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Flecainide use before and after CAST: A systematic review. 在CAST前后使用氟氯胺:一项系统回顾。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-14 DOI: 10.1016/j.hrthm.2026.02.010
Christopher O Y Li, Samantha Wong, Melanie D Kardel, Sonia Franciosi, Abhay Katyal, Matthew Cheung, Sabrina Wei, Melissa Braschel, Thomas M Roston, Sakethram Saravu Vijayashankar, Shubhayan Sanatani
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引用次数: 0
A Novel Electrocardiographic Risk Score for Sudden Cardiac Arrest in Patients with Atrial Fibrillation: The SCAAF-ERS. 心房颤动患者心脏骤停的一种新的心电图风险评分:scaf - ers。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-14 DOI: 10.1016/j.hrthm.2026.02.009
Thien Tan Tri Tai Truyen, Elizabeth Heckard, Kotoka Nakamura, Audrey Uy-Evanado, Harpriya Chugh, Ali A Sovari, Jacob Tfelt-Hansen, Kyndaron Reinier, Sumeet S Chugh

Background: Atrial fibrillation (AF) increases the risk of sudden cardiac arrest (SCA). Patients with AF are excluded from ECG risk predictor analyses for SCA due to their complex ECG.

Objective: Develop an ECG risk score (ERS) to predict SCA in the population with AF.

Methods: We performed a case-control study using SCA cases from a community-based study in Oregon, USA (population∼1 million; 2002-2020). Subjects aged ≥18 with medical records and pre-arrest ECGs showing AF were included. Controls had AF ECGs without SCA history. For validation, cases and controls were selected from a California study (population∼850,000; 2015-2023). Significant ECG variables were used to develop an ECG-based score for SCA risk prediction (SCAAF-ERS).

Results: In the discovery group (447 cases, 138 controls; mean age 74.9 years; 73.5% male), the SCAAF-ERS (0-5) was developed by assigning one point for each significant factor: prolonged QTc, QRS-T angle >100°, Tpeak-Tend >94ms, LVH, and delayed QRS transition. Adjusting for demographics and comorbidities, SCA risk increased 1.5 times (95%CI: 1.2-1.9) per point increase. An SCAAF-ERS ≥4(16%) resulted in an OR of 12.9 (95%CI: 3.4-48.1). In the validation group (315 cases, 138 controls; mean age 77.7 years; 64.5% male), SCA risk doubled per point increase (OR: 2.4,95%CI: 1.9-3.1). An SCAAF-ERS ≥3 (27%) resulted an OR 22.9 (95%CI: 9.0-58.3).

Conclusion: SCA risk was predicted successfully from AF ECGs, with a novel risk score displaying 13 to 23-fold increased odds. Further validation of the SCAAF-ERS in larger, diverse populations is warranted.

背景:心房颤动(AF)增加心脏骤停(SCA)的风险。房颤患者由于其复杂的ECG,被排除在SCA的ECG风险预测分析之外。目的:建立心电图风险评分(ERS)来预测af人群中的SCA。方法:我们对来自美国俄勒冈州社区研究的SCA病例进行了病例对照研究(人口约100万;2002-2020)。受试者年龄≥18岁,有医疗记录,心跳骤停前心电图显示房颤。对照组有无SCA病史的AF心电图。为了验证,从加利福尼亚的一项研究中选择病例和对照(人群~ 850,000;2015-2023)。使用重要的心电图变量来建立基于心电图的SCA风险预测评分(scaf - ers)。结果:发现组(447例,对照组138例,平均年龄74.9岁,男性73.5%),对QTc延长、QRS- t角度>100°、t峰值-倾向>94ms、LVH、QRS转换延迟等重要因素分别给予1分,形成SCAAF-ERS(0-5)评分。调整人口统计学和合并症,每增加一个点,SCA风险增加1.5倍(95%CI: 1.2-1.9)。如果scaf - ers≥4(16%),则OR为12.9 (95%CI: 3.4-48.1)。在验证组(315例,138例对照,平均年龄77.7岁,64.5%为男性),每增加一个点,SCA风险增加一倍(OR: 2.4,95%CI: 1.9-3.1)。如果scaf - ers≥3(27%),则OR为22.9 (95%CI: 9.0-58.3)。结论:房颤心电图成功预测了SCA风险,新的风险评分显示增加了13至23倍的风险。在更大、更多样化的人群中进一步验证scaf - ers是有必要的。
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引用次数: 0
2026 HRS/EHRA Scientific Statement on Pulsed Field Ablation for Cardiac Arrhythmias. 2026 HRS/EHRA关于心律失常脉冲场消融的科学声明。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-12 DOI: 10.1016/j.hrthm.2026.02.006
Atul Verma, Mélèze Hocini, Jason Andrade, Edward Gerstenfeld, Suraj Kapa, Dhanunjaya Lakkireddy, Damijan Miklavcic, Andrea Natale, Jonathan Piccini, Boris Schmidt, Melanie A Gunawardene
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引用次数: 0
Adverse Events During Catheter Ablation in Patients with Cardiac Implantable Electronic Devices. 心脏植入式电子装置患者导管消融过程中的不良事件。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-12 DOI: 10.1016/j.hrthm.2026.02.008
Shiloh Sison, Kenneth A Ellenbogen, Charles D Swerdlow

Background: Reviews of adverse events (AEs) in patients with cardiac implantable electronic devices (CIEDs) during catheter ablation predate newer devices, procedures, and ablation technologies (e.g., pulsed field [PF], lattice-tip radiofrequency [RF]).

Objective: To characterize AEs in contemporary CIED patients undergoing ablation and identify implications for safer practice.

Methods: We analyzed 433 AEs from the FDA's Manufacturer and User Facility Device Experience (MAUDE) database and peer-reviewed literature (2020-2025), focusing on newer technologies.

Results: Ablation catheters/energy caused 97% of AEs (RF: 89%; PF: 8%). Mechanical interactions caused 32% of AEs (30% dislodgements). Electromagnetic interactions (68%) included myocardial thermal injury (34%), generator dysfunction (20%), oversensing (9%), and induction of ventricular fibrillation (VF) (5%). Interventions (generator/lead replacement/repositioning) occurred in 56% of cases. Thermal injury with loss of capture necessitated replacement/repositioning of 53 transvenous leads and 32 leadless pacemakers, often when ablation sites were ≥2 cm from tip electrodes. Lattice-tip RF near Biotronik ICD leads induced 14 of 15 RF-related VFs. PF accounted for all 6 confirmed generator failures. No cryoablation AEs were reported.

Conclusion: This first and largest comprehensive series of ablation-related complications in contemporary CIED patients underscores underappreciated risks of older technology and highlights novel risks of evolving technologies. A notable finding is that most threshold elevations requiring intervention occurred at ablation sites remote from CIED tip electrodes. More than 40% of AEs appeared largely preventable, suggesting gaps in procedural workflows. Our results suggest opportunities to improve clinical practices and CIED design to safeguard patients.

背景:对心脏植入式电子设备(cied)患者在导管消融期间不良事件(ae)的回顾早于较新的设备、程序和消融技术(如脉冲场[PF]、点阵尖端射频[RF])。目的:探讨当代CIED患者行消融术时ae的特征,并确定其安全性。方法:我们分析了FDA制造商和用户设施设备体验(MAUDE)数据库和同行评议文献(2020-2025)中的433个ae,重点分析了较新的技术。结果:消融导管/能量导致97%的ae (RF: 89%; PF: 8%)。机械相互作用导致32%的ae(30%的移位)。电磁相互作用(68%)包括心肌热损伤(34%)、发电机功能障碍(20%)、过度敏感(9%)和诱发心室颤动(VF)(5%)。干预措施(发生器/导联置换/重新定位)发生在56%的病例中。丢失捕获的热损伤需要更换/重新定位53个经静脉导线和32个无导线起搏器,通常当消融部位距离尖端电极≥2cm时。Biotronik ICD导联附近的栅格尖端射频诱发15例射频相关VFs中的14例。PF是所有6个已确认的发电机故障的原因。未见冷冻消融ae的报道。结论:这是当代CIED患者中第一个也是最大的综合性消融相关并发症系列,强调了旧技术的未被充分认识的风险,并突出了新技术的发展风险。一个值得注意的发现是,大多数需要干预的阈值升高发生在远离CIED尖端电极的消融部位。40%以上的事件似乎基本上是可以预防的,这表明程序性工作流程存在差距。我们的结果提示了改善临床实践和CIED设计以保护患者的机会。
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引用次数: 0
Response to the letter to the editor entitled "Stylet-in-sheath: A new solution to an old problem?" 作者回复《时尚:老问题的新解决方案?》
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-10 DOI: 10.1016/j.hrthm.2026.01.050
Anindya Ghosh, Chenni S Sriram, Ulhas M Pandurangi
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引用次数: 0
Stylet-in-Sheath: A New Solution to An Old Problem? 时尚护套:老问题的新解决方案?
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-10 DOI: 10.1016/j.hrthm.2025.12.051
Michael V Orlov, Brian Olshansky, Pugazhendhi Vijayaraman, Zachary I Whinnett
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引用次数: 0
HRS/ISACHD/PACES Scientific Statement on Provider and Institutional Best Practices for Comprehensive Adult Congenital Heart Disease Electrophysiology Programs. HRS/ISACHD/PACES关于成人先天性心脏病电生理综合项目提供者和机构最佳实践的科学声明。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-10 DOI: 10.1016/j.hrthm.2026.02.002
Paul Khairy, Susan P Etheridge, John Jairo Araujo, Konstantinos N Aronis, Vanessa M Kalis, Anna N Kamp, Michael S Lloyd, Malini Madhavan, Elizabeth D Sherwin, John K Triedman, Jeremy P Moore

The global population of adults with congenital heart disease now exceeds that of children, reflecting decades of surgical and medical advances. As this cohort has aged, arrhythmias have emerged as a predominant cause of morbidity, hospitalization, and late mortality. Arrhythmia mechanisms in adult congenital heart disease (ACHD) arise from congenital abnormalities, surgically altered substrates, progressive hemodynamic lesions, multisystem comorbidities, and acquired cardiovascular risk factors, creating a distinct electrophysiologic phenotype that diverges fundamentally from that of populations without congenital heart disease. Despite increasing complexity and demand, ACHD electrophysiology care remains fragmented, with marked variability in provider training, institutional resources, and access to specialized expertise. This joint Heart Rhythm Society (HRS)/Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) statement defines provider- and center-level best practices for comprehensive ACHD electrophysiology programs. It establishes ACHD electrophysiology as a distinct subspecialty and proposes a competency-based framework spanning five domains: congenital cardiac anatomy and physiology, arrhythmia mechanisms and risk stratification, non-invasive electrophysiology assessment, procedural proficiency in ablation and device therapy, and longitudinal ACHD arrhythmia care. The statement outlines key elements of pre-procedural evaluation, procedural planning, and peri- and post-procedural management and specifies institutional requirements for personnel, facilities, and infrastructure. Recognizing the paucity of validated ACHD-specific performance metrics, the document proposes candidate indicators of procedural efficacy, safety, efficiency, longitudinal outcomes, and patient-reported health status and articulates principles for credentialing and institutional accountability. These recommendations are intended as scalable standards to support regional centers of excellence and harmonized training pathways, thereby promoting safe, effective, and equitable ACHD arrhythmia care.

全球患有先天性心脏病的成人人数现已超过儿童人数,这反映出几十年来外科和医学的进步。随着年龄的增长,心律失常已成为发病、住院和晚期死亡的主要原因。成人先天性心脏病(ACHD)的心律失常机制源于先天性异常、手术改变的底物、进行性血流动力学病变、多系统合并症和获得性心血管危险因素,形成了与无先天性心脏病人群截然不同的电生理表型。尽管复杂性和需求不断增加,但ACHD电生理护理仍然是碎片化的,在提供者培训、机构资源和获得专业知识方面存在明显差异。这份联合心律学会(HRS)/儿科和先天性电生理学会(pace)/国际成人先天性心脏病学会(ISACHD)的声明定义了综合ACHD电生理项目的提供者和中心层面的最佳实践。它将ACHD电生理学建立为一个独特的亚专科,并提出了一个基于能力的框架,涵盖五个领域:先天性心脏解剖和生理学,心律失常机制和风险分层,无创电生理学评估,消融和器械治疗的程序熟练程度,以及ACHD心律失常的纵向护理。该声明概述了程序前评估、程序规划以及程序前后管理的关键要素,并详细说明了对人员、设施和基础设施的机构要求。认识到缺乏经过验证的achd特定绩效指标,该文件提出了程序有效性、安全性、效率、纵向结果和患者报告的健康状况的候选指标,并阐明了认证和机构问责制的原则。这些建议旨在作为可扩展的标准,以支持区域卓越中心和协调培训途径,从而促进安全、有效和公平的ACHD心律失常护理。
{"title":"HRS/ISACHD/PACES Scientific Statement on Provider and Institutional Best Practices for Comprehensive Adult Congenital Heart Disease Electrophysiology Programs.","authors":"Paul Khairy, Susan P Etheridge, John Jairo Araujo, Konstantinos N Aronis, Vanessa M Kalis, Anna N Kamp, Michael S Lloyd, Malini Madhavan, Elizabeth D Sherwin, John K Triedman, Jeremy P Moore","doi":"10.1016/j.hrthm.2026.02.002","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.02.002","url":null,"abstract":"<p><p>The global population of adults with congenital heart disease now exceeds that of children, reflecting decades of surgical and medical advances. As this cohort has aged, arrhythmias have emerged as a predominant cause of morbidity, hospitalization, and late mortality. Arrhythmia mechanisms in adult congenital heart disease (ACHD) arise from congenital abnormalities, surgically altered substrates, progressive hemodynamic lesions, multisystem comorbidities, and acquired cardiovascular risk factors, creating a distinct electrophysiologic phenotype that diverges fundamentally from that of populations without congenital heart disease. Despite increasing complexity and demand, ACHD electrophysiology care remains fragmented, with marked variability in provider training, institutional resources, and access to specialized expertise. This joint Heart Rhythm Society (HRS)/Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) statement defines provider- and center-level best practices for comprehensive ACHD electrophysiology programs. It establishes ACHD electrophysiology as a distinct subspecialty and proposes a competency-based framework spanning five domains: congenital cardiac anatomy and physiology, arrhythmia mechanisms and risk stratification, non-invasive electrophysiology assessment, procedural proficiency in ablation and device therapy, and longitudinal ACHD arrhythmia care. The statement outlines key elements of pre-procedural evaluation, procedural planning, and peri- and post-procedural management and specifies institutional requirements for personnel, facilities, and infrastructure. Recognizing the paucity of validated ACHD-specific performance metrics, the document proposes candidate indicators of procedural efficacy, safety, efficiency, longitudinal outcomes, and patient-reported health status and articulates principles for credentialing and institutional accountability. These recommendations are intended as scalable standards to support regional centers of excellence and harmonized training pathways, thereby promoting safe, effective, and equitable ACHD arrhythmia care.</p>","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146179222","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Arrhythmia Substrates Accessible from the Aortic Root and Immediate Sub-aortic Areas: Mapping and Ablation. 心律失常底物可从主动脉根部和直接主动脉下区域:定位和消融。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-10 DOI: 10.1016/j.hrthm.2026.01.055
Roy M John, Zaniar Ghazizadeh, Scott R Ceresnak

The aortic root overlies the left ventricular ostium and the interatrial septum. Muscle sleeves supporting the aortic sinuses and extensions to the inter-leaflet triangle are sources for outflow tract arrhythmias. The sinuses of Valsalva, in addition, provide access to the inter-atrial septum and left ventricular infra-aortic regions where substrates for arrhythmias reside in both idiopathic arrhythmias and structural heart disease. This review summarizes the anatomical relationship of the aortic root to the various arrhythmic substrates and discusses approaches to ablation.

主动脉根部覆盖在左心室口和房间隔上。支撑主动脉窦的肌套和向叶间三角延伸的肌套是流出道心律失常的来源。此外,Valsalva的鼻窦可进入房间隔和左室主动脉下区域,在这些区域,心律失常的底物存在于特发性心律失常和结构性心脏病中。本文综述了主动脉根部与各种心律失常基底的解剖关系,并讨论了消融的方法。
{"title":"Arrhythmia Substrates Accessible from the Aortic Root and Immediate Sub-aortic Areas: Mapping and Ablation.","authors":"Roy M John, Zaniar Ghazizadeh, Scott R Ceresnak","doi":"10.1016/j.hrthm.2026.01.055","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.01.055","url":null,"abstract":"<p><p>The aortic root overlies the left ventricular ostium and the interatrial septum. Muscle sleeves supporting the aortic sinuses and extensions to the inter-leaflet triangle are sources for outflow tract arrhythmias. The sinuses of Valsalva, in addition, provide access to the inter-atrial septum and left ventricular infra-aortic regions where substrates for arrhythmias reside in both idiopathic arrhythmias and structural heart disease. This review summarizes the anatomical relationship of the aortic root to the various arrhythmic substrates and discusses approaches to ablation.</p>","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146179137","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Hypertrophic cardiomyopathy caused by Filamin-C (FLNC) variants has restrictive and extracardiac features and a distinctive ECG. 由纤维蛋白c (FLNC)变异引起的肥厚性心肌病具有限制性和心外特征,并具有独特的ECG。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-09 DOI: 10.1016/j.hrthm.2026.01.054
Carin de Villiers, Elizabeth Ormondroyd, Kate Thomson, Julian O M Ormerod, Rizwan Sarwar, Adam Waring, Richard D Bagnall, Alexander Sparrow, Violetta Steeples, Edward Blair, Rachel J Buchan, Alfonso Bueno-Orovio, Timothy Dent, Martin Farrall, Andrew R Harper, Robert Hastings, Samuel Jones, Neesha Krishnan, Stefano Lise, Alistair T Pagnamenta, Silvia Salatino, Lydia Seed, Jenny C Taylor, Robert G Weintraub, Dominique West, James S Ware, Jodie Ingles, Christopher Semsarian, Hugh Watkins

Background: Filamin-C (FLNC) gene variants are associated with cardiac and skeletal muscle diseases including a clear role of loss-of-function variants in dilated cardiomyopathy.

Objective: To assess the contribution of rare FLNC variants to hypertrophic/restrictive cardiomyopathy (HCM/RCM).

Methods: Family-based studies in two specialist services, and statistical modelling of rare FLNC missense variants, using a cohort of 3,289 sarcomere-negative HCM cases and 122,348 genome aggregation database controls.

Results: Clinical evaluation of patients with HCM/RCM and a rare FLNC variant identified a distinct ECG repolarisation phenotype in 37% (19/51 individuals, from 12 families) which was observed in only 1.0% (2/197) of a control HCM cohort. FLNC variant carriers with the characteristic ECG had smaller LV cavity size, lower contractility, more severe diastolic dysfunction, and were more likely to have a restrictive phenotype. Heart failure death, transplant or cardiac arrest occurred in at least one individual in seven of the 12 families (58%) in the 'ECG positive' group, and musculoskeletal abnormalities were present in four families (33%). Five of 12 variants (41.7%) in the 'ECG positive' group co-segregated, and two were apparently de novo. Eleven variants were missense, one splice site. Rare FLNC missense variant burden indicated a low case excess amongst all HCM cases (etiological fraction 0.45, 95% CI [0.36-0.54]), but in 'ECG positive' cases the etiological fraction was substantially higher (0.98, 95% CI [0.97-0.99]).

Conclusion: Pathogenic FLNC variants in patients with HCM/RCM are non-truncating and cause a discrete phenotype comprising a characteristic ECG, hypertrophic and restrictive features without hypercontractility, and extra-cardiac abnormalities.

背景:纤维蛋白c (FLNC)基因变异与心脏和骨骼肌疾病相关,包括在扩张型心肌病中功能丧失变异的明确作用。目的:评估罕见FLNC变异对肥厚/限制性心肌病(HCM/RCM)的影响。方法:在两家专业机构进行基于家庭的研究,并对罕见的FLNC错义变异进行统计建模,其中包括3289例肌瘤阴性HCM病例和122348例基因组聚集数据库对照。结果:HCM/RCM和罕见FLNC变异患者的临床评估发现,37%(19/51人,来自12个家族)的患者存在明显的ECG复极表型,而在对照HCM队列中,这一现象仅在1.0%(2/197)的患者中出现。具有特征性ECG的FLNC变异携带者左室腔尺寸较小,收缩力较低,舒张功能障碍更严重,更容易出现限制性表型。在心电图阳性组的12个家庭中,有7个家庭(58%)至少有1人出现心力衰竭死亡、移植或心脏骤停,有4个家庭(33%)出现肌肉骨骼异常。在“ECG阳性”组中,12个变异中有5个(41.7%)共分离,2个明显是新生的。11个变异是错义的,1个剪接位点。罕见的FLNC错义变异负担在所有HCM病例中显示出较低的病例过量(病因分数0.45,95% CI[0.36-0.54]),但在“ECG阳性”病例中,病因分数明显较高(0.98,95% CI[0.97-0.99])。结论:HCM/RCM患者的致病性FLNC变异是非截断性的,并导致离散表型,包括特征性ECG,肥厚和限制性特征,无过度收缩,心脏外异常。
{"title":"Hypertrophic cardiomyopathy caused by Filamin-C (FLNC) variants has restrictive and extracardiac features and a distinctive ECG.","authors":"Carin de Villiers, Elizabeth Ormondroyd, Kate Thomson, Julian O M Ormerod, Rizwan Sarwar, Adam Waring, Richard D Bagnall, Alexander Sparrow, Violetta Steeples, Edward Blair, Rachel J Buchan, Alfonso Bueno-Orovio, Timothy Dent, Martin Farrall, Andrew R Harper, Robert Hastings, Samuel Jones, Neesha Krishnan, Stefano Lise, Alistair T Pagnamenta, Silvia Salatino, Lydia Seed, Jenny C Taylor, Robert G Weintraub, Dominique West, James S Ware, Jodie Ingles, Christopher Semsarian, Hugh Watkins","doi":"10.1016/j.hrthm.2026.01.054","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.01.054","url":null,"abstract":"<p><strong>Background: </strong>Filamin-C (FLNC) gene variants are associated with cardiac and skeletal muscle diseases including a clear role of loss-of-function variants in dilated cardiomyopathy.</p><p><strong>Objective: </strong>To assess the contribution of rare FLNC variants to hypertrophic/restrictive cardiomyopathy (HCM/RCM).</p><p><strong>Methods: </strong>Family-based studies in two specialist services, and statistical modelling of rare FLNC missense variants, using a cohort of 3,289 sarcomere-negative HCM cases and 122,348 genome aggregation database controls.</p><p><strong>Results: </strong>Clinical evaluation of patients with HCM/RCM and a rare FLNC variant identified a distinct ECG repolarisation phenotype in 37% (19/51 individuals, from 12 families) which was observed in only 1.0% (2/197) of a control HCM cohort. FLNC variant carriers with the characteristic ECG had smaller LV cavity size, lower contractility, more severe diastolic dysfunction, and were more likely to have a restrictive phenotype. Heart failure death, transplant or cardiac arrest occurred in at least one individual in seven of the 12 families (58%) in the 'ECG positive' group, and musculoskeletal abnormalities were present in four families (33%). Five of 12 variants (41.7%) in the 'ECG positive' group co-segregated, and two were apparently de novo. Eleven variants were missense, one splice site. Rare FLNC missense variant burden indicated a low case excess amongst all HCM cases (etiological fraction 0.45, 95% CI [0.36-0.54]), but in 'ECG positive' cases the etiological fraction was substantially higher (0.98, 95% CI [0.97-0.99]).</p><p><strong>Conclusion: </strong>Pathogenic FLNC variants in patients with HCM/RCM are non-truncating and cause a discrete phenotype comprising a characteristic ECG, hypertrophic and restrictive features without hypercontractility, and extra-cardiac abnormalities.</p>","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146165397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of atrial fibrillation ablation on biatrial strain: Differences between left and right atrial function. 心房颤动消融对双房应变的影响:左右心房功能的差异。
IF 5.7 2区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS Pub Date : 2026-02-09 DOI: 10.1016/j.hrthm.2026.01.051
Shinichi Tachibana, Osamu Inaba, Yukihiro Inamura, Takamitsu Takagi, Shin Meguro, Kentaro Nakata, Yuhei Isonaga, Hiroaki Ohya, Yutaka Matsumura, Shinsuke Miyazaki, Tetsuo Sasano

Background: Catheter ablation for atrial fibrillation (AF) can improve atrial function; however, little is known about chamber-specific remodeling between the left (LA) and right atria (RA).

Objective: We investigated the differences in biatrial strain and its predictive value for atrial arrhythmia (AF/atrial tachycardia [AT]) recurrence.

Methods: We retrospectively analyzed patients undergoing initial AF ablation who underwent pre- and postprocedural echocardiography. Atrial strain was quantified by 2-dimensional speckle-tracking echocardiography.

Results: 81 patients were analyzed. Both the mean LA (LA reservoir strain [LASr]: 15.3% ± 10.0% to 19.9% ± 8.2%) and RA (RA reservoir strain [RASr]: 18.3% ± 10.8% to 27.3% ± 9.3%) improved significantly after AF ablation (P < .001 for both), with a greater improvement in RASr than LASr (ΔRASr 9.0% ± 10.7% vs ΔLASr 4.5% ± 8.4%; P < .001). Moreover, although LA conduit strain did not change significantly (-9.3% ± 5.6% to -10.3% ± 4.4%; P = .071), RA conduit strain improved significantly (-10.3% ± 6.3% to -14.1% ± 6.2%; P < .001). Postprocedural LASr (hazard ratio 0.92; 95% confidence interval 0.85-1.0; P = .049) and RASr (hazard ratio 0.94; 95% confidence interval 0.89-0.99; P = .016) were independent predictors of AF/AT recurrence. Receiver operating characteristic analysis yielded similar discriminative ability for LASr (area under the curve 0.75; cutoff value; 20.0%) and RASr (area under the curve 0.69; cutoff value 27.0%) (P = .367).

Conclusion: AF ablation led to significant biatrial functional recovery, with RA improvement exceeding that of LA. Both postprocedural LASr and RASr independently predicted AF/AT recurrence, supporting chamber-specific atrial strain as a sensitive marker of reverse remodeling and procedural outcome.

背景:导管消融治疗心房颤动(AF)可改善心房功能;然而,关于左心房(左心房)和右心房(右心房)之间的室特异性重构知之甚少。目的:探讨双心房应变的差异及其对心房速性心律失常(AF/AT)复发的预测价值。方法:回顾性分析首次房颤消融术前和术后超声心动图检查的患者。采用二维斑点跟踪超声心动图定量心房应变。结果:共分析81例患者。房颤消融后平均LA (LASr: 15.3±10.0 ~ 19.9±8.2%)和RA (RASr: 18.3±10.8 ~ 27.3±9.3%)的库区负荷均有显著改善(p结论:房颤消融后双房功能恢复明显,RA的改善优于LA。术后LASr和RASr均可独立预测AF/AT复发,支持室特异性心房应变作为逆转重构和手术结果的敏感标志物。
{"title":"Impact of atrial fibrillation ablation on biatrial strain: Differences between left and right atrial function.","authors":"Shinichi Tachibana, Osamu Inaba, Yukihiro Inamura, Takamitsu Takagi, Shin Meguro, Kentaro Nakata, Yuhei Isonaga, Hiroaki Ohya, Yutaka Matsumura, Shinsuke Miyazaki, Tetsuo Sasano","doi":"10.1016/j.hrthm.2026.01.051","DOIUrl":"10.1016/j.hrthm.2026.01.051","url":null,"abstract":"<p><strong>Background: </strong>Catheter ablation for atrial fibrillation (AF) can improve atrial function; however, little is known about chamber-specific remodeling between the left (LA) and right atria (RA).</p><p><strong>Objective: </strong>We investigated the differences in biatrial strain and its predictive value for atrial arrhythmia (AF/atrial tachycardia [AT]) recurrence.</p><p><strong>Methods: </strong>We retrospectively analyzed patients undergoing initial AF ablation who underwent pre- and postprocedural echocardiography. Atrial strain was quantified by 2-dimensional speckle-tracking echocardiography.</p><p><strong>Results: </strong>81 patients were analyzed. Both the mean LA (LA reservoir strain [LASr]: 15.3% ± 10.0% to 19.9% ± 8.2%) and RA (RA reservoir strain [RASr]: 18.3% ± 10.8% to 27.3% ± 9.3%) improved significantly after AF ablation (P < .001 for both), with a greater improvement in RASr than LASr (ΔRASr 9.0% ± 10.7% vs ΔLASr 4.5% ± 8.4%; P < .001). Moreover, although LA conduit strain did not change significantly (-9.3% ± 5.6% to -10.3% ± 4.4%; P = .071), RA conduit strain improved significantly (-10.3% ± 6.3% to -14.1% ± 6.2%; P < .001). Postprocedural LASr (hazard ratio 0.92; 95% confidence interval 0.85-1.0; P = .049) and RASr (hazard ratio 0.94; 95% confidence interval 0.89-0.99; P = .016) were independent predictors of AF/AT recurrence. Receiver operating characteristic analysis yielded similar discriminative ability for LASr (area under the curve 0.75; cutoff value; 20.0%) and RASr (area under the curve 0.69; cutoff value 27.0%) (P = .367).</p><p><strong>Conclusion: </strong>AF ablation led to significant biatrial functional recovery, with RA improvement exceeding that of LA. Both postprocedural LASr and RASr independently predicted AF/AT recurrence, supporting chamber-specific atrial strain as a sensitive marker of reverse remodeling and procedural outcome.</p>","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146165393","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Heart rhythm
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