JACC. Clinical electrophysiology最新文献

{"title":"A Gain-of -Function SLC4A3 Mutation Causes Short-QT Syndrome","authors":"Moshe Giladi MD, PhD ,&nbsp;Odelia Chorin MD ,&nbsp;Silvia Piccirillo PhD ,&nbsp;Elon Prass MD ,&nbsp;Haike Reznik Wolf PhD ,&nbsp;Jana Shamash PhD ,&nbsp;Ariela Haimovich PhD ,&nbsp;Ortal Barel PhD ,&nbsp;Dana Viskin MD ,&nbsp;Shir Frydman MD ,&nbsp;Raphael Rosso MD ,&nbsp;Shmuel Banai MD ,&nbsp;Sami Viskin MD ,&nbsp;Ehud Chorin MD, PhD","doi":"10.1016/j.jacep.2025.03.027","DOIUrl":"10.1016/j.jacep.2025.03.027","url":null,"abstract":"<div><h3>Background</h3><div>Congenital short-QT syndrome (SQTS) is a genetic disorder characterized by short QT interval on electrocardiography (ECG) and a high risk for malignant ventricular tachyarrhythmias.</div></div><div><h3>Objectives</h3><div>The aim of this study was to describe a new variant in the SQTS-associated gene <em>SLC4A3</em> at the molecular and clinical levels.</div></div><div><h3>Methods</h3><div>Using whole-exome sequencing, a novel missense variant in <em>SLC4A3</em> was identified, encoding for the cardiac anion exchanger 3. The mutant was characterized using computational structural modeling and functional transport studies in human embryonic kidney 293 cells. Patients were assessed using resting ECG, 12-lead Holter recordings, and a novel diagnostic test termed here the Ippon test.</div></div><div><h3>Results</h3><div>A novel heterozygous <em>SLC4A3</em> variant (p.R1016G) was detected in a family with 6 cases of sudden cardiac death and a case of documented polymorphic ventricular tachycardia in 5 generations. Functional analyses in human embryonic kidney 293 cells revealed gain of function rather than the loss of function expected on the basis of previously reported SQTS-associated <em>SLC4A3</em> variants. Although affected family members exhibited shorter corrected QT intervals on resting ECG compared with nonaffected members (360 ± 20 ms vs 380 ± 30 ms; <em>P</em> = 0.0068) and 12-lead Holter monitoring (350 ± 20 ms vs 380 ± 30 ms; <em>P</em> = 0.0013), significant overlap existed. The sudden heart rate deceleration provoked by the Ippon test revealed that the QT interval in carriers failed to prolong in response to the sudden bradycardia, resulting in inappropriately short corrected QT intervals, leading to a better distinction of affected from nonaffected patients (340 ± 30 ms vs 370 ± 10 ms, respectively; <em>P</em> = 0.0003).</div></div><div><h3>Conclusions</h3><div><em>SLC4A3</em> p.R1016G is a novel SQTS-associated variant associated with a gain-of-function effect. The Ippon test is a new provocation maneuver that identifies SQTS variant carriers with high diagnostic accuracy.</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1583-1594"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144173808","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":"High-Density Mapping for Ablation of a Manifest Nodofascicular/Ventricular Pathway","authors":"Nawin L. Ramdat Misier BSc ,&nbsp;William A. Scott MD ,&nbsp;Natasja M.S. de Groot MD, PhD ,&nbsp;Hoang H. Nguyen MD","doi":"10.1016/j.jacep.2025.03.036","DOIUrl":"10.1016/j.jacep.2025.03.036","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1674-1682"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144173745","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":"Off-Target hERG Blockade by Ivabradine","authors":"Sodikdjon A. Kodirov PhD","doi":"10.1016/j.jacep.2025.05.032","DOIUrl":"10.1016/j.jacep.2025.05.032","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1634-1635"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144722852","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":"Implantation of Atrial Leadless Pacemaker Via the Internal Jugular Approach","authors":"Dilesh Patel MD ,&nbsp;Daniel Cortez MD ,&nbsp;Gaurang Gandhi MD ,&nbsp;Marshall Winner MD ,&nbsp;Hemal Shah MD ,&nbsp;Muhammad Athar MD ,&nbsp;Ann Canterbury MD ,&nbsp;Taraneh Zamani MD ,&nbsp;Godbless Ajenaghughrure MD ,&nbsp;Emile G. Daoud MD","doi":"10.1016/j.jacep.2025.03.022","DOIUrl":"10.1016/j.jacep.2025.03.022","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1669-1673"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144173776","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":"Calcium Homeostatic Feedback Control Predicts Atrial Fibrillation Initiation, Remodeling, and Progression","authors":"Nicolae Moise MD ,&nbsp;Seth H. Weinberg PhD","doi":"10.1016/j.jacep.2025.03.004","DOIUrl":"10.1016/j.jacep.2025.03.004","url":null,"abstract":"<div><h3>Background</h3><div>Atrial fibrillation (AF) is a progressive disorder, with arrhythmia episodes becoming increasingly longer and ultimately permanent. The chaotic electrical activity by itself is well known to drive progression, a process classically summarized as “AF begets AF.” However, the mechanisms underlying this progression are not yet well defined.</div></div><div><h3>Objectives</h3><div>We hypothesize that calcium homeostatic feedback regulating ion channel expression is a critical mechanistic component of this pathological process.</div></div><div><h3>Methods</h3><div>We propose a modeling framework that tracks both short-term beat-to-beat electrical and calcium activity and long-term tissue substrate remodeling as a single coupled dynamical system. Importantly, the full AF progression from healthy to pathological remodeled tissue is reproduced, in contrast with prior studies that consider “snapshots” of various AF stages.</div></div><div><h3>Results</h3><div>Simulations predict that single cells respond to fast pacing by maintaining intracellular calcium concentrations through dynamic ion channel expression and electrical phenotype changes. In 2-dimensional homogeneous tissue, spontaneous spiral waves stabilize into permanent re-entry. In 2-dimensional heterogeneous tissue, we observe the initiation of re-entrant activity in response to fast pacing, followed by increasingly longer intermittent, and then permanent, arrhythmic activity. Simulations predict critical properties of re-entrant wave locations, leading to a novel hypothesis: spiral wave activity itself drives underlying substrate remodeling and the emergence of remodeled tissue “niches” that support the stabilization of fast re-entrant activity.</div></div><div><h3>Conclusions</h3><div>Thus, the model joins multiple lines of inquiry (ie, long-term calcium regulation, ion channel coexpression and remodeling, and tissue-scale arrhythmia spatiotemporal organization) into a single coherent framework, and for the first time, captures the dynamics of the long-term natural history of AF.</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1415-1435"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144005044","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":"Use of High-Power, Short-Duration Radiofrequency Applications to Target Purkinje Tissue and Ablate Ventricular Fibrillation","authors":"Robert N. Kerley MD ,&nbsp;Uyanga Batnyam MD ,&nbsp;Babak Nazer MD ,&nbsp;Esseim Sharma MD ,&nbsp;Jorge E. Romero MD ,&nbsp;Usha B. Tedrow MD, MS ,&nbsp;William H. Sauer MD","doi":"10.1016/j.jacep.2025.04.014","DOIUrl":"10.1016/j.jacep.2025.04.014","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1622-1624"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144015799","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":"Characterization of the Thermal Properties of Healthy vs Infarcted Ventricular Tissue","authors":"Maite Izquierdo MD, PhD ,&nbsp;Juan J. Pérez PhD ,&nbsp;Omérim Gaona MD ,&nbsp;Josep Navarro MD ,&nbsp;Javier Navarrete MD ,&nbsp;Luis Martinez-Dolz MD, PhD ,&nbsp;Luis Almenar PhD ,&nbsp;Oscar Cano MD, PhD ,&nbsp;Nuria Mancheño MD ,&nbsp;Ana González-Suárez PhD ,&nbsp;Joaquín Osca MD, PhD ,&nbsp;Enrique Berjano PhD","doi":"10.1016/j.jacep.2025.02.032","DOIUrl":"10.1016/j.jacep.2025.02.032","url":null,"abstract":"<div><h3>Background</h3><div>Radiofrequency (RF) ablation is the technique of choice for treating ventricular tachycardias in ischemic cardiomyopathy. However, recent experimental studies have found it to be ineffective. Poor heat transmission in collagen-rich scar tissue was 1 of the explanations given, even though the thermal properties of infarcted tissue have never been studied.</div></div><div><h3>Objectives</h3><div>The purpose of the present study was to measure the thermal properties of chronically infarcted myocardium.</div></div><div><h3>Methods</h3><div>A total of 5 freshly explanted human hearts from patients undergoing heart transplantation with previous chronic myocardial infarction were studied in the operating room. A 2-needle thermal sensor model SH-3 Tempos (Meter Group) was introduced into different infarcted and healthy areas in the left ventricle to measure the thermal conductivity, volumetric heat capacity, and thermal diffusivity. All the measured areas were excised for histological analysis. An in silico model of radiofrequency catheter ablation was then built to evaluate the impact of the thermal parameters obtained. Stiffness, electrical properties, and vascularization were also included to simulate realistic healthy and infarcted myocardium according to previously published data.</div></div><div><h3>Results</h3><div>More than one-half of the area of the infarcted samples was composed of collagen. No significant differences were found between the thermal properties of infarcted and healthy tissue. The RF lesion depths obtained from the computational model did not have any clinically relevant differences (&lt;0.4 mm in depth) between the infarcted and healthy tissue.</div></div><div><h3>Conclusions</h3><div>Thermal properties of infarcted tissue are not sufficiently different from healthy tissue to justify different RF lesion sizes, according to our computational model.</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1465-1474"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144005659","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":"Subcutaneous Implantable Defibrillator Therapy in Patients With Brugada Syndrome","authors":"Federico Migliore MD, PhD ,&nbsp;Luca Ottaviano MD ,&nbsp;Alberto Arestia MD ,&nbsp;Gerardo Nigro MD ,&nbsp;Antonio Dello Russo MD ,&nbsp;Stefano Viani MD ,&nbsp;Valter Bianchi MD ,&nbsp;Antonio Bisignani MD ,&nbsp;Paolo Pieragnoli MD ,&nbsp;Gennaro Vitulano MD ,&nbsp;Roberto Rordorf MD ,&nbsp;Pietro Francia MD ,&nbsp;Erika Taravelli MD ,&nbsp;Ennio Pisanò MD ,&nbsp;Carlo Lavalle MD ,&nbsp;Roberta Brambilla MD ,&nbsp;Matteo Ziacchi MD ,&nbsp;Antonio Rapacciuolo MD ,&nbsp;Miguel Viscusi MD ,&nbsp;Paolo De Filippo MD ,&nbsp;Silvia G. Priori MD, PhD","doi":"10.1016/j.jacep.2025.03.003","DOIUrl":"10.1016/j.jacep.2025.03.003","url":null,"abstract":"<div><h3>Background</h3><div>The implantable cardioverter-defibrillator (ICD) is recognized as the most effective life-saving therapy in patients with Brugada syndrome (BrS). However, transvenous ICD is associated with a notable rate of complications over time. The subcutaneous implantable cardioverter-defibrillator (S-ICD) has emerged as a promising alternative to the transvenous ICD. Nevertheless, long-term data from large cohorts of BrS patients with S-ICDs are lacking.</div></div><div><h3>Objectives</h3><div>This multicenter study aimed to assess the long-term outcomes of S-ICD therapy in patients with BrS.</div></div><div><h3>Methods</h3><div>The study included 450 consecutive BrS patients (mean age 43 ± 12; 86% male) who underwent S-ICD implantation between 2014 and 2024.</div></div><div><h3>Results</h3><div>During a median follow-up of 52 months (25th-75th percentile: 29-72), appropriate shocks were delivered in 3% of patients (1.2%; 95% CI: 0.2-2.2, at 12 months), with a first-shock success rate of 90% (100% with 2 shocks). Inappropriate shocks occurred in 7% of patients (1.4%; 95% CI: 0.3-2.5, at 12 months). Shock zone programmed at 250 beats/min (HR: 0.40; 95% CI: 0.18-0.89; <em>P</em> = 0.025) and more than 1 suitable vector on screening (HR: 0.39; 95% CI: 0.17–0.87; <em>P</em> = 0.023) were independent protective factors against inappropriate shock. Device-related complications were reported in 4% of patients (2.5%; 95% CI: 1.0-3.9 at 12 months). The need for antibradycardia pacing was reported in 3 patients (0.7%). No device explantation because of the need for antitachycardia pacing was noted.</div></div><div><h3>Conclusions</h3><div>Our findings support the S-ICD as a viable alternative to the transvenous ICD for preventing sudden cardiac death in BrS patients without pacing indication (Arrhythmias Detection in a Real World Population [RHYTHM DETECT]; <span><span>NCT02275637</span><svg><path></path></svg></span>)</div></div>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1572-1582"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143742934","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":"Lead Implantation in the Setting of Venous Occlusion","authors":"Thomas A. Boyle MD,&nbsp;David Callans MD,&nbsp;Rajat Deo MD,&nbsp;Fermin Garcia MD,&nbsp;Gustavo Guandalini MD,&nbsp;Matthew C. Hyman MD, PhD,&nbsp;David Lin MD,&nbsp;Saman Nazarian MD, PhD,&nbsp;Michael Riley MD, PhD,&nbsp;Poojita Shivamurthy MD,&nbsp;Gregory Supple MD,&nbsp;Michelle Walsh,&nbsp;Robert D. Schaller DO ,&nbsp;David S. Frankel MD","doi":"10.1016/j.jacep.2025.04.022","DOIUrl":"10.1016/j.jacep.2025.04.022","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages 1616-1618"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144325704","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":"Editorial Board/Officers Page","authors":"","doi":"10.1016/S2405-500X(25)00476-1","DOIUrl":"10.1016/S2405-500X(25)00476-1","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":"11 7","pages":"Pages A1-A5"},"PeriodicalIF":7.7,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144722390","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}