Pub Date : 2026-02-14DOI: 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
{"title":"Flecainide use before and after CAST: A systematic review.","authors":"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","doi":"10.1016/j.hrthm.2026.02.010","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.02.010","url":null,"abstract":"","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146201397","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}
Pub Date : 2026-02-14DOI: 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.
{"title":"A Novel Electrocardiographic Risk Score for Sudden Cardiac Arrest in Patients with Atrial Fibrillation: The SCAAF-ERS.","authors":"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","doi":"10.1016/j.hrthm.2026.02.009","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.02.009","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Objective: </strong>Develop an ECG risk score (ERS) to predict SCA in the population with AF.</p><p><strong>Methods: </strong>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).</p><p><strong>Results: </strong>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).</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146206893","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}
Pub Date : 2026-02-12DOI: 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
{"title":"2026 HRS/EHRA Scientific Statement on Pulsed Field Ablation for Cardiac Arrhythmias.","authors":"Atul Verma, Mélèze Hocini, Jason Andrade, Edward Gerstenfeld, Suraj Kapa, Dhanunjaya Lakkireddy, Damijan Miklavcic, Andrea Natale, Jonathan Piccini, Boris Schmidt, Melanie A Gunawardene","doi":"10.1016/j.hrthm.2026.02.006","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.02.006","url":null,"abstract":"","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146197298","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}
Pub Date : 2026-02-12DOI: 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.
{"title":"Adverse Events During Catheter Ablation in Patients with Cardiac Implantable Electronic Devices.","authors":"Shiloh Sison, Kenneth A Ellenbogen, Charles D Swerdlow","doi":"10.1016/j.hrthm.2026.02.008","DOIUrl":"https://doi.org/10.1016/j.hrthm.2026.02.008","url":null,"abstract":"<p><strong>Background: </strong>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]).</p><p><strong>Objective: </strong>To characterize AEs in contemporary CIED patients undergoing ablation and identify implications for safer practice.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":12886,"journal":{"name":"Heart rhythm","volume":" ","pages":""},"PeriodicalIF":5.7,"publicationDate":"2026-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146197319","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}
Pub Date : 2026-02-10DOI: 10.1016/j.hrthm.2026.01.050
Anindya Ghosh, Chenni S Sriram, Ulhas M Pandurangi
{"title":"Response to the letter to the editor entitled \"Stylet-in-sheath: A new solution to an old problem?\"","authors":"Anindya Ghosh, Chenni S Sriram, Ulhas M Pandurangi","doi":"10.1016/j.hrthm.2026.01.050","DOIUrl":"10.1016/j.hrthm.2026.01.050","url":null,"abstract":"","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":"146179203","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}
Pub Date : 2026-02-10DOI: 10.1016/j.hrthm.2025.12.051
Michael V Orlov, Brian Olshansky, Pugazhendhi Vijayaraman, Zachary I Whinnett
{"title":"Stylet-in-Sheath: A New Solution to An Old Problem?","authors":"Michael V Orlov, Brian Olshansky, Pugazhendhi Vijayaraman, Zachary I Whinnett","doi":"10.1016/j.hrthm.2025.12.051","DOIUrl":"https://doi.org/10.1016/j.hrthm.2025.12.051","url":null,"abstract":"","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":"146179154","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}
Pub Date : 2026-02-10DOI: 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.
{"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}
Pub Date : 2026-02-10DOI: 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.
{"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}
Pub Date : 2026-02-09DOI: 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.
{"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}
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.
{"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}