Circulation. Arrhythmia and electrophysiology最新文献

{"title":"Modified Unipolar Return Pulsed Field Ablation in Ventricular Myocardium.","authors":"Maria Terricabras, Peter Lombergar, Terenz Escartin, Bor Kos, Philippa Krahn, Jennifer Barry, Graham Wright, Tomaž Jarm, Jernej Štublar, Matej Kranjc, Nicolas Coulombe, Lars Mattison, Daniel C Sigg, Damijan Miklavčič, Atul Verma","doi":"10.1161/CIRCEP.125.014006","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.014006","url":null,"abstract":"<p><strong>Background: </strong>Various pulsed field ablation (PFA) parameters have been proposed to improve lesion depth. This study evaluated a modified unipolar return PFA system to create deep lesions in healthy and infarcted ventricular myocardia.</p><p><strong>Methods: </strong>Numerical modeling was used to compare a modified unipolar return PFA system configuration with a conventional unipolar return (skin patch). We then performed ablation in 14 swine (5 with chronic myocardial infarction and 9 healthy). PFA lesions were created in the left ventricle using a focal catheter (4-mm tip) with a return electrode positioned in the inferior vena cava (biphasic, microsecond pulses of 1300 and 1500 V, 1-16 trains). Electroanatomical mapping guided ablation and lesion localization on magnetic resonance imaging were performed 48 hours post-ablation in the infarcted group and at 1 day, 7 days, and 6 weeks post-ablation in the healthy group.</p><p><strong>Results: </strong>Numerical modeling demonstrated that the modified unipolar return PFA system produced deeper lesions with reduced variability compared with the skin patch. In healthy pigs (n=35 lesions), depths of 6.8±1.8 mm and widths of 11.5±4.7 mm were achieved with 8 pulse trains. Depths of 8.2±2.8 mm and widths of 14.0±4.7 mm were achieved with 16 trains. The maximum lesion depths were 8.8 and 11.6 mm for 8 and 16 trains, respectively. In the infarcted cohort (n=22 lesions), all lesions applied to scar tissue penetrated through fibrotic regions, with epicardial involvement observed in 57% of lesions.</p><p><strong>Conclusions: </strong>The modified unipolar return PFA system effectively creates large lesions and can achieve transmurality in healthy and infarcted animals. Compared with conventional unipolar, it may offer greater lesion depth, width, and consistency.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e014006"},"PeriodicalIF":9.8,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145198363","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":"Dual Epicardial and Endocardial Procedure (DEEP) for Persistent or Longstanding Persistent Atrial Fibrillation.","authors":"Kenneth A Ellenbogen, Ali Khoynezhad, Mark La Meir, Carlo de Asmundis, Jayanthi Koneru, John Johnkoski, Kevin Rist, Mubashir Mumtaz, Michael G Link, Joris R de Groot, Antoine H G Driessen, Mark Y Lee, Steven J Hoff, David Bello, Gansevoort Dunnington, Susan Eisenberg, Margot Vloka, Benedict J Taylor, Stephen D Jones, Jonathan M Philpott, Thomas M Beaver, William M Miles, Junaid H Khan, Steven Kang, Gaurang D Gandhi, Eric J Okum, Nitish Badhwar, Tina Baykaner, Anson M Lee, Paul A Vesco, J Michael Smith, Sydney Gaynor, Ken Frazier, Randall Lee, Vigneshwar Kasirajan","doi":"10.1161/CIRCEP.125.013692","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.013692","url":null,"abstract":"<p><strong>Background: </strong>Despite advances in endocardial catheter ablation (ECA) for persistent atrial fibrillation (PersAF), undertreatment persists, especially in ECA nonresponders and in longstanding PersAF (LSPersAF), with disappointing ablation results. These patients need effective clinical treatment options.</p><p><strong>Methods: </strong>The DEEP (Dual Epicardial and Endocardial Procedure) was a prospective, multicenter, single-arm, investigational device exemption trial to establish the safety and effectiveness of a combined epicardial/endocardial ablation procedure with left atrial appendage exclusion for PersAF/LSPersAF. Eligibility included age 18 to 75 years; symptomatic PersAF/LSPersAF refractory to ≥1 Class I/III antiarrhythmic drug; and ≤2 previous failed ECAs. Two-stage hybrid ablation included ECA performed at 91 to 121 days after the epicardial first stage (including left atrial appendage exclusion), followed by a 90-day blanking and 90-day antiarrhythmic drug optimization period. Primary effectiveness was defined as freedom from documented atrial fibrillation/atrial flutter/atrial tachycardia episodes >30 seconds through the 12-month follow-up, absent Class I/III antiarrhythmic drugs, except previously failed antiarrhythmic drugs at doses not exceeding those previously failed. Primary safety was defined as a composite of device/procedure-related serious adverse events within 30 days of epicardial ablation and 7 days of ECA.</p><p><strong>Results: </strong>Ninety patients enrolled from February 2015 to December 2020; 83.3% (75/90) were male and mean±SD age was 63.4±7.7 years. AF classification was 83.3% (75/90) PersAF/16.7% (15/90) LSPersAF, and 47.8% (43/90) had prior ECA. The composite serious adverse events rate was 6.7% (6/90 [95% CI, 2.5%-13.9%]; <i>P</i><0.001 versus safety goal), including 3 patients experiencing serious adverse events within 30 days of the epicardial procedure and 3 patients within 7 days of the endocardial procedure, all of whom were anticoagulated at the time of the event. Primary effectiveness through 12 months was 71.8% (61/85 [95% CI, 62.2%-81.3%]; <i>P</i>=0.0134 versus performance goal) and was 62.4% (53/85 [95% CI, 52.1%-72.7%]) through 2 years.</p><p><strong>Conclusions: </strong>A collaborative hybrid ablation approach to treating PersAF/LSPersAF is safe and effective.</p><p><strong>Registration: </strong>URL: https://www.clinicaltrials.gov; Unique identifier: NCT02393885.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013692"},"PeriodicalIF":9.8,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145191243","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":"Rapid Electrocardiographic Protocol for Identifying Ventricular Arrhythmias From Specific Sites of the Right Coronary Cusp.","authors":"Cheng Zheng, Wu-Min Hu, Wei-Qian Lin, Yi-Fan Lin, Jia-Meng Shao, Bing Shen, Rong-Hua Liu, Xiao Lu, Guo-Juan Xu, Lucia D'Angelo, Shea Michaela James, Jia-Feng Lin","doi":"10.1161/CIRCEP.124.012936","DOIUrl":"https://doi.org/10.1161/CIRCEP.124.012936","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012936"},"PeriodicalIF":9.8,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145191345","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":"Inhibition of Satellite Glial Cell Activation in Stellate Ganglia Prevents Ventricular Arrhythmogenesis and Remodeling After Myocardial Infarction.","authors":"Zhen Zhou, Hanyu Zhang, Hongbo Xiong, Ke-Qiong Deng, Meng Zheng, Yongkang Zhang, Zhendong Xu, Ruifeng Tian, Tong Zhang, Xiangjie Kong, Yingying Hu, Yinhua Luo, Huanhuan Cai, Di Fan, Qing K Wang, Bo He, Qiongxin Wang, Zhibing Lu","doi":"10.1161/CIRCEP.125.013866","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.013866","url":null,"abstract":"<p><strong>Background: </strong>Hyperactivity of sympathetic neurons in the stellate ganglia (SG) contributes to ventricular arrhythmias and remodeling postmyocardial infarction (MI). However, the role of satellite glial cells (SGCs) surrounding the neurons in this process remains unknown.</p><p><strong>Methods: </strong>SGC-specific chemogenetic manipulation was locally applied to modulate SG-SGC activity dual-directionally in the rats with naïve or infarcted hearts. Subsequently, cardiac sympathetic neural activity and ventricular electrophysiological stability in response to stimulation were evaluated, as well as cardiac neural and structural remodeling post-MI. SG bulk RNA sequencing and the interaction between SGCs and sympathetic neurons isolated from SG were used to explore the underpinning mechanisms.</p><p><strong>Results: </strong>SG-SGC excitation increased SG neural activity and ventricular electrophysiological instability in rats with naïve hearts, whereas its inhibition influenced none of the above under physiological conditions. Of note, 2-hour-MI provoked SG-SGC activation that positively correlated with cardiac sympathetic neurotransmitter (norepinephrine) release. Accordingly, SGC activation in the SG enhanced cardiac sympathetic hyperactivity 2 hours post-MI, whereas SG-SGC inhibition suppressed MI-induced cardiac sympathetic hyperexcitability. Moreover, the persistent inhibition of SG-SGCs improved ventricular remodeling and dysfunction, alleviated SG and ventricular sympathetic nerve sprouting 7 days post-MI. In addition, the bulk RNA sequencing with SG and pharmacological purinergic P2Y1R (P2Y1 receptor) blockage indicated that P2Y1R/IGFBP2 (insulin-like growth factor-binding protein 2) signaling mediated the effects of SG-SGC activation on cardiac sympathetic hyperexcitability post-MI, and IGFBP2 bridged the interaction between the neurons and surrounding SGCs.</p><p><strong>Conclusions: </strong>SGC inhibition in SG rectifies cardiac sympathetic hyperactivity, stabilizes ventricular electrophysiological properties, and alleviates cardiac structural and neural remodeling post-MI, thereby preventing ventricular arrhythmias and cardiac dysfunction. Neuromodulation targeting SG-SGCs exhibits a safe and fruitful strategy for the treatment of MI.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013866"},"PeriodicalIF":9.8,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145191195","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":"Prediction of Atrial Fibrillation From the ECG in the Community Using Deep Learning: A Multinational Study.","authors":"Luisa C C Brant, Antônio H Ribeiro, Oseiwe B Eromosele, Marcelo M Pinto-Filho, Sandhi M Barreto, Bruce B Duncan, Martin G Larson, Emelia J Benjamin, Antonio L P Ribeiro, Honghuang Lin","doi":"10.1161/CIRCEP.125.013734","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.013734","url":null,"abstract":"<p><strong>Background: </strong>We aimed to refine and validate a deep neural network model from the ECG to predict atrial fibrillation (AF) risk, using samples from diverse backgrounds: the Framingham Heart Study (FHS), UK Biobank, and Estudo Longitudinal da Saúde do Adulto (ELSA-Brasil). We compared the model's performance to the clinical Cohorts for Heart and Aging Research in Genomic Epidemiology consortium (CHARGE-AF) risk score and evaluated the association with other cardiovascular outcomes.</p><p><strong>Methods: </strong>The ECG-derived deep-learning prediction of AF (ECG-AF) model was refined using 60% of FHS samples free of AF. Its performance was then tested in the remaining FHS samples, UK Biobank, and ELSA-Brasil, with discrimination assessed by the area under the receiver operating characteristic curve. The association of ECG-AF with cardiovascular outcomes was assessed using Cox proportional hazards models.</p><p><strong>Results: </strong>The study sample included 10 097 FHS participants (mean age 53±12 years; 54.9% women), 49 280 participants from the UK Biobank (mean age 64±8 years, 47.9% women), and 12 284 participants from ELSA-Brasil (mean age 53±8 years, 54.7% women). The ECG-AF model showed moderate discrimination for incident AF (area under the curve, 0.82 [95% CI, 0.80-0.84]) in the FHS, comparable to the CHARGE-AF score (area under the curve, 0.83 [95% CI, 0.81-0.85]), and incremental when combined (area under the curve, 0.85 [95% CI, 0.83-0.87]). In UK Biobank and ELSA-Brasil, combining ECG-AF and CHARGE also improved prediction. Higher ECG-AF scores were associated with increased risks of heart failure, myocardial infarction, stroke, and all-cause mortality in all 3 cohorts.</p><p><strong>Conclusions: </strong>In multinational cohort studies, the single-input ECG-AF deep neural network model demonstrated good performance in predicting AF and other cardiovascular outcomes, comparable to a multivariable clinical risk score, with improved performance when combined.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013734"},"PeriodicalIF":9.8,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145191315","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":"Prevalence and Clinical Impact of Postural Orthostatic Tachycardia Syndrome in Highly Symptomatic Long COVID.","authors":"Mikael Björnson, Klara Wijnbladh, Anna Törnberg, Anna Svensson-Raskh, Annie Svensson, Marcus Ståhlberg, Michael Runold, Artur Fedorowski, Malin Nygren Bonnier, Judith Bruchfeld","doi":"10.1161/CIRCEP.124.013629","DOIUrl":"https://doi.org/10.1161/CIRCEP.124.013629","url":null,"abstract":"<p><strong>Background: </strong>The incidence of postural orthostatic tachycardia syndrome (POTS) in long COVID has been a growing concern since the first cases were reported in 2021. The aim of this study was to assess the prevalence and clinical impact of POTS in a series of well-characterized patients with long COVID.</p><p><strong>Methods: </strong>We prospectively analyzed 467 nonhospitalized, highly symptomatic (sick leave ≥50%) patients with long COVID, and studied differences in demographics and clinical assessment outcomes between those diagnosed with POTS and the remaining long COVID patients. Examinations were performed at a median of 12 months after acute COVID-19, followed by a cardiologist evaluation with 48-hour ECG, head-up tilt test, and Active Stand Test for those with clinically suspected POTS.</p><p><strong>Results: </strong>Of all long COVID patients, 143 (31%) were diagnosed with POTS, 128 (27%) did not fulfill POTS criteria, while 196 (42%) had no clinical signs of POTS. Patients with POTS were younger (mean age, 40.0 versus 44.0 versus 47.0 years, respectively; <i>P</i>≤0.001) and predominantly female (91%). They had significantly lower physical activity compared with the other 2 groups, as measured with the Frändin-Grimby scale (<i>P</i>=0.001). Heart rates during the 6-minute walk test were significantly higher in the POTS group, both during walking and at rest afterward, with a significantly shorter walking distance (448 m versus 472 m versus 509 m, respectively; <i>P</i>≤0.001). However, the distribution of symptoms showed no significant differences between the groups.</p><p><strong>Conclusions: </strong>In this cohort of predominantly younger women with highly symptomatic long COVID, POTS is common and presents with overlapping symptoms between POTS and non-POTS patients. Long COVID POTS confers lower physical activity and capacity compared with non-POTS long COVID and should be systematically assessed in this condition.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013629"},"PeriodicalIF":9.8,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145191367","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":"Efficacy of Low-Voltage-Area Ablation Is Enhanced in Patients With Advanced Left Atrial Enlargement: A Subanalysis of the SUPPRESS-AF Trial.","authors":"Masaharu Masuda, Yasuhiro Matsuda, Hiroyuki Uematsu, Hirotaka Ooka, Satoshi Kudo, Mizuki Ochi, Toshiaki Mano, Akihiro Sunaga, Nobuaki Tanaka, Tetsuya Watanabe, Hitoshi Minamiguchi, Yasuyuki Egami, Takafumi Oka, Tomoko Minamisaka, Takashi Kanda, Masato Okada, Masato Kawasaki, Koji Tanaka, Nobuhiko Makino, Hirota Kida, Shungo Hikoso, Tomoharu Dohi, Koichi Inoue, Yohei Sotomi, Yasushi Sakata","doi":"10.1161/CIRCEP.125.014210","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.014210","url":null,"abstract":"<p><strong>Background: </strong>In the randomized controlled SUPPRESS-AF trial, the efficacy of low-voltage-area (LVA) ablation was highly dependent on the degree of atrial remodeling, while the efficacy was not statistically significant in total patients. This subanalysis of the SUPPRESS-AF trial aimed to compare the efficacy of LVA ablation in patient groups classified by left atrial diameter (LAD), which is a commonly used atrial remodeling index.</p><p><strong>Methods: </strong>The SUPPRESS-AF trial included patients with persistent AF and left atrial LVAs, and compared rhythm outcomes between patients randomized to undergo pulmonary vein isolation (PVI) followed by left atrial LVA ablation group (n=170) or PVI-alone group (n=172). In this post hoc subanalysis, patients in each of the 2 randomly allocated groups were further divided into 2 groups using a median LAD of 44 mm.</p><p><strong>Results: </strong>Atrial fibrillation or atrial tachycardia recurrence-free rates did not differ between patients with LAD>44 mm and ≤44 mm (60.1% versus 53.7%; <i>P</i>=0.261). Among patients with a LAD>44 mm, the LVA ablation group demonstrated a higher atrial fibrillation or atrial tachycardia-recurrence-free rate than the PVI-alone group (62.5% versus 43.4%; <i>P</i>=0.016). In contrast, no difference in atrial fibrillation or atrial tachycardia recurrence-free rate was found between the 2 groups of patients with a LAD≤44 mm (60.8% versus 59.6%; <i>P</i>=0.986).</p><p><strong>Conclusions: </strong>The efficacy of LVA ablation in addition to PVI for the treatment of persistent AF was more pronounced in patients with a large left atrium.</p><p><strong>Registration: </strong>URL: https://www.umin.ac.jp/ctr; Unique identifier: UMIN000035940.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e014210"},"PeriodicalIF":9.8,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145148222","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":"Prevalence and Clinical Course of Suspected Acute Pericarditis Following Atrial Pulsed-Field Ablation.","authors":"Aleksandar Izda, Anika Sonig, Alyson Brown, Erin Capodanno, Maria Matteo, Bryan Baranowski, Mandeep Bhargava, Thomas Callahan, Thomas J Dresing, Koji Higuchi, Ayman Hussein, Mohamed Kanj, Arshneel Kochar, Robert Koeth, Justin Lee, David Martin, Shady Nakhla, Walid Saliba, Tyler Taigen, Niraj Varma, Oussama Wazni, Pasquale Santangeli, Mina K Chung, Jakub Sroubek","doi":"10.1161/CIRCEP.125.014263","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.014263","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e014263"},"PeriodicalIF":9.8,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145147798","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":"Intellectual and Neurodevelopmental Delays in Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: Distinct Characteristics and a More Malignant Neurocardiac Phenotype.","authors":"Christina Y Miyake, Dania Kallas, Sara B Stephens, Oliver M Moore, Xander H T Wehrens, Peter S Fischbach, Martin J LaPage, Andrew P Landstrom, Ian H Law, Allison C Hill, Prince J Kannankeril, Frank A Fish, Taylor S Howard, Santiago O Valdes, Tam Dam Pham, Jeffrey J Kim, Santokh Dhillon, Christopher L Johnsrude, Ulrich Krause, Georgia Sarquella-Brugada, Peter Kubus, Terezia Tavacova, Sit-Yee Kwok, Susan P Etheridge, Svjetlana Tisma-Dupanovic, Adam C Kean, Andrew D Krahn, Mohammed Ebrahim, Joseph Atallah, Anne Fournier, Anjan S Batra, Ming-Lon Young, James Perry, Joshua R Kovach, Anna N Kamp, Bradley C Clark, Erick Jimenez, Fatme Charafeddine, Robert M Hamilton, Seshadri Balaji, Shubhayan Sanatani","doi":"10.1161/CIRCEP.124.013437","DOIUrl":"https://doi.org/10.1161/CIRCEP.124.013437","url":null,"abstract":"<p><strong>Background: </strong>Marked intellectual and neurodevelopmental delay (INDD) was noted in 6 unrelated patients diagnosed with <i>RYR2</i>-related catecholaminergic polymorphic ventricular tachycardia (CPVT) from a single center. Patients exhibited similar distinct phenotypic features not previously described. We aimed to determine the prevalence of INDD in CPVT, compare clinical characteristics between patients with CPVT with and without INDD, and investigate the possibility of a unique neurocardiac CPVT phenotype.</p><p><strong>Methods: </strong>Retrospective combined review of patients with <i>RYR2</i>-related CPVT diagnosed ≤18 years with and without INDD from a single center and the International Pediatric CPVT Registry. Patients with hypoxic ischemic insult were excluded unless INDD preceded injury.</p><p><strong>Results: </strong>Among a total of 168 patients, INDD was reported in 19 (11.3% [95% CI, 7.0%-17.1%]). When compared with cases without INDD, patients with INDD exhibited distinct features including (1) younger age at onset of symptoms (median 7.0 versus 10.0 years; <i>P</i>=0.04); (2) higher frequency of atrial tachyarrhythmias (84.2% versus 16.3%, <i>P</i><0.001); (3) atrial or ventricular tachycardia without adrenergic stimulation (81.3% versus 2.2%, <i>P</i><0.001, 31.6% versus 4.5%, <i>P</i>=0.001 respectively); (4) cardiac structural changes or systolic dysfunction (36.8% versus 1.3%, <i>P</i><0.001); and (5) higher incidence of cardiac arrest or sudden death after diagnosis (26.3% versus 2.7%, <i>P</i>=0.001). INDD-related <i>RYR2</i> genetic variants clustered within the central and channel domains and may be specific to certain variants.</p><p><strong>Conclusions: </strong>This study demonstrates a wider spectrum of <i>RYR2</i>-related disease, with a subset associated with extracardiac manifestations. Certain <i>RYR2</i> variants may lead to a neurocardiac phenotype with distinct features that are important to recognize, as these patients may be at higher risk.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013437"},"PeriodicalIF":9.8,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145148149","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":"Comparison of Extrastimulus Pacing Strategies for the Detection of Arrhythmogenic Substrate for Ventricular Tachycardia: Insights From a Porcine Ischemia-Reperfusion Injury Model.","authors":"Ashwin Bhaskaran, Tejas Deshmukh, Dinesh Selvakumar, Richard Bennett, Samual Turnbull, Timothy G Campbell, Yasuhito Kotake, Michael A Barry, Juntang Lu, Lachlan Pearson, Eddy Kizana, James J H Chong, Saurabh Kumar","doi":"10.1161/CIRCEP.125.013902","DOIUrl":"https://doi.org/10.1161/CIRCEP.125.013902","url":null,"abstract":"<p><strong>Background: </strong>Multiple extrastimulus (ES) pacing protocols exist for ventricular substrate mapping. Despite being increasingly adopted in clinical practice, direct protocol comparisons have been limited. This study aims to compare the substrate delineation and mapping efficiency of right ventricular pacing+ES (RVp+ES) and sensed ES pacing strategies in a large animal ischemia-reperfusion injury model.</p><p><strong>Methods: </strong>Four swine underwent 90-minute balloon occlusion of the mid-left anterior descending artery, followed by late gadolinium-enhanced cardiac magnetic resonance between days 30 and 58 and invasive electroanatomic mapping. Late gadolinium-enhanced cardiac magnetic resonances were segmented for scar topography and border zone channel geometry.</p><p><strong>Results: </strong>Sensed ES substrate maps had greater point density (12.90±4.20 pts/cm² versus 5.75±0.52 pts/cm²; <i>P</i>=0.032) and faster acquisition (113.71±22.38 s/pt per cm² versus 228.57±77.30 s/pt per cm²; <i>P</i>=0.027) than RVp+ES. Despite this, RVp+ES substrate maps had greater uncovering of split potentials within border zone channels (76.5% [15.4%-95.5%] versus 16.7% [0%-52.9%]; <i>P</i>=0.028), higher sensitivity (53% versus 30%), and similarly high specificity (91% versus 93%) than sensed ES, as well as better visual correlation on decrement-evoked potential maps. Bipolar voltage in sinus rhythm and RVp did not reliably predict tissue response to ES, with 46% to 57% of split potentials within border zone channels arising from seemingly normal voltage (≥1.5 mV).</p><p><strong>Conclusions: </strong>RVp+ES is more sensitive than sensed ES and highly specific for the detection of late gadolinium-enhanced cardiac magnetic resonance border zone channels postmyocardial infarct.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013902"},"PeriodicalIF":9.8,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145136645","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}