Marco Bergonti, Philipp Krisai, Ciro Ascione, Thomas Pambrun, Domenico G Della Rocca, Paolo Compagnucci, Tardu Özkartal, Lorenzo Marcon, Teba Gonzalez Ferrero, Luigi Pannone, Michael Kühne, Matteo Anselmino, Michela Casella, Teodor Serban, Claudio Tondo, Moises Rodríguez-Mañero, Maria Luce Caputo, Patrick Badertscher, Nicolas Derval, Carlo de Asmundis, Gian Battista Chierchia, Hein Heidbuchel, Pierre Jaïs, Angelo Auricchio, Andrea Sarkozy, Giulio Conte
{"title":"Different Approaches to Atrial Fibrillation Ablation in Heart Failure Patients: Temporal Trends and Clinical Outcomes.","authors":"Marco Bergonti, Philipp Krisai, Ciro Ascione, Thomas Pambrun, Domenico G Della Rocca, Paolo Compagnucci, Tardu Özkartal, Lorenzo Marcon, Teba Gonzalez Ferrero, Luigi Pannone, Michael Kühne, Matteo Anselmino, Michela Casella, Teodor Serban, Claudio Tondo, Moises Rodríguez-Mañero, Maria Luce Caputo, Patrick Badertscher, Nicolas Derval, Carlo de Asmundis, Gian Battista Chierchia, Hein Heidbuchel, Pierre Jaïs, Angelo Auricchio, Andrea Sarkozy, Giulio Conte","doi":"10.1016/j.jacep.2024.12.004","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.12.004","url":null,"abstract":"<p><strong>Background: </strong>Catheter ablation of atrial fibrillation has been proven beneficial in patients with heart failure and reduced ejection fraction (HFrEF). On top of pulmonary vein isolation (PVI), additional ablation strategies such as empirical lines/posterior box/low voltage ablation (PVI+L/LVA) are often used. Whether PVI+L/LVA provides additional benefits in this population has never been systematically investigated.</p><p><strong>Objectives: </strong>This study sought to analyze the temporal trends and comparative outcomes of PVI+L/LVA vs PVI alone.</p><p><strong>Methods: </strong>Patients with HFrEF undergoing atrial fibrillation ablation were retrospectively enrolled. The 2 coprimary endpoints were ventricular function recovery and atrial fibrillation recurrence-free survival at 1 year. The performance of PVI and PVI+L/LVA was compared in the overall population and in 2 matched groups. A sensitivity analysis for measured confounders was performed.</p><p><strong>Results: </strong>A total of 955 HFrEF patients (62.1 years, 24.4% female) from 9 international centers were included (PVI only 51.6% vs PVI+L/LVA 48.4%). At 12 months after the procedure, 62.3% of the patients remained free from arrhythmia recurrences and 65.4% experienced ventricular function recovery. Comparing PVI to PVI+L/LVA, no significant difference in the 2 coprimary endpoints was observed, neither in the overall nor in the matched cohorts. The use of PVI+L/LVA increased from 27% in 2013 to 68% in 2022. Patients undergoing PVI+L/LVA experienced more complications (3.8 vs 1.2%; P = 0.018).</p><p><strong>Conclusions: </strong>Catheter ablation is associated with significant improvements in systolic function, irrespective of the ablation strategy used. The use of PVI+L/LVA in HFrEF patients is progressively expanding over time. Although the benefits of this practice remain unproven, it is associated with an increased risk of overall and nonvascular complications. These results warrant caution regarding the growing use of PVI+ in HFrEF patients.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143078955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandra Pia Porretta, Mathieu Le Bloa, Zahurul Alam Bhuiyan, Nicole Sekarski, Isis Atallah, Cédric Le Caignec, Fabrice Extramiana, Etienne Pruvot
{"title":"Arrhythmogenic Potential of Heterozygous TECRL Variants in Type 3 Catecholaminergic Polymorphic Ventricular Tachycardia.","authors":"Alessandra Pia Porretta, Mathieu Le Bloa, Zahurul Alam Bhuiyan, Nicole Sekarski, Isis Atallah, Cédric Le Caignec, Fabrice Extramiana, Etienne Pruvot","doi":"10.1016/j.jacep.2024.12.015","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.12.015","url":null,"abstract":"<p><p>TECRL is the causative gene of an autosomal-recessive form of catecholaminergic polymorphic ventricular tachycardia (CPVT), the so-called type 3 CPVT. However, only 17 families have been reported worldwide and no case of symptomatic heterozygous carriers has been described. We report herein genotypes and clinical phenotypes of a family of European ancestry harboring a new TECRL pathogenic variant and, for the first time, a CPVT-like phenotype in a TECRL heterozygous variant carrier. Due to this novel evidence, clinicians should be aware that TECRL heterozygous variant carriers should undergo cardiac assessment and therapy introduction in case of a CPVT clinical diagnosis.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143407814","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Soonil Kwon, So-Ryoung Lee, Eue-Keun Choi, Bongsung Kim, Kyung-Do Han, JungMin Choi, Kyung-Yeon Lee, Hyo-Jeong Ahn, Seil Oh, Gregory Y H Lip
{"title":"Benefits of Early Catheter Ablation in Patients With Atrial Fibrillation: A Nationwide Cohort Study.","authors":"Soonil Kwon, So-Ryoung Lee, Eue-Keun Choi, Bongsung Kim, Kyung-Do Han, JungMin Choi, Kyung-Yeon Lee, Hyo-Jeong Ahn, Seil Oh, Gregory Y H Lip","doi":"10.1016/j.jacep.2024.12.023","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.12.023","url":null,"abstract":"<p><strong>Background: </strong>Although early rhythm control showed better clinical outcomes in patients with atrial fibrillation (AF), limited data exist on the relationship between the timing of atrial fibrillation catheter ablation (AFCA) and clinical outcomes.</p><p><strong>Objectives: </strong>This study investigated the impact of early vs late AFCA on clinical outcomes.</p><p><strong>Methods: </strong>Using the Korean claims database, 944,710 AF patients without previous AFCA were investigated. These patients were categorized into 3 groups based on the duration from their AF diagnosis to AFCA: <1 year (early-AFCA), 1 to <3 years (intermediate-AFCA), and ≥3 years (late-AFCA). The study outcomes included ischemic stroke, heart failure (HF) hospitalization, myocardial infarction, major adverse cardiovascular events (comprising the aforementioned three), all-cause mortality, a composite outcome (encompassing all mentioned outcomes), and AF recurrence.</p><p><strong>Results: </strong>A total of 57,804 AF patients (38,536 without AFCA and 19,268 with AFCA; mean age 57.8 years; 73.5% men) were followed up for an average of 2.6 ± 1.5 years. AFCA was associated with lower risks across all study outcomes (HRs: 0.26-0.41 for all outcomes; all P < 0.001). Compared with the early-AFCA group, the intermediate- and late-AFCA groups showed higher risks of HF hospitalization and all-cause mortality. Additionally, the late-AFCA group had higher risks of major adverse cardiovascular events, composite outcomes, and AF recurrence than those of the early- and intermediate-AFCA groups. After weighing the propensity scores, the results were largely consistent with the primary analysis.</p><p><strong>Conclusions: </strong>Early AFCA showed improved clinical outcomes, primarily caused by decreased risk of HF hospitalization and AF recurrence.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hursh Naik, Matthew J Price, Samir Kapadia, Brian K Whisenant, Peter Tadros, Raj Makkar, Anita W Asgar, Neil Fam, Gilbert H L Tang, Shamir R Mehta, Timothy Byrne, Gagan Singh, Sidakpal S Panaich, Kelli Peterman, Phillip M Trusty, Nadira Hamid, Rebecca T Hahn, David H Adams, Paul Sorajja
{"title":"Tricuspid Transcatheter Edge-to-Edge Repair in Patients With Transvalvular CIED Leads: The TRILUMINATE Pivotal Trial.","authors":"Hursh Naik, Matthew J Price, Samir Kapadia, Brian K Whisenant, Peter Tadros, Raj Makkar, Anita W Asgar, Neil Fam, Gilbert H L Tang, Shamir R Mehta, Timothy Byrne, Gagan Singh, Sidakpal S Panaich, Kelli Peterman, Phillip M Trusty, Nadira Hamid, Rebecca T Hahn, David H Adams, Paul Sorajja","doi":"10.1016/j.jacep.2025.01.001","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.001","url":null,"abstract":"<p><strong>Background: </strong>Patients with tricuspid regurgitation (TR) frequently have transvalvular cardiac implantable electronic device (CIEDs).</p><p><strong>Objectives: </strong>The aim of this study was to determine the safety and efficacy of tricuspid transcatheter edge-to-edge repair in patients with transvalvular CIED leads.</p><p><strong>Methods: </strong>The TRILUMINATE (Trial to Evaluate Cardiovascular Outcomes in Patients Treated With the Tricuspid Valve Repair System) Pivotal Trial (NCT03904147) is an international randomized, controlled trial in symptomatic subjects with severe TR. Subjects with CIED leads were screened by an eligibility committee prior to inclusion into the randomized or single-arm cohorts. Safety events were adjudicated by an independent review committee. All echocardiograms were analyzed by an independent core laboratory.</p><p><strong>Results: </strong>A total of 98 subjects (of 469) with attempted TriClip procedures had transvalvular CIED leads. CIED+ subjects were older (80.2 ± 8.6 years vs 78.2 ± 7.6 years; P = 0.02), with a higher prevalence of renal disease (46.9% vs 31.5%; P = 0.004) and lower health status (Kansas City Cardiomyopathy Questionnaire overall summary score 51.9 ± 21.0 vs 55.0 ± 23.1) at baseline compared with CIED- subjects. Slightly fewer clips were implanted in CIED+ subjects (mean 1.9) than in CIED- subjects (mean 2.2) (P = 0.0018). Procedural times were significantly shorter in CIED+ subjects (132.9 ± 63.3 minutes vs 155.9 ± 71.9 minutes; P = 0.0043) although greater in those with lead-induced TR (149.5 ± 87.5 minutes). Major adverse events (1.0% vs 1.1%) and major bleeding (3.1% vs 3.0%) were infrequent in CIED+ and CIED- subjects. At 30 days, TR reduction to moderate or less was similar in CIED+ and CIED- subjects (88% vs 87%) and was sustained out to 1 year in the majority of subjects (81% vs 84%). Compared with baseline, Kansas City Cardiomyopathy Questionnaire overall summary score significantly improved through 1 year in CIED+ subjects (18.7 ± 22.6; P < 0.0001) and CIED- subjects (16.8 ± 22.6; P < 0.0001). Heart failure symptoms were reduced in both groups at 30 days, with 85% of CIED+ subjects and 87% of CIED- subjects in NYHA functional class I or II. There were no differences in mortality, heart failure hospitalization, and need for tricuspid valve surgery or intervention between groups. No lead revisions, removals, or replacements were reported through follow-up.</p><p><strong>Conclusions: </strong>Tricuspid transcatheter edge-to-edge repair with the TriClip system was safe and effective in selected CIED+ subjects and did not affect CIED function. CIED+ subjects experienced similar TR reduction and quality-of-life improvements as CIED- subjects. Future work should define the treatable scope of patients with transvalvular CIED leads. (TRILUMINATE Pivotal Trial; NCT03904147).</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Tricuspid Transcatheter Edge-to-Edge Repair: An Approach Whose Time has Come for Electrophysiology.","authors":"Jamil Aboulhosn, Kalyanam Shivkumar","doi":"10.1016/j.jacep.2025.01.002","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.002","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
John A Staples, Daniel Daly-Grafstein, Isaac Robinson, Mayesha Khan, Nathaniel M Hawkins, Herbert Chan, Shannon Erdelyi, Christian Steinberg, K Malcolm Maclure, Andrew D Krahn, Jeffrey R Brubacher
{"title":"Cardioverter-Defibrillator Implantation as a Risk Factor For Motor Vehicle Crash.","authors":"John A Staples, Daniel Daly-Grafstein, Isaac Robinson, Mayesha Khan, Nathaniel M Hawkins, Herbert Chan, Shannon Erdelyi, Christian Steinberg, K Malcolm Maclure, Andrew D Krahn, Jeffrey R Brubacher","doi":"10.1016/j.jacep.2024.12.002","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.12.002","url":null,"abstract":"<p><strong>Background: </strong>Limited empirical evidence informs fitness-to-drive recommendations after implantable cardioverter-defibrillator (ICD) implantation. Cohort designs can be deceptive because ICD recipients differ from control individuals and may temporarily cease driving after implantation.</p><p><strong>Objectives: </strong>This study sought to generate evidence to inform medical driving restrictions after ICD implantation.</p><p><strong>Methods: </strong>We used population-based data to identify all drivers involved in a serious motor vehicle crash in British Columbia, Canada, from 1997 to 2019. Exposure was defined as ICD implantation in the 6 months before a crash. One analysis used a case-crossover design to control for relatively fixed individual characteristics like driving experience. Another analysis used a responsibility design to account for road exposure (miles of driving per week). Both analyses used logistic regression with adjustment for potential confounders.</p><p><strong>Results: </strong>In the case-crossover analysis of crash-involved ICD recipients, ICD implantation occurred in 212 of 3,299 precrash intervals and in 485 of 6,598 control intervals, suggesting no temporal association between ICD implantation and subsequent crash (6.4% vs 7.4%; adjusted odds ratio [aOR]: 0.86; 95% CI: 0.71-1.03; P = 0.11). In the analysis of all crash-involved drivers with determinate crash responsibility, 14 of 22 drivers with recent ICD implantation and 532,741 of 1,035,433 drivers without recent ICD implantation were deemed responsible for their crash, suggesting no association between ICD implantation and crash responsibility (crude proportion responsible, 64% vs 51%; aOR: 2.20; 95% CI: 0.94-5.30; P = 0.08).</p><p><strong>Conclusions: </strong>The 6-month interval after ICD implantation is not associated with increased odds of crash nor with increased likelihood of crash responsibility. Contemporary driving restrictions in the first weeks after ICD implantation appear to adequately mitigate the potential increase in crash risk.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143079997","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Superior Vena Cava Isolation With Pulsed Field Ablation: Feasible and Safe, but Necessary?","authors":"David Schaack, K R Julian Chun, Boris Schmidt","doi":"10.1016/j.jacep.2024.12.019","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.12.019","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chase M Fiore, Kate Quigley, Igor Vorobyov, Colleen E Clancy, Robert D Harvey
{"title":"Effect of the Membrane Environment on Pharmacologic Inhibition of hERG K<sup>+</sup> Channel Activity.","authors":"Chase M Fiore, Kate Quigley, Igor Vorobyov, Colleen E Clancy, Robert D Harvey","doi":"10.1016/j.jacep.2024.11.016","DOIUrl":"10.1016/j.jacep.2024.11.016","url":null,"abstract":"<p><strong>Background: </strong>hERG encodes K<sub>V</sub>11.1 voltage-gated K<sup>+</sup> channels, which generate the rapidly activating delayed rectifier K<sup>+</sup> current that contributes to repolarization of the cardiac action potential. In addition to being targeted by many class III antiarrhythmic agents, these channels are also inhibited by a multitude of other pharmacological compounds, which can produce acquired long QT syndrome, leading to polymorphic ventricular tachycardia. While most drugs are thought to interact with a hydrophilic binding site in the channel pore, it has been postulated that some compounds act by perturbing the membrane environment or acting at hydrophobic sites accessed through the plasma membrane.</p><p><strong>Objectives: </strong>Because hERG channels reside in cholesterol rich lipid raft domains, we hypothesized that disrupting the membrane environment by depleting cholesterol might alter inhibition of channel activity by certain drugs.</p><p><strong>Methods: </strong>We tested our hypothesis by examining the effect that depleting membrane cholesterol with methyl-β-cyclodextrin has on the ability of several compounds to inhibit hERG channels expressed in HEK293 cells.</p><p><strong>Results: </strong>We found that cholesterol depletion significantly increased the sensitivity of the whole cell current to inhibition by ibutilide, while decreasing the currents sensitivity to dofetilide and amiodarone at negative membrane potentials.</p><p><strong>Conclusions: </strong>These results support the idea that the lipid environment of the plasma membrane plays a role in the ability of certain drugs to inhibit hERG channel activity. Differences in membrane cholesterol content may affect the ability of some hERG channel blockers to produce arrhythmogenic behavior.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143079189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Steven Liskov, Jenna A Milstein, Andreas S Barth, Ari Cedars, Bret A Mettler, Danielle Gottlieb Sen, Konstantinos N Aronis
{"title":"Epicardial Mapping and Ablation of Focal Atrial Tachycardia from the Crista Terminalis During Cardiac Surgery.","authors":"Steven Liskov, Jenna A Milstein, Andreas S Barth, Ari Cedars, Bret A Mettler, Danielle Gottlieb Sen, Konstantinos N Aronis","doi":"10.1016/j.jacep.2024.12.021","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.12.021","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Brett C Austin, Gurukripa N Kowlgi, Raquel Almeida Lopes Neves, Konstantinos C Siontis, J Martijn Bos, John R Giudicessi, Michael J Ackerman
{"title":"Incidence and Clinical Management of Supraventricular Arrhythmias in Patients With Catecholaminergic Polymorphic Ventricular Tachycardia.","authors":"Brett C Austin, Gurukripa N Kowlgi, Raquel Almeida Lopes Neves, Konstantinos C Siontis, J Martijn Bos, John R Giudicessi, Michael J Ackerman","doi":"10.1016/j.jacep.2024.11.018","DOIUrl":"https://doi.org/10.1016/j.jacep.2024.11.018","url":null,"abstract":"<p><strong>Background: </strong>Smaller studies suggest supraventricular arrhythmias (SVAs) are common in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT).</p><p><strong>Objectives: </strong>This study aimed to determine the incidence, type, and clinical management of SVAs observed within a large, single-center cohort of CPVT cases.</p><p><strong>Methods: </strong>The electronic medical records of 206 patients (51% female; average age at diagnosis 21 ± 17 years) diagnosed clinically with CPVT between January 2000 and September 2023 were reviewed for electrocardiographic evidence of SVAs, including atrial fibrillation (AF), atrial flutter (AFL), atrial tachycardia (AT), and supraventricular tachycardia. SVAs were considered clinically significant when sustained for >30 seconds, with or without symptoms, ultimately necessitating clinical evaluation. SVA type, symptoms, and therapeutic efficacy were assessed.</p><p><strong>Results: </strong>Overall, 17 (8.3%) of 206 patients had evidence of an SVA (AF/AFL in 8, AT in 9, and supraventricular tachycardia in 1 [1 patient experienced both AT and AF/AFL]). The median age at SVA diagnosis was 28 years (Q1-Q3: 16-34 years). A total of 11 (65%) of 17 patients were symptomatic, 3 (27%) of whom experienced inappropriate shocks. All patients were trialed on antiarrhythmics. Owing to drug failure, intolerance, or patient/provider preference; 5 (29%) of 17 patients with SVAs underwent radiofrequency ablation. Notably, over a median follow-up duration of 11 months (Q1-Q3: 5-45 months), 1 SVA recurrence was observed in a patient treated medically.</p><p><strong>Conclusions: </strong>In comparison with prior studies, the incidence of SVAs in this large, single-center CPVT cohort was substantially lower (8.3% vs 26%-35%). Although a larger multicenter study is needed to confirm, this study suggests that radiofrequency ablation durably treats CPVT-associated SVAs.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143364769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}