious adverse events recorded in 2 patients resolved post-procedurally without clinical sequelae. Further investigation of both acute and chronic outcomes is warranted and ongoing., Competing Interests: Funding Support and Author Disclosures All study expenses have been paid by Adagio Medical, Inc. Dr De Potter has received consulting fees and equity options from Adagio Medical, Inc. Drs Boersma and Verma have received consulting fees from Adagio Medical, Inc. Dr Grigorov is an employee of Adagio Medical, Inc. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

In the setting of structural heart disease, ventricular tachycardia (VT) is typically associated with a re-entrant mechanism. In patients with hemodynamically tolerated VTs, activation and entrainment mapping remain the gold standard for the identification of the critical parts of the circuit. However, this is rarely accomplished, as most VTs are not hemodynamically tolerated to permit mapping during tachycardia. Other limitations include noninducibility of arrhythmia or nonsustained VT. This has led to the development of substrate mapping techniques during sinus rhythm, eliminating the need for prolonged periods of mapping during tachycardia. Recurrence rates following VT ablation are high; therefore, new mapping techniques for substrate characterization are required. Advances in catheter technology and especially multielectrode mapping of abnormal electrograms has increased the ability to identify the mechanism of scar-related VT. Several substrate-guided approaches have been developed to overcome this, including scar homogenization and late potential mapping. Dynamic substrate changes are mainly identified within regions of myocardial scar and can be identified as local abnormal ventricular activities. Furthermore, mapping strategies incorporating ventricular extrastimulation, including from different directions and coupling intervals, have been shown to increase the accuracy of substrate mapping. The implementation of extrastimulus substrate mapping and automated annotation require less extensive ablation and would make VT ablation procedures less cumbersome and accessible to more patients., (© 2022 Heart Rhythm Society. Published by Elsevier Inc.)

Background: Women with congenital heart disease at high risk for sudden cardiac death have been poorly studied thus far., Objectives: The aim of this study was to assess sex-related differences in patients with tetralogy of Fallot (TOF) and implantable cardioverter-defibrillators (ICDs)., Methods: Data were analyzed from the DAI-T4F (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator) cohort study, which has prospectively enrolled all patients with TOF with ICDs in France since 2010. Clinical events were centrally adjudicated by a blinded committee., Results: A total of 165 patients (mean age 42.2 ± 13.3 years) were enrolled from 40 centers, including 49 women (29.7%). Among the 9,692 patients with TOF recorded in the national database, the proportion of women with ICDs was estimated to be 1.1% (95% CI: 0.8%-1.5%) vs 2.2% (95% CI: 1.8%-2.6%) in men (P &lt; 0.001). The clinical profiles of patients at implantation, including the number of risk factors for ventricular arrhythmias, were similar between women and men. During a median follow-up period of 6.8 years (IQR: 2.5-11.4 years), 78 patients (47.3%) received at least 1 appropriate ICD therapy, without significant difference in annual incidences between women (12.1%) and men (9.9%) (HR: 1.22; 95% CI: 0.76-1.97; P = 0.40). The risk for overall ICD-related complications was similar in women and men (HR: 1.33; 95% CI: 0.81-2.19; P = 0.30), with 24 women (49.0%) experiencing at least 1 complication., Conclusions: Our findings suggest that women with TOF at high risk for sudden cardiac death have similar benefit/risk balance from ICD therapy compared with men. Whether ICD therapy is equally offered to at-risk women vs men warrants further evaluation in TOF as well as in other congenital heart disease populations. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574)., Competing Interests: Funding Support and Author Disclosures This work was supported by the French Institute of Health and Medical Research, Fédération Française de Cardiologie, and Société Française de Cardiologie. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to introduce a computed tomography (CT) protocol for optimal planning of vein of Marshall (VOM) catheterization., Background: Ethanol infusion into the VOM (Et-VOM) is increasingly used in atrial fibrillation ablation., Methods: Preprocedural CT was performed with either a conventional (conv-CT; n = 132) or an optimized CT protocol (VOM-CT; n = 126) designed for obtaining on a single image both left atrial and coronary sinus (CS) enhancement. The detection rate and anatomical features of the CT-derived VOM were analyzed and the utility of VOM-CT protocol was assessed by comparing the procedural data., Results: VOM was detected in 35% in conv-CT versus 63% in VOM-CT (P < 0.001). The VOM-CT protocol did not impair the assessment of left atrial anatomy and appendage patency. In VOM-CT, the detection of the VOM was related to body mass index and width of epicardial space on posterior wall. Mean distance between CS ostium and VOM was 36 ± 7 mm. Mean VOM diameter was 1.6 ± 0.3 mm. On the CS circumference, the VOM emerged superiorly in 68% and postero-superiorly in 32%. Ethanol infusion into the VOM was attempted in 165 patients (77 conv-CT, 70 VOM-CT, and 18 without-CT). After registration in CARTO, the VOM segmented on CT matched its location on venography in all cases. As compared with conv-CT and without-CT, procedures guided by VOM-CT showed significantly shorter radiation time, shorter procedure time, lower amount of the contrast medium, and fewer contrast injections to obtain VOM catheterization., Conclusions: The proposed CT protocol allows for improved visualization of the VOM, translating into easier VOM catheterization., Competing Interests: Funding Support and Author Disclosures Support was provided by the French National Agency for Research, Equipex-MUSIC ANR-11-EQPX-0030, and IHU-LIRYC ANR-10-IAHU-04. Drs Derval, Pambrun, Duchateau, and Sacher have received consulting fees and speaking honoraria from Biosense Webster. Drs Derval, Sacher, and Jaïs have received speaking honoraria from Boston Scientific. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study aimed to assess the impact of pulmonary valve replacement (PVR) on ventricular arrhythmias burden in a population of tetralogy of Fallot (TOF) patients with continuous cardiac monitoring by implantable cardioverter-defibrillators (ICDs)., Background: Sudden cardiac death is a major cause of death in TOF, and right ventricular overload is commonly considered to be a potential trigger for ventricular arrhythmias., Methods: Data were analyzed from a nationwide French ongoing study (DAI-T4F) including all TOF patients with an ICD since 2000. Survival data with recurrent events were used to compare the burden of appropriate ICD therapies before and after PVR in patients who underwent PVR over the study period., Results: A total of 165 patients (mean age 42.2 ± 13.3 years, 70.1% male) were included from 40 centers. Over a median follow-up period of 6.8 (interquartile range: 2.5 to 11.4) years, 26 patients (15.8%) underwent PVR. Among those patients, 18 (69.2%) experienced at least 1 appropriate ICD therapy. When considering all ICD therapies delivered before (n = 62) and after (n = 16) PVR, the burden of appropriate ICD therapies was significantly lower after PVR (HR: 0.21; 95% confidence interval [CI]: 0.08 to 0.56; p = 0.002). Respective appropriate ICD therapies rates per 100 person-years were 44.0 (95% CI: 35.7 to 52.5) before and 13.2 (95% CI: 7.7 to 20.5) after PVR (p < 0.001). In the overall cohort, PVR before ICD implantation was also independently associated with a lower risk of appropriate ICD therapy in primary prevention patients (HR: 0.29 [95% CI: 0.10 to 0.89]; p = 0.031)., Conclusions: In this cohort of high-risk TOF patients implanted with an ICD, the burden of appropriate ICD therapies was significantly reduced after PVR. While optimal indications and timing for PVR are debated, these findings suggest the importance of considering ventricular arrhythmias in the overall decision-making process. (French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator [DAI-T4F]; NCT03837574)., Competing Interests: Funding Support and Author Disclosures The Paris-Sudden Death Expertise Center activities are supported by the Institut National de la Santé et de la Recherche Médicale (INSERM), University of Paris, Assistance Publique-Hôpitaux de Paris, Fondation Coeur et Artères, Global Heart Watch, Fédération Française de Cardiologie, Société Française de Cardiologie, Fondation Recherche Medicale, as well as unrestricted grants from industrial partners (Abbott, Biotronik, Boston Scientific, Medtronic, MicroPort, Schiller and Zoll). SDEC Executive Committee is part of the ESCAPE-NET project (Horizon2020 programme). The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Several electrocardiographic (ECG) indices have been proposed to predict the origin of premature ventricular complexes (PVCs) with precordial transition in lead V 3 . However, the accuracy of these algorithms is limited., Objectives: We sought to evaluate a new ECG criterion differentiating the origin of outflow tract with precordial transition in lead V 3 ., Methods: We included in our study patients exhibiting outflow tract PVCs with precordial transition in lead V 3 referred for ablation. We analyzed a novel new ECG criterion, RV 1 -V 3 transition ratio, for distinguishing right from left idiopathic outflow tract PVCs with precordial transition in lead V 3 . The RV 1 -V 3 transition ratio was defined as (RV1+RV2+RV3) PVC / (RV1+RV2+RV3) SR (sinus rhythm)., Results: We included 58 patients in our study. The ratio was lower for right ventricular outflow tract origins than left ventricular outflow tract (LVOT) origins (median [interquartile range], 0.6953 [0.4818-1.0724] vs 1.5219 [1.1582-2.4313], P < .001). Receiver operating characteristic analysis revealed an area under the curve of 0.856 for the ratio, and a cut-off value of ≥0.9 predicting LVOT origin with 94% sensitivity and 73% specificity. This ratio was superior to any previously proposed ECG criterion for differentiating right from left outflow tract PVCs., Conclusion: The RV 1 -V 3 transition ratio is a simple and accurate novel ECG criterion for distinguishing right from left idiopathic outflow tract PVCs with precordial transition in lead V 3 ., (© 2021 Heart Rhythm Society. Published by Elsevier Inc.)

Objectives: This study systematically evaluated mechanisms of atrial tachycardia (AT) by using ultra-high-resolution mapping in a large cohort of patients., Background: An incomplete understanding of the mechanism of AT is a major determinant of ablation failure., Methods: Consecutive patients with ≥1 AT (excluding cavotricuspid isthmus-dependent flutter) were included. Mapping was performed with a 64-pole mapping catheter. The AT mechanism was defined based on activation mapping and confirmed by entrainment in selected cases., Results: A total of 132 patients were included (60 ± 12 years; 31 [23%] female; 111 [84%] previous atrial fibrillation [AF] ablation; 5 [4%] previous left atriotomy). One hundred four (94%) of the 111 post-AF ablation AT patients had substrate-based ablation during the index AF ablation. A total of 214 ATs were mapped, with complete definition of the AT mechanism in 206 (96%). A total of 129 (60%) had anatomic macro-re-entry (circuit diameter 44.2 ± 9.6 mm), 57 (27%) had scar-related localized re-entry (circuit diameter 25.8 ± 12.2 mm), and 20 (9%) had focal AT. Fifty-eight (45%) patients had multiple ATs (27 [20%] dual-loop re-entry; 60 [43%] sequential AT) with complex and highly variable transitions between AT circuits. A total of 116 (90%) of 129 macro-re-entrant ATs, 56 (98%) of 57 localized AT, and 20 (100%) of 20 focal ATs terminated after radiofrequency ablation. After a mean follow-up of 13 ± 9 months, 57 (46%) patients experienced recurrence of AT., Conclusions: Among patients with AT in the context of previous atrial interventions, particularly post-AF ablation patients, multiple complex AT circuits are common. Despite complete delineation of arrhythmia circuits using ultra-high-resolution mapping and high acute ablation success rates, long-term freedom from AT is modest., (Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Idiopathic ventricular fibrillation is diagnosed in patients who survived a ventricular fibrillation episode without any identifiable structural or electrical cause after extensive investigations. It is a common cause of sudden death in young adults. The study reviews the diagnostic value of systematic investigations and the new insights provided by detailed electrophysiological mapping. Recent studies have shown the high incidence of microstructural cardiomyopathic areas, which act as the substrate of ventricular fibrillation re-entries. These subclinical alterations require high-density endo- and epicardial mapping to be identified using electrogram criteria. Small areas are involved and located individually in various sites (mostly epicardial). Their characteristics suggest a variety of genetic or acquired pathological processes affecting cellular connectivity or tissue structure, such as cardiomyopathies, myocarditis, or fatty infiltration. Purkinje abnormalities manifesting as triggering ectopy or providing a substrate for re-entry represent a second important cause. The documentation of ephemeral Purkinje ectopy requires continuous electrocardiography monitoring for diagnosis. A variety of diseases affecting Purkinje cell function or conduction are potentially at play in their pathogenesis. Comprehensive investigations can therefore allow the great majority of idiopathic ventricular fibrillation to ultimately receive diagnoses of a cardiac disease, likely underlain by a mosaic of pathologies. Precise phenotypic characterization has significant implications for interpretation of genetic variants, the risk assessment, and individual therapy. Future improvements in imaging or electrophysiological methods may hopefully allow the identification of the subjects at risk and the development of primary prevention strategies., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives: A new electroanatomic mapping system (Rhythmia, Boston Scientific, Marlborough, Massachusetts) using a 64-electrode mapping basket is now available; we systematically assessed its use in complex congenital heart disease (CHD)., Background: The incidence of atrial arrhythmias post-surgery for CHD is high. Catheter ablation has emerged as an effective treatment, but is hampered by limitations in the mapping system's ability to accurately define the tachycardia circuit., Methods: Mapping and ablation data of 61 patients with CHD (35 males, age 45 ± 14 years) from 8 tertiary centers were reviewed., Results: Causes were as follows: Transposition of Great Arteries (atrial switch) (n = 7); univentricular physiology (Fontans) (n = 8); Tetralogy of Fallot (n = 10); atrial septal defect (ASD) repair (n = 15); tricuspid valve (TV) anomalies (n = 10); and other (n = 11). The total number of atrial arrhythmias was 86. Circuits were predominantly around the tricuspid valve (n = 37), atriotomy scar (n = 10), or ASD patch (n = 4). Although the majority of peri-tricuspid circuits were cavo-tricuspid-isthmus dependent (n = 30), they could follow a complex route between the annulus and septal resection, ASD patch, coronary sinus, or atriotomy. Immediate ablation success was achieved in all but 2 cases; with follow-up of 12 ± 8 months, 7 patients had recurrence., Conclusions: We demonstrate the feasibility of the basket catheter for mapping complex CHD arrhythmias, including with transbaffle and transhepatic access. Although the circuits often involve predictable anatomic landmarks, the precise critical isthmus is often difficult to predict empirically. Ultra-high-density mapping enables elucidation of circuits in this complex anatomy and allows successful treatment at the isthmus with a minimal lesion set., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to identify atrial tachycardia (AT) demonstrating atrial activation duration (AAD) lasting longer than the length of the tachycardia cycle (TCL); to assess AT prevalence; and to evaluate the mechanisms and characteristics associated with these AT episodes by using the Rhythmia system (Boston Scientific, Marlborough, Massachusetts)., Background: Ultra-high-density mapping allows very accurate characterization of mechanisms involved in AT. Some complex patterns may involve AAD which is longer than the tachycardia cycle length (TCL) which makes maps difficult to interpret. Prevalence and characteristics of such ATs are unknown., Methods: A cohort of 100 consecutive patients undergoing ablation of 125 right (n = 21) or left (n = 104) ATs using ultra-high-density mapping were retrospectively included. Offline calculation of right or left AAD was compared to TCL., Results: Mean TCL was 293 ± 65 ms, and mean AAD was 291 ± 74 ms (p = NS). AT mechanisms were macro-re-entry in 74 cases (59%), localized re-entry in 27 cases (22%), and focal AT in 21 cases (17%) (types were mixed in 3 cases). Fifteen ATs (12%) had AADs that were longer than the TCL (71 ± 45 ms longer, from 10 to 150 ms). TCL was equal to the AAD in 97 ATs (78%), whereas 13 ATs (10%) had AAD shorter than the TCL (focal AT in each case). There were no differences between right and left atria for prevalence of ATs with AADs that were longer than the TCLs. There were significant differences in AT mechanisms according to the AAD-to-TCL ratio (p < 0.0001), with localized re-entry showing more often that AAD was longer than the TCL compared to that in focal AT and macro-re-entry., Conclusions: ATs with AAD lasting longer than the TCL were present in approximately 10% of the ATs referred for ablation, mostly in ATs caused by localized re-entry. Ultra-high-density mapping allows detection of these complex patterns of activation., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study reports the use of a novel "Lumipoint" algorithm in ventricular tachycardia (VT) ablation., Background: Automatic mapping systems aid rapid acquisition of activation maps. However, they may annotate farfield rather than nearfield signal in low voltage areas, making maps difficult to interpret. The Lumipoint algorithm analyzes the complete electrogram tracing and therefore includes nearfield signals in its analysis., Methods: Twenty-two patients with ischemic cardiomyopathy and 5 with dilated cardiomyopathy underwent mapping using the ultra-high density Rhythmia system. Lumipoint algorithms were applied retrospectively., Results: In all left ventricular substrate maps, changing the window of interest to the post-QRS phase automatically identified late potentials. In 25 of 27 left ventricular VT activation maps, a minimum spatial window of interest correctly identified the VT isthmus as seen by the manually annotated map, entrainment, and response to ablation. In 6 maps, the algorithm identified the isthmus where the standard automatically annotated map did not., Conclusions: The Lumipoint algorithm automatically highlights areas with electrograms having specific characteristics or timings. This can identify late and fractionated potentials and regions that exhibit discontinuous activation, as well as the isthmus of a VT circuit. These features may enhance human interpretation of the electrogram signals during a case, particularly where the circuit lies in partial scar with low amplitude nearfield signals and potentially allow a more targeted ablation strategy., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study hypothesized that the association of D-dimer blood level and several clinical items in a new risk score could predict the absence of atrial thrombus., Background: Symptomatic and drug resistant atrial fibrillation (AF) can be treated by catheter ablation. The procedure-related risk of thromboembolism is limited by the pre-operative use of transesophageal echocardiography (TEE) to detect atrial thrombi., Methods: Patients admitted for catheter ablation of AF (n = 2,494) were prospectively included in a multicenter study. TEE was systematically performed before the procedure to search for atrial thrombus (primary endpoint). D-dimer level, CHADS 2 score, left ventricular ejection fraction, pre-operative anticoagulation regimen, and medical history were collected. A logistic regression model was used to identify factors associated with the presence of atrial thrombus (hypertension, history of stroke, heart failure, D-dimer level >270 ng/ml). These factors were aggregated in a new score called atrial thrombus exclusion (ATE)., Results: The incidence of atrial thrombus was 1.92%. CHADS 2 score and D-dimer level were significantly associated with atrial thrombus (p < 0.0001 and p < 0.0001, respectively). A zero CHADS 2 score failed to exclude all atrial thrombi (5 false negatives; sensitivity: 89.58%, specificity: 52.2%). No false negative was found with a zero ATE score, which had a specificity of 37% and a higher sensitivity (100%) than the CHADS 2 score (p < 0.031) to predict the absence of intra-atrial thrombi on TEE. Conversely, the positive predictive value was poor, and the ATE score should not be used to conclude a positive diagnosis of thrombus., Conclusions: An ATE score of zero was strongly associated with the absence of atrial thrombus. This new score could be useful to rule out a diagnosis of atrial thrombus before catheter ablation of AF., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to evaluate the relation between bipolar electrode spacing and far- and near-field electrograms., Background: The detailed effects of bipolar spacing on electrograms (EGMs) is not well described., Methods: With a HD-Grid catheter, EGMs from different bipole pairs could be created in each acquisition. This study analyzed the effect of bipolar spacing on EGMs in 7 infarcted sheep. A segment was defined as a 2-mm center-to-center bipole. In total, 4,768 segments (2,020 healthy, 1,542 scar, and 1,206 in border areas, as defined by magnetic resonance imaging [MRI]) were covered with an electrode pair of spacing of 2 mm (Bi-2), 4 mm (Bi-4), and 8 mm (Bi-8)., Results: A total of 3,591 segments in Bi-2 were free from local abnormal ventricular activities (LAVAs); 1,630 segments were within the MRI-defined scar and/or border area. Among them, 172 (10.6%) segments in Bi-4 and 219 (13.4%) segments in Bi-8 showed LAVAs. In contrast, LAVAs were identified in 1,177 segments in Bi-2; 1,118 segments were within the MRI-defined scar and/or border area. Among them, LAVAs were missed in 161 (14.4%) segments in Bi-4 and in 409 (36.6%) segments in Bi-8. In segments with LAVAs, median far-field voltage increased from 0.09 mV (25th to 75th percentile: 0.06 to 0.14 mV) in Bi-2, to 0.16 mV (25th to 75th percentile: 0.10 to 0.24 mV) in Bi-4, and to 0.28 mV (25th to 75th percentile: 0.20 to 0.42 mV) in Bi-8 (p < 0.0001). Median near-field voltage increased from 0.14 mV (25th to 75th percentile: 0.08 to 0.25 mV) in Bi-2, to 0.21 mV (25th to 75th percentile: 0.12 to 0.35 mV) in Bi-4, and to 0.32 mV (25th to 75th percentile: 0.17 to 0.48 mV) in Bi-8 (p < 0.0001). The median near-/far-field voltage ratio decreased from 1.67 in Bi-2, to 1.43 in Bi-4, and 1.23 in Bi-8 (p < 0.0001)., Conclusions: Closer spacing better discriminates surviving tissue from dead scar area. Although far-field voltage systematically increases with spacing, near-field voltages were more variable, depending on local surviving muscular bundles. Near-field EGMs are more easily observed with smaller spacing, largely due to the reduction of the far-field effect., (Copyright © 2019 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study aimed to evaluate the incidence, clinical impact, and predictors of late ventricular arrhythmias (VAs) in left ventricular assist device (LVAD) recipients aiming to clarify implantable cardioverter-defibrillator (ICD) indications., Background: The arrhythmic risk and need for ICD in patients implanted with an LVAD are not very well known., Methods: This observational study was conducted in 19 centers between 2006 and 2016. Late VAs were defined as sustained ventricular tachycardia or fibrillation occurring >30 days post-LVAD implantation, without acute reversible cause and requiring appropriate ICD therapy, external electrical shock, or medical therapy., Results: Among 659 LVAD recipients, 494 (median 58.9 years of age; mean left ventricular ejection fraction 20.7 ± 7.4%; 73.1% HeartMate II, 18.6% HeartWare, 8.3% Jarvik 2000) were discharged alive from hospital and included in the final analysis. Late VAs occurred in 133 (26.9%) patients. Multivariable analysis identified 6 independent predictors of late VAs: VAs before LVAD implantation, atrial fibrillation before LVAD implantation, idiopathic etiology of the cardiomyopathy, heart failure duration >12 months, early VAs (<30 days post-LVAD), and no angiotensin-converting enzyme inhibitors during follow-up. The "VT-LVAD score" was created, identifying 4 risk groups: low (score 0 to 1), intermediate (score 2 to 4), high (score 5 to 6), and very high (score 7 to 10). The rates of VAs at 1 year were 0.0%, 8.0%, 31.0% and 55.0%, respectively., Conclusions: Late VAs are common after LVAD implantation. The VT-LVAD score may help to identify patients at risk of late VAs and guide ICD indications in previously nonimplanted patients. (Determination of Risk Factors of Ventricular Arrhythmias [VAs] after implantation of continuous flow left ventricular assist device with continuous flow left ventricular assist device [CF-LVAD] [ASSIST-ICD]; NCT02873169)., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study evaluated a new algorithm relying on maximal pre-excitation., Background: Prior knowledge of accessory pathway (AP) location facilitates an individual ablation strategy. Delta-wave analysis on a 12-lead electrocardiogram is recognized as crucial for predicting ablation site, but can be ambiguous at basal state., Methods: An algorithm based on maximal pre-excitation, as induced by atrial pacing during an electrophysiological study, was initially developed in 132 patients with a single manifest AP. The maximally pre-excited QRS features included the global polarity in lead V 1 (step 1), inferior leads (step 2), and leads V 3 or I (step 3), as well as the morphology in lead II (step 4). Three investigators prospectively tested the new algorithm in 207 consecutive patients by comparing its efficacy to a control algorithm relying on basal pre-excitation., Results: The accuracy, defined as the percent of patients with an exact prediction of AP location, was significantly greater with the new algorithm (90% vs. 63%; p < 0.001). The reproducibility, defined as the level of agreement between investigators in determining AP location, was excellent (κ > 0.75; p < 0.05) with the new algorithm and fair (0.40 < κ < 0.75; p < 0.05) with the control algorithm., Conclusions: An algorithm based on maximal pre-excitation allows accurate and reproducible localization of manifest APs. When ablation is indicated, the analysis of maximal pre-excitation is a sensible approach for giving a head start in endocardial mapping., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to evaluate the influence of slow conducting anatomic isthmuses (SCAI) as dominant ventricular tachycardia (VT) substrate on QRS duration., Background: QRS prolongation has been associated with VT in repaired tetralogy of Fallot., Methods: Seventy-eight repaired tetralogy of Fallot patients (age 37 ± 15 years, 52 male, QRS duration 153 ± 29 ms, 67 right bundle branch blocks [RBBB]) underwent programmed stimulation and electroanatomic activation mapping during sinus rhythm. Right ventricular (RV) surface, RV activation pattern, RV activation time, conduction velocity at AI, and remote RV sites were determined., Results: Twenty-four patients were inducible for VT (VT+); SCAI was present in 22 of 24 VT+ but only in 2 of 54 patients without inducible VT (VT-). Conduction velocity through AI was slower in VT+ patients (median of 0.3 [0.3 to 0.4] vs. 0.7 [0.6 to 0.9] m/s; p < 0.01) but conduction velocity in the remote RV did not differ between groups. In non-RBBB, QRS duration was similar in VT+ patients (n = 6) and VT- patients (n = 5), but RV activation within SCAI exceeded QRS offset in VT+ patients (37 ± 20 ms vs. -5 ± 9 ms, p < 0.01). In RBBB, both QRS duration and RV activation time were longer in VT+ patients (n = 18, 17 of 18 QRS > 150 ms) compared with VT- patients (n = 49, 27 of 49 QRS > 150 ms) (173 ± 22 ms vs. 156 ± 20 ms; p < 0.01; 141 ± 22 ms vs. 129 ± 21 ms; p = 0.04). In VT+ patients, QRS prolongation >150 ms (n = 17) was due to SCAI or blocked isthmus in 15 patients (88%) and 1 (6%). In contrast, in VT- patients, QRS prolongation >150 ms (n = 27) was due to enlarged RV or blocked isthmus in 10 patients (37%) and 8 (30%), but due to SCAI in only 1 (4%). After exclusion of a severely enlarged RV, a QRS duration >150 ms was highly predictive for SCAI/blocked AI (OR: 17; 95% CI: 3.3 to 84; p < 0.01)., Conclusions: A narrow QRS interval does not exclude VT-related SCAI. In the presence of RBBB, SCAI further prolongs QRS duration. QRS duration >150 ms is highly suspicious for SCAI or isthmus block distinguishable by electroanatomic mapping., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to identify using implantable loop recorder (ILR) monitoring the mechanisms leading to sudden death (SD) in patients undergoing hemodialysis (HD)., Background: SD accounts for 11% to 25% of death in HD patients., Methods: Continuous rhythm monitoring was performed using the remote monitoring capability of the ILR device in patients undergoing HD at 8 centers. Clinical, biological, and technical HD parameters were recorded and analyzed., Results: Seventy-one patients (mean age 65 ± 9 years, 73% men) were included. Left ventricular ejection fraction was <50% in 16%. Twelve patients (17%) had histories of atrial fibrillation or flutter at inclusion. During a mean follow-up period of 21.3 ± 6.9 months, 16 patients died (14% patient-years), 7 (44%) of cardiovascular causes. Four SDs occurred, with progressive bradycardia followed by asystole. The incidence of patients presenting with significant conduction disorder and with ventricular arrhythmia was 14% and 9% patient-years, respectively. In multivariate survival frailty analyses, a higher risk for conduction disorder was associated with plasma potassium >5.0 mmol/l, bicarbonate <22 mmol/l, hemoglobin >11.5 g/dl, pre-HD systolic blood pressure >140 mm Hg, the longer interdialytic period, history of coronary artery disease, previous other arrhythmias, and diabetes mellitus. A higher risk for ventricular arrhythmia was associated with potassium <4.0 mmol/l, no antiarrhythmic drugs, and previous other arrhythmias. With ILR monitoring, de novo atrial fibrillation or flutter was diagnosed in 14 patients (20%)., Conclusions: ILR may be considered in HD patients prone to significant conduction disorders, ventricular arrhythmia, or atrial fibrillation or flutter to allow early identification and initiation of adequate treatment. Therapeutic strategies reducing serum potassium variability could decrease the rate of SD in these patients. (Implantable Loop Recorder in Hemodialysis Patients [RYTHMODIAL]; NCT01252823)., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this study was to describe and identify useful electrocardiographic characteristics to help identify the mechanism of atrial tachycardia (AT) occurring after persistent atrial fibrillation (PsAF) ablation., Background: Electrocardiographic analysis to help identify the mechanism of AT after PsAF ablation is much limited by the fact that remodeling and ablation alter the normal activation pattern., Methods: All consecutive patients who underwent mapping and ablation of AT after PsAF ablation were included. Surface P waves were analyzed during higher (>2:1) grades of atrioventricular block., Results: One hundred ninety-six ATs with visible P waves were identified in 127 patients (macro-re-entry in 57%, centrifugal AT in 43%). One-third displayed low-voltage P waves (≤0.1 mV). An isoelectric line >80 ms was more common in centrifugal compared with macro-re-entrant AT (47% vs. 24%; p < 0.001), but its positive predictive value was limited (60%). A minority of peritricuspid ATs displayed the classic saw-tooth pattern (27% [n = 22]). However, the "precordial transition" (a gradual transition from an upright component in lead V 1 to a negative component with progression across the precordium) remained often observed and specifically identified peritricuspid AT (specificity, 98%; sensitivity, 59%). Only 2 unique features could help identify perimitral AT (n = 60). First, the presence of a negative or negative-positive P-wave in any of leads V 2 to V 6 identified perimitral AT with 97% specificity and 30% sensitivity. Second, a "notched" negative component at the beginning of a positive P-wave in the inferior leads specifically identified clockwise perimitral AT (specificity, 98%; sensitivity, 25%)., Conclusions: Only few unique electrocardiographic characteristics help identify the mechanism of AT after PsAF ablation. Knowledge of these characteristics may aid in planning and performing ablation., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to assess the relationship between fibrosis and re-entrant activity in persistent atrial fibrillation (AF)., Background: The mechanisms involved in sustaining re-entrant activity during AF are poorly understood., Methods: Forty-one patients with persistent AF (age 56 ± 12 years; 6 women) were evaluated. High-resolution electrocardiographic imaging (ECGI) was performed during AF by using a 252-chest electrode array, and phase mapping was applied to locate re-entrant activity. Sites of high re-entrant activity were defined as re-entrant regions. Late gadolinium-enhanced (LGE) cardiac magnetic resonance (CMR) was performed at 1.25 × 1.25 × 2.5 mm resolution to characterize atrial fibrosis and measure atrial volumes. The relationship between LGE burden and the number of re-entrant regions was analyzed. Local LGE density was computed and characterized at re-entrant sites. All patients underwent catheter ablation targeting re-entrant regions, the procedural endpoint being AF termination. Clinical, CMR, and ECGI predictors of acute procedural success were then analyzed., Results: Left atrial (LA) LGE burden was 22.1 ± 5.9% of the wall, and LA volume was 74 ± 21 ml/m 2 . The number of re-entrant regions was 4.3 ± 1.7 per patient. LA LGE imaging was significantly associated with the number of re-entrant regions (R = 0.52, p = 0.001), LA volume (R = 0.62, p < 0.0001), and AF duration (R = 0.54, p = 0.0007). Regional analysis demonstrated a clustering of re-entrant activity at LGE borders. Areas with high re-entrant activity showed higher local LGE density as compared with the remaining atrial areas (p < 0.0001). Failure to achieve AF termination during ablation was associated with higher LA LGE burden (p < 0.001), higher number of re-entrant regions (p < 0.001), and longer AF duration (p = 0.008)., Conclusions: The number of re-entrant regions during AF relates to the extent of LGE on CMR, with the location of these regions clustering to LGE areas. These characteristics affect procedural outcomes of ablation.

Objectives: Using high-resolution 3-dimensional (3D) mapping, the aim of this study was to further characterize right atrial macro-re-entrant tachycardias and answer unresolved questions in the understanding of this arrhythmia., Background: Despite advances in understanding of the mechanisms of right atrial macro-re-entrant tachycardias, many questions lack definitive answers. The advent of high-resolution 3D mapping provides an opportunity to gain further insights into the nature of these common circuits., Methods: A total of 25 patients with right atrial macro-re-entrant tachycardia were studied. High-resolution 3D mapping (Rhythmia mapping system, Boston Scientific, Natick, Massachusetts) was performed. Regional voltage and conduction velocity were determined. Maps were analyzed to characterize wave front propagation patterns in all atrial regions. The relationship between substrate and conduction was evaluated., Results: A total of 42 right atrial macro-re-entrant circuits were observed. The most common location of the posterior line of block was the posteromedial right atrium (73%). This line of block continued superiorly into the superior vena cava, taking an oblique course to finish on the anterior superior vena cava aspect in 73%. Conduction delay at the crista terminalis was less common (23%). Conduction slowing or block was seen at the limbus of the fossa ovalis (73%) and Eustachian ridge (77%). Highly variable and localized areas of slow conduction were also observed in the inferior septum (45%), superior septum (27%), anterosuperior right atrium (23%), and lateral right atrium (23%). Localized conduction slowing was seen in the cavotricuspid isthmus in 50% of patients, but there was no generalized conduction slowing in this isthmus. The voltage in regions of slow conduction was significantly lower compared with areas of normal conduction velocity (p < 0.001). Conduction channels were observed in 55% of patients., Conclusions: High-resolution 3D mapping has provided new insights into the nature of right atrial macro-re-entrant tachycardias. Variable regions of abnormal atrial substrate were associated with conduction slowing and block. Individual variation in propagation patterns was observed in association with this variable substrate. (Mapping of Atrial Arrhythmias Using High Spatial Resolution Mapping Catheters and the Rhythmia Mapping System; ACTRN12615000544572)., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: The early repolarization (ER) pattern is a common ECG finding. Recent studies established a definitive clinical association between ER and fatal ventricular arrhythmias. However, the arrhythmogenic substrate of ER in the intact human heart has not been characterized., Objectives: To map the epicardial electrophysiological (EP) substrate in ER syndrome patients using noninvasive Electrocardiographic Imaging (ECGI), and to characterize substrate properties that support arrhythmogenicity., Methods: Twenty-nine ER syndrome patients were enrolled, 17 of which had a malignant syndrome. Characteristics of the abnormal EP substrate were analyzed using data recorded during sinus rhythm. The EP mapping data were analyzed for electrogram morphology, conduction and repolarization. Seven normal subjects provided control data., Results: The abnormal EP substrate in ER syndrome patients has the following properties: (1) Abnormal epicardial electrograms characterized by presence of J-waves in localized regions; (2) Absence of conduction abnormalities, including delayed activation, conduction block, or fractionated electrograms; (3) Marked abbreviation of ventricular repolarization in areas with J-waves. The action potential duration (APD) was significantly shorter than normal (196±19 vs. 235±21 ms, p<0.05). Shortening of APD occurred heterogeneously, leading to steep repolarization gradients compared to normal control (45±17 vs.7±5 ms/cm, p<0.05). Premature ventricular contractions (PVCs) were recorded in 2 patients. The PVC sites of origin were closely related to the abnormal EP substrate with J-waves and steep repolarization gradients., Conclusions: Early Repolarization is associated with steep repolarization gradients caused by localized shortening of APD. Results suggest association of PVC initiation sites with areas of repolarization abnormalities. Conduction abnormalities were not observed.

Objectives: This study sought to characterize the clinical characteristics, atrial substrate, and prognosis in a subgroup of patients with persistent atrial fibrillation (AF) from the onset (PsAFonset)., Background: Patients with AF frequently progress from trigger-driven paroxysmal arrhythmias to substrate-dependent persistent arrhythmias., Methods: Patients referred for persistent AF (PsAF) ablation were enrolled from 3 centers. Consecutive patients with PsAFonset (n = 129) were compared with patients with PsAF that progressed from paroxysmal AF (n = 231). In addition, 90 patients (30 patients with PsAFonset and 60 control subjects) were studied with noninvasive mapping to characterize the AF drivers. The degree of fractionation and endocardial voltages were assessed invasively., Results: Patients with PsAFonset were younger (p = 0.047) and more obese (p < 0.001); there were more men (p = 0.034), more patients with hypertension (p = 0.044), and these patients had larger left (p < 0.05) and right atria (p < 0.05). Baseline AF cycle length was shorter in the PsAFonset group (p < 0.01); the degree of fractionation was higher (p < 0.001 for both atria), and the endocardial voltage was lower (p < 0.05 for both atria). Patients with PsAFonset had higher a number of re-entrant driver regions (p < 0.001) and extrapulmonary vein regions that had re-entrant drivers (p < 0.05), whereas control subjects displayed more focal driver regions (p = 0.029). The acute AF termination rate was lower in the PsAFonset group (42% vs. 81%; p < 0.001). During a mean follow-up of 17 ± 11 months from the last procedure, patients with PsAFonset had significantly higher AF, atrial tachycardia (AT), and AF/AT recurrence rates (p < 0.01)., Conclusions: Patients with PsAFonset represent a distinct subgroup defined by specific demographics, underlying diffuse biatrial substrate disease, and worse clinical outcome. The findings highlight the importance of defining criteria for early detection of atrial substrate disease., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)