{"title":"Decades of Defibrillation in Brugada Syndrome","authors":"Andrew D. Krahn MD, Benjamin M. Moore MBBS, PhD","doi":"10.1016/j.jacep.2025.03.034","DOIUrl":"10.1016/j.jacep.2025.03.034","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1189-1192"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144173742","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}
Sean Gaine MD , Thomas Rolland MD , Babken Asatryan MD, PhD , Mikael Laredo MD, PhD , James Sampognaro MD , Richard T. Carrick MD, PhD , Giovanni Peretto MD, PhD , Steven Muller MD , Andrea Villatore MD , Brittney Murray CGC , Crystal Tichnell CGC, RN , Anneline S.J.M. te Riele MD, PhD , Peter Loh MD, PhD , Paolo Compagnucci MD, PhD , Michela Casella MD, PhD , Marika Martini MD , Marco Schiavone MD , Claudio Tondo MD, PhD , Chiara Cappelletto MD , Gianfranco Sinagra MD, PhD , Alessio Gasperetti MD, PhD
{"title":"Long-Term Follow-Up Data on Flecainide Use as an Antiarrhythmic in Arrhythmogenic Right Ventricular Cardiomyopathy","authors":"Sean Gaine MD , Thomas Rolland MD , Babken Asatryan MD, PhD , Mikael Laredo MD, PhD , James Sampognaro MD , Richard T. Carrick MD, PhD , Giovanni Peretto MD, PhD , Steven Muller MD , Andrea Villatore MD , Brittney Murray CGC , Crystal Tichnell CGC, RN , Anneline S.J.M. te Riele MD, PhD , Peter Loh MD, PhD , Paolo Compagnucci MD, PhD , Michela Casella MD, PhD , Marika Martini MD , Marco Schiavone MD , Claudio Tondo MD, PhD , Chiara Cappelletto MD , Gianfranco Sinagra MD, PhD , Alessio Gasperetti MD, PhD","doi":"10.1016/j.jacep.2025.02.023","DOIUrl":"10.1016/j.jacep.2025.02.023","url":null,"abstract":"<div><h3>Background</h3><div>Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with a high risk of ventricular arrhythmia (VA). Several animal models have been used to postulate a therapeutic role of the inhibition of the ryanodine 2 receptor via the use of flecainide for this disease. Clinical data describing its use are scarce, however, especially in patients without implantable cardioverter-defibrillators or with left ventricular (LV) involvement.</div></div><div><h3>Objectives</h3><div>This study sought to report safety and effectiveness long-term, multicenter data on the impact of flecainide therapy on arrhythmic outcomes in patients with a definite diagnosis of ARVC.</div></div><div><h3>Methods</h3><div>Patients with definite ARVC receiving flecainide at 12 academic institutions were enrolled in the study. Baseline was defined as the time of flecainide initiation. Premature ventricular complex burdens, nonsustained ventricular tachycardia (NSVT) rates, and sustained VA yearly/rates were collected and compared while on and off flecainide. Side effects and flecainide discontinuation were tracked. Analyses were performed in the overall cohort as well as stratifying for genotype (gene positive vs negative; plakohpillin-2 [PKP-2] vs non PKP-2) and for LV involvement.</div></div><div><h3>Results</h3><div>A total of 191 patients (age 37.9 ± 13.7 years; 69.0% male; 89.0% probands; 59.2% having implantable cardioverter-defibrillators; 33.0% with prior VA; 43.5% PKP-2<sup>+</sup>; LV ejection fraction 55.9 ± 7.3%; right ventricular ejection fraction 44.5 ± 10.5% at baseline) were enrolled, with 66 patients (34.6%) showing LV involvement. The median dose of flecainide was 200 mg/d [150-200 mg/d], with 166 patients (86.9%) also taking a beta-blocker. The median follow-up time on flecainide was 4.2 years [1.9-6.3 years]. Flecainide was well tolerated, with a low (7.9%) discontinuation rate. After flecainide initiation, a significant reduction in the 24-hour premature ventricular complex burden and in the rate of nonsustained ventricular tachycardia was observed (2,190 vs 418; <em>P</em> < 0.001; 35.1% vs 21.5%; <em>P</em> = 0.003). For patients with prior VA events, a significant reduction in the amount of VA episodes/y (1.1 [0.4-1.6] episodes/y vs 0 [0-0.3] episodes/y; <em>P</em> < 0.001) was observed. These safety and effectiveness findings were consistent across genotype subgroups, as well as in patients with and without LV involvement.</div></div><div><h3>Conclusions</h3><div>Flecainide use had a favorable safety profile and was associated with an observed to a significant reduction in arrhythmic burden in patients with ARVC, irrespective of the underlying genotype or LV involvement.</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1159-1170"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144003482","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":"Time to Begin Again?","authors":"Benjamin A. Steinberg MD, MHS","doi":"10.1016/j.jacep.2025.03.033","DOIUrl":"10.1016/j.jacep.2025.03.033","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1263-1264"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144173816","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}
Cinzia Monaco MD , Maria Cespon-Fernandez MD, PhD , Luigi Pannone MD , Alvise Del Monte MD , Domenico Della Rocca MD, PhD , Anais Gauthey MD, PhD , Sahar Mouram MD , Lorenzo Marcon MD , Giampaolo Vetta MD , Charles Audiat MD , Ioannis Doundoulakis MD , Antonio Bisignani MD , Vincenzo Miraglia MD , Gudrun Pappaert MSc , Ivan Eltsov MD , Gezim Bala MD, PhD , Antonio Sorgente MD, PhD , Ingrid Overeinder MD , Alexandre Almorad MD , Erwin Stroker MD, PhD , Carlo de Asmundis MD, PhD
{"title":"Implantable Cardioverter-Defibrillator Therapy in Brugada Syndrome","authors":"Cinzia Monaco MD , Maria Cespon-Fernandez MD, PhD , Luigi Pannone MD , Alvise Del Monte MD , Domenico Della Rocca MD, PhD , Anais Gauthey MD, PhD , Sahar Mouram MD , Lorenzo Marcon MD , Giampaolo Vetta MD , Charles Audiat MD , Ioannis Doundoulakis MD , Antonio Bisignani MD , Vincenzo Miraglia MD , Gudrun Pappaert MSc , Ivan Eltsov MD , Gezim Bala MD, PhD , Antonio Sorgente MD, PhD , Ingrid Overeinder MD , Alexandre Almorad MD , Erwin Stroker MD, PhD , Carlo de Asmundis MD, PhD","doi":"10.1016/j.jacep.2025.01.013","DOIUrl":"10.1016/j.jacep.2025.01.013","url":null,"abstract":"<div><h3>Background</h3><div>Brugada syndrome (BrS) continues to pose clinical challenges, despite 3 decades of dedicated research and therapeutic advancements. The pivotal role of implantable cardioverter-defibrillator (ICD) therapy in safeguarding high-risk BrS patients from sudden cardiac death due to ventricular arrhythmias is undeniable. However, the debate on risk stratification and the use of ICDs for primary prevention remains ongoing.</div></div><div><h3>Objectives</h3><div>This study aimed to evaluate the clinical features, management, and long-term outcomes of ICD therapy in patients with Brugada syndrome.</div></div><div><h3>Methods</h3><div>BrS-diagnosed patients were prospectively enrolled. Inclusion criteria were: 1) a Brugada type 1 electrocardiogram pattern, either spontaneous or drug induced; 2) ICD implantation; and 3) consistent follow-up. Risk stratification was based on prior arrhythmic events, and the multiparametric Brussel risk score was used from 2017. High-risk patients underwent video-thoracoscopic epicardial ablation starting in 2016. ICD implantation strategies evolved over time, guided by patients' clinical and demographic characteristics.</div></div><div><h3>Results</h3><div>A total of 306 consecutive Brugada patients (186 male [61%]; mean age 41 ± 17 years; range: 1-82 years) received ICDs at our institution from 1992 to 2022. ICDs were implanted for secondary prevention in 16% of patients. Over the 3 decades, the proportions of secondary prevention implants and asymptomatic patients remained stable, while risk factors fluctuated in the first two decades before stabilizing. During long-term follow-up (median 103 months [63-147 months]), 14% of patients experienced at least 1 sustained ventricular arrhythmia (VA) (1.59 per 100 person-years), 15% had at least 1 inappropriate ICD shock—unaffected by the presence of single or dual leads—and 27% required device revision and/or lead replacement. Patients with secondary prevention ICDs had a higher incidence of both ventricular and supraventricular arrhythmias compared to those with primary prevention ICDs. Loss-of-function mutations and prior nonsustained VAs were associated with sustained VAs. Among high-risk patients, those who underwent epicardial ablation experienced significantly fewer ventricular events. The overall mortality rate was 5.88%, with 22.2% of deaths attributed to cardiac causes.</div></div><div><h3>Conclusions</h3><div>This 30-year study highlights ICD therapy’s critical role in preventing fatal arrhythmias in Brugada syndrome, but also reveals frequent device-related complications, especially in younger patients. Thoracoscopic epicardial ablation significantly reduced VA in high-risk patients, offering a promising adjunctive therapy. These findings emphasize the need for individualized treatment strategies to balance the benefits of ICDs with their risks, and underscore the potential of ablation to improve long-term outcomes.</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1174-1188"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143633954","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}
Jeremy William MBBS , Aleksandr Voskoboinik MBBS, PhD
{"title":"Pulsed Field Ablation for Atrial Fibrillation in Hypertrophic Cardiomyopathy","authors":"Jeremy William MBBS , Aleksandr Voskoboinik MBBS, PhD","doi":"10.1016/j.jacep.2025.03.028","DOIUrl":"10.1016/j.jacep.2025.03.028","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1216-1217"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144173811","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}
So-Ryoung Lee MD, PhD , Eue-Keun Choi MD, PhD , Kyung-Yeon Lee MD , JungMin Choi MD , Hyo-Jeong Ahn MD , Soonil Kwon MD , Young-Keun On MD, PhD , Ji Hyun Lee MD, PhD , Youngjin Cho MD, PhD , Il-Young Oh MD, PhD , Hong-Euy Lim MD, PhD , Min-Soo Cho MD, PhD , Gi-Byoung Nam MD, PhD , Gregory Y.H. Lip MD , Seil Oh MD, PhD
{"title":"Reduction of AF Burden After Cryoballoon Ablation in Patients With Early Persistent AF","authors":"So-Ryoung Lee MD, PhD , Eue-Keun Choi MD, PhD , Kyung-Yeon Lee MD , JungMin Choi MD , Hyo-Jeong Ahn MD , Soonil Kwon MD , Young-Keun On MD, PhD , Ji Hyun Lee MD, PhD , Youngjin Cho MD, PhD , Il-Young Oh MD, PhD , Hong-Euy Lim MD, PhD , Min-Soo Cho MD, PhD , Gi-Byoung Nam MD, PhD , Gregory Y.H. Lip MD , Seil Oh MD, PhD","doi":"10.1016/j.jacep.2025.02.006","DOIUrl":"10.1016/j.jacep.2025.02.006","url":null,"abstract":"<div><h3>Background</h3><div>Limited data exist on reducing the atrial fibrillation (AF) burden, the regression from persistent to paroxysmal AF, and symptom improvement after cryoablation in patients with persistent AF.</div></div><div><h3>Objectives</h3><div>This study sought to evaluate the efficacy of cryoablation in reducing the AF burden, regression to paroxysmal AF, and symptom improvement in patients with early persistent AF.</div></div><div><h3>Methods</h3><div>This investigator-initiated, multicenter, and prospective cohort study enrolled the patients with early persistent AF (≤3 years). All patients were inserted an implantable loop recorder at the time of trial entry. AF burden (percentage of time in AF) and actual AF type were evaluated during pre-cryoablation and 12-month follow-up after cryoablation. AF-related symptoms and quality of life were assessed.</div></div><div><h3>Results</h3><div>A total of 130 patients were enrolled (median age 61 years [Q1-Q3: 54-67 years]; median duration of persistent AF 6 months [Q1-Q3: 4-10 months]). The mean AF burden before cryoablation was 77 ± 34% (25.4% of high-burden paroxysmal AF; 74.6% of implantable loop recorder–confirmed persistent AF), which was reduced to 9 ± 21% after 12-month follow-up (mean 68 ± 37% of AF burden reduction; <em>P <</em> 0.001). Of the total, 38.5% had no recurrence of AF, 43.1% had a recurrence of paroxysmal AF, and 18.5% had a recurrence of persistent AF. Among those with confirmed persistent AF at baseline, 76% of patients showed regression to paroxysmal AF or no recurrence. After cryoablation, 77% of patients improved AF-related symptoms and quality of life as assessed by 36-Item Short Form Survey questionnaires.</div></div><div><h3>Conclusions</h3><div>In patients with early persistent AF, cryoablation significantly reduced AF burden and achieved regression of AF type followed by symptom improvement. (Cryoablation for Pulmonary Vein Isolation Alone in Patients with Early Persistent AF Assessed by Continuous Monitoring [COOL-PER]; <span><span>NCT05507749</span><svg><path></path></svg></span>).</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1234-1247"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143719280","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}
Willem Gerrits MD , Philippe C. Wouters MD, PhD , Cheyenne S.L. Chiu MD , Marco Guglielmo MD , Maarten J. Cramer MD, PhD , Pim van der Harst MD, PhD , Kevin Vernooy MD, PhD , Antonius M.W. van Stipdonk MD, PhD , Vokko P. van Halm MD, PhD , Vincent F. van Dijk MD, PhD , Abdul Ghani MD, PhD , Alexander H. Maass MD, PhD , Sing-Cien Yap MD, PhD , Frebus J. van Slochteren PhD , Mathias Meine MD, PhD
{"title":"Optimizing CRT Lead Placement Accuracy With CMR-Guided On-Screen Targeting","authors":"Willem Gerrits MD , Philippe C. Wouters MD, PhD , Cheyenne S.L. Chiu MD , Marco Guglielmo MD , Maarten J. Cramer MD, PhD , Pim van der Harst MD, PhD , Kevin Vernooy MD, PhD , Antonius M.W. van Stipdonk MD, PhD , Vokko P. van Halm MD, PhD , Vincent F. van Dijk MD, PhD , Abdul Ghani MD, PhD , Alexander H. Maass MD, PhD , Sing-Cien Yap MD, PhD , Frebus J. van Slochteren PhD , Mathias Meine MD, PhD","doi":"10.1016/j.jacep.2025.01.022","DOIUrl":"10.1016/j.jacep.2025.01.022","url":null,"abstract":"<div><h3>Background</h3><div>To improve cardiac resynchronization therapy (CRT) an on-screen image-guidance platform, CARTBox-Suite (CART-Tech B.V.), was developed to identify left ventricular pacing electrode (LVPE) implantation sites and facilitate precise LVPE placement. This multicenter randomized trial evaluated the efficacy of image guidance on LVPE implantation accuracy and its impact on left ventricular end-systolic volume (LVESV) reduction 6 months after CRT.</div></div><div><h3>Objectives</h3><div>The aim of this trial is to improve the accuracy and efficacy of LVPE placement in CRT.</div></div><div><h3>Methods</h3><div>A total of 131 heart failure patients (80% with Class I CRT indication) were enrolled across 7 hospitals in the Netherlands. CARTBox-Suite, which utilizes a cloud-based AI algorithm, was used to identify a target area with late mechanical activation based on cardiac magnetic resonance imaging. Scarred areas marked by late gadolinium enhancement were excluded. Patients were randomized to image-guided implantation, with on-screen guidance during the procedure or conventional implantation.</div></div><div><h3>Results</h3><div>The primary endpoint, LVPE implantation in the target area, was achieved significantly more often in the image-guided group (66.7% vs 29.2%; <em>P</em> < 0.001). The secondary endpoint was fewer LVPE placed in scarred areas in the image-guided group (7.1% vs 36.4%; <em>P =</em> 0.006). Mean LVESV reduction was greater in the image-guided group (43.2% vs. 37.6%), although not significantly <em>(P =</em> 0.166). Patients with myocardial scar showed greater LVESV reduction with image guidance (40.7% vs 27.7%; <em>P =</em> 0.028).</div></div><div><h3>Conclusions</h3><div>Image-guided implantation resulted in significantly more LVPE placed in the target area and greater LVESV reduction in patients with myocardial scar.</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 6","pages":"Pages 1293-1305"},"PeriodicalIF":8.0,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143673860","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}