Milind Y Desai, Shada Jadam, Mohammed Abusafia, Katy Rutkowski, Susan Ospina, Andrew Gaballa, Sanaa Sultana, Maran Thamilarasan, Bo Xu, Zoran B Popovic
{"title":"Real-World Artificial Intelligence-Based Electrocardiographic Analysis to Diagnose Hypertrophic Cardiomyopathy.","authors":"Milind Y Desai, Shada Jadam, Mohammed Abusafia, Katy Rutkowski, Susan Ospina, Andrew Gaballa, Sanaa Sultana, Maran Thamilarasan, Bo Xu, Zoran B Popovic","doi":"10.1016/j.jacep.2025.02.024","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.02.024","url":null,"abstract":"<p><strong>Background: </strong>There is an emerging interest in artificial intelligence-enhanced 12-lead electrocardiogram (AI-ECG) in detection of hypertrophic cardiomyopathy (HCM).</p><p><strong>Objectives: </strong>This study describes the initial real-world experience of using AI-ECG (Viz-HCM, developed using a convolutional neural network trained algorithm) in our center.</p><p><strong>Methods: </strong>All patients undergoing 12-lead electrocardiograms at Cleveland Clinic, Cleveland, Ohio, between February 19, 2024, and November 1, 2024, were prospectively analyzed for potential HCM using AI-ECG. The numbers of patients flagged for potential HCM were recorded. Presence of confirmed HCM, a new diagnosis of HCM following AI-ECG assessment (with a negative prior clinical evaluation), and alternative non-HCM diagnosis were recorded. Assessment of AI-ECG diagnostic performance was done using various HCM probability thresholds (≥0.95, ≥0.90, and ≥0.85).</p><p><strong>Results: </strong>Of 103,492 electrocardiograms analyzed in 45,873 patients, AI-ECG flagged potential HCM in 1,265 (2.7%) unique patients. Of these, 511 (40.4%) had confirmed HCM, 63 (5%) had new HCM diagnosis, and 691 (54.6%) had an alternate diagnosis. HCM probability threshold of ≥0.85 provided the highest sensitivity (95%) for diagnosis of HCM with high specificity and accuracy (all >98%). The positive predictive value was the highest (66%) at the cutoff ≥0.95 but with a lower sensitivity at 50%. The AI-ECG algorithm performed similarly in both men and women, and was more sensitive in individuals <50 years but more specific in individuals ≥50 years.</p><p><strong>Conclusions: </strong>Prospective real-world application of the AI-ECG algorithm to detect HCM was associated with a high degree of accuracy, varying with the chosen probability threshold. It also enabled the identification of 5% of patients with no prior HCM diagnosis.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143656748","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}
Dustin Nash, Anthony G Pompa, Jennifer N Avari Silva
{"title":"Automated Diagnosis of Pediatric ECG through AI-ECG: As good (and bad) as the experts.","authors":"Dustin Nash, Anthony G Pompa, Jennifer N Avari Silva","doi":"10.1016/j.jacep.2025.03.002","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.03.002","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143656825","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":"TEMPORARY REMOVAL: JACC: Clinical Electrophysiology 2024 Young Author Achievement Award Winner.","authors":"Kalyanam Shivkumar","doi":"10.1016/j.jacep.2025.02.002","DOIUrl":"10.1016/j.jacep.2025.02.002","url":null,"abstract":"<p><p>The publisher regrets that this article has been temporarily removed. A replacement will appear as soon as possible in which the reason for the removal of the article will be specified, or the article will be reinstated. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/policies/article-withdrawal.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143567131","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}
Joshua Mayourian, William G La Cava, Sarah D de Ferranti, Douglas Mah, Mark Alexander, Edward Walsh, John K Triedman
{"title":"Expert-Level Automated Diagnosis of the Pediatric ECG Using a Deep Neural Network.","authors":"Joshua Mayourian, William G La Cava, Sarah D de Ferranti, Douglas Mah, Mark Alexander, Edward Walsh, John K Triedman","doi":"10.1016/j.jacep.2025.02.003","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.02.003","url":null,"abstract":"<p><strong>Background: </strong>Disparate access to expert pediatric cardiologist care and interpretation of electrocardiograms (ECGs) persists worldwide. Artificial intelligence-enhanced ECG (AI-ECG) has shown promise for automated diagnosis of ECGs in adults but has yet to be explored in the pediatric setting.</p><p><strong>Objectives: </strong>This study sought to determine whether an AI-ECG model can accurately perform automated diagnosis of pediatric ECGs.</p><p><strong>Methods: </strong>This retrospective single-center cohort study included all patients with an ECG at Boston Children's Hospital read by an experienced pediatric cardiologist (≥5,000 reads) between 2000 and 2022. A convolutional neural network was trained (75% of patients) and internally tested (25% of patients) on ECGs to predict ECG diagnoses. The primary outcome was a composite of any ECG abnormality (ie, detecting normal vs abnormal ECG). Secondary outcomes include Wolff-Parkinson-White syndrome (WPW) and prolonged QTc. Model performance was assessed with area under the receiver-operating (AUROC) and precision recall (AUPRC) curves.</p><p><strong>Results: </strong>The main cohort consisted of 201,620 patients (49% male; 11% with known congenital heart disease) and 583,134 ECGs (median age 11.7 years [Q1-Q3: 3.1-16.9 years]; 56% any ECG abnormality, 1.0% WPW, and 5.3% with prolonged QTc). The AI-ECG model outperformed the commercial software interpretations for detecting any abnormality (AUROC 0.94; AUPRC 0.96), WPW (AUROC 0.99; AUPRC 0.88), and prolonged QTc (AUROC 0.96; AUPRC 0.63). During readjudication of ECGs with AI-ECG/original cardiologist read discordance, blinded expert readers were more likely to agree with AI-ECG classification than the original reader to detect any abnormality (P = 0.001), WPW (P = 0.01), and prolonged QTc (P = 0.07).</p><p><strong>Conclusions: </strong>Our model provides expert-level automated diagnosis of the pediatric 12-lead ECG, which may improve access to care.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143656829","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}
Angelo Auricchio, Kris Siejko, Nicholas Wold, Yinghong Yu, Michael R Gold
{"title":"The Association of Electrical Delay on Hemodynamic Response With Cardiac Resynchronization Therapy.","authors":"Angelo Auricchio, Kris Siejko, Nicholas Wold, Yinghong Yu, Michael R Gold","doi":"10.1016/j.jacep.2025.01.011","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.011","url":null,"abstract":"<p><strong>Background: </strong>The effect of electrical delay at the left ventricular (LV) pacing site on acute hemodynamic response with cardiac resynchronization therapy (CRT) has been investigated only in small observational studies.</p><p><strong>Objectives: </strong>This study evaluates the impact of electrical delay, as assessed by interventricular (right ventricular-LV]) interval, on the acute hemodynamic response to CRT in a large, diverse multicenter cohort.</p><p><strong>Methods: </strong>A total of 144 patients in 3 prospective studies, the PATH-CHF (Pacing Therapies in Congestive Heart Failure) I and II and the CRTAVO (CRT Optimization Algorithm Validation Study), were pooled and analyzed. At the time of CRT implantation, all pacing leads, pressure catheters placed in the right ventricle and left ventricle, and the surface electrocardiogram were connected to an external pacing computer. A standardized, randomized stimulation protocol was used to assess response.</p><p><strong>Results: </strong>The RV-LV interval was associated with an increase in the rate of LV pressure rise response. In the full cohort, LV effective contractility monotonically increased with a prolongation of the RV-LV time. Other significant predictors of the increase in rate of LV pressure rise were QRS duration and, to a lesser extent, female sex and ischemic etiology.</p><p><strong>Conclusions: </strong>RV-LV and QRS durations are strong predictors of the acute hemodynamic response with CRT. These findings may help in patient selection, lead placement, and pacing benefit expectation.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143633956","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":"Chemical Ablation of Atrial Fibrillation and the Fine Balancing Act of Neuromodulation in Cardiology.","authors":"Robert Lemery","doi":"10.1016/j.jacep.2025.01.008","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.008","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143567129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Cinzia Monaco, Maria Cespon-Fernandez, Luigi Pannone, Alvise Del Monte, Domenico Della Rocca, Anais Gauthey, Sahar Mouram, Lorenzo Marcon, Giampaolo Vetta, Charles Audiat, Ioannis Doudoulakis, Antonio Bisignani, Vincenzo Miraglia, Gudrun Pappaert, Ivan Eltsov, Gezim Bala, Antonio Sorgente, Ingrid Overeinder, Alexandre Almorad, Erwin Stroker, Andrea Sarkozy, Francis Wellens, Gian Battista Chierchia, Mark La Meir, Juan Sieira, Pedro Brugada, Carlo de Asmundis
{"title":"Implantable Cardioverter-Defibrillator Therapy in Brugada Syndrome: A 30-Year Single-Center Experience.","authors":"Cinzia Monaco, Maria Cespon-Fernandez, Luigi Pannone, Alvise Del Monte, Domenico Della Rocca, Anais Gauthey, Sahar Mouram, Lorenzo Marcon, Giampaolo Vetta, Charles Audiat, Ioannis Doudoulakis, Antonio Bisignani, Vincenzo Miraglia, Gudrun Pappaert, Ivan Eltsov, Gezim Bala, Antonio Sorgente, Ingrid Overeinder, Alexandre Almorad, Erwin Stroker, Andrea Sarkozy, Francis Wellens, Gian Battista Chierchia, Mark La Meir, Juan Sieira, Pedro Brugada, Carlo de Asmundis","doi":"10.1016/j.jacep.2025.01.013","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.013","url":null,"abstract":"<p><strong>Background: </strong>Brugada syndrome (BrS) continues to pose clinical challenges, despite 3 decades of dedicated research and therapeutic advancements. The pivotal role of implantable cardioverter-defibrillator (ICD) therapy in safeguarding high-risk BrS patients from sudden cardiac death due to ventricular arrhythmias is undeniable. However, the debate on risk stratification and the use of ICDs for primary prevention remains ongoing.</p><p><strong>Objectives: </strong>This study aimed to evaluate the clinical features, management, and long-term outcomes of ICD therapy in patients with Brugada syndrome.</p><p><strong>Methods: </strong>BrS-diagnosed patients were prospectively enrolled. Inclusion criteria were: 1) a Brugada type 1 electrocardiogram pattern, either spontaneous or drug induced; 2) ICD implantation; and 3) consistent follow-up. Risk stratification was based on prior arrhythmic events, and the multiparametric Brussel risk score was used from 2017. High-risk patients underwent video-thoracoscopic epicardial ablation starting in 2016. ICD implantation strategies evolved over time, guided by patients' clinical and demographic characteristics.</p><p><strong>Results: </strong>A total of 306 consecutive Brugada patients (186 male [61%]; mean age 41 ± 17 years; range: 1-82 years) received ICDs at our institution from 1992 to 2022. ICDs were implanted for secondary prevention in 16% of patients. Over the 3 decades, the proportions of secondary prevention implants and asymptomatic patients remained stable, while risk factors fluctuated in the first two decades before stabilizing. During long-term follow-up (median 103 months [63-147 months]), 14% of patients experienced at least 1 sustained ventricular arrhythmia (VA) (1.59 per 100 person-years), 15% had at least 1 inappropriate ICD shock-unaffected by the presence of single or dual leads-and 27% required device revision and/or lead replacement. Patients with secondary prevention ICDs had a higher incidence of both ventricular and supraventricular arrhythmias compared to those with primary prevention ICDs. Loss-of-function mutations and prior nonsustained VAs were associated with sustained VAs. Among high-risk patients, those who underwent epicardial ablation experienced significantly fewer ventricular events. The overall mortality rate was 5.88%, with 22.2% of deaths attributed to cardiac causes.</p><p><strong>Conclusions: </strong>This 30-year study highlights ICD therapy's critical role in preventing fatal arrhythmias in Brugada syndrome, but also reveals frequent device-related complications, especially in younger patients. Thoracoscopic epicardial ablation significantly reduced VA in high-risk patients, offering a promising adjunctive therapy. These findings emphasize the need for individualized treatment strategies to balance the benefits of ICDs with their risks, and underscore the potential of ablation to improve long-term outcomes.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143633954","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The Reappraisal of Neuropeptide Y as Biomarker and Therapeutic Target in Arrhythmic Disorders.","authors":"Tania Zaglia, Olujimi A Ajijola","doi":"10.1016/j.jacep.2025.01.012","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.012","url":null,"abstract":"","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143633957","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}
Pasquale Santangeli, Nolan Hight, Arwa Younis, Ioan Liuba, Justin Lee, Koji Higuchi, Jakub Sroubek, Shady Nakhla, Roy Chung, Walid Saliba, Mandeep Bhargava, Ayman Hussein, Mohamed Kanj, Paul Schoenhagen, Paresh Vasandani, Oussama Wazni, Samir Kapadia
{"title":"A Novel Radiofrequency-Assisted Trans-RAA Perforation Technique for Pericardial CO<sub>2</sub> Insufflation to Facilitate Epicardial Access.","authors":"Pasquale Santangeli, Nolan Hight, Arwa Younis, Ioan Liuba, Justin Lee, Koji Higuchi, Jakub Sroubek, Shady Nakhla, Roy Chung, Walid Saliba, Mandeep Bhargava, Ayman Hussein, Mohamed Kanj, Paul Schoenhagen, Paresh Vasandani, Oussama Wazni, Samir Kapadia","doi":"10.1016/j.jacep.2025.01.020","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.020","url":null,"abstract":"<p><strong>Background: </strong>Perforation of a coronary venous branch for pericardial insufflation of carbon dioxide (CO<sub>2</sub>) enhances safety of epicardial access. The multiple procedural steps and length of the procedure remain a major limitation of this approach impacting its routine clinical adoption.</p><p><strong>Objectives: </strong>This study describes a novel and streamlined technique for pericardial CO<sub>2</sub> insufflation via radiofrequency (RF)-assisted right atrial appendage (RAA) perforation.</p><p><strong>Methods: </strong>Between 2023 and 2024, 18 patients (age: 55 ± 11 years; left ventricular ejection fraction: 44 ± 13%) underwent epicardial access for mapping and ablation of ventricular arrhythmias facilitated by pericardial CO<sub>2</sub> insufflation via a microperforation of the RAA with a custom-made telescopic crossing assembly consisting of a stiff 0.014-inch guidewire within 1.8-Fr microcatheter delivered inside a 4-Fr support catheter. The proximal end of the guidewire was connected to a unipolar RF generator (20-30 W in \"cut mode\", <1 s burst) to obtain a controlled RAA wall microperforation and facilitate advancement of the 1.8-Fr microcatheter in the pericardial space for CO<sub>2</sub> insufflation.</p><p><strong>Results: </strong>Successful RAA exit with the RF guidewire-microcatheter assembly was achieved in all patients, and epicardial access was completed in 17 (94%) patients. In 1 patient, significant pericardial adhesions were detected after RAA exit, and epicardial access was deferred. The median time from femoral venous insertion of the catheter assembly to CO<sub>2</sub> insufflation was 4 min (range: 3-7 min). Significant bleeding (>80 mL) occurred in 1 patient (patient #3; 150 mL), which led to a modification of the technique (shortening of the RF burst to <1 s with 1-2 mm guidewire exposure past the microcatheter and minimal advancement during RF). After technique modification (15 patients), pericardial bleeding ranged from 5 mL-30 mL (median: 10 mL; IQR: 5-20 mL). No patient had inadvertent right ventricle puncture or damage to a coronary artery. Three patients had pericardial pain after the procedure requiring 1 week of therapy with nonsteroidal anti-inflammatory drugs and colchicine.</p><p><strong>Conclusions: </strong>Intentional RAA perforation for pericardial CO<sub>2</sub> insufflation with a custom RF guidewire-microcatheter assembly can be safely and efficiently performed to facilitate epicardial access for ventricular arrhythmias ablation.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143633952","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}
Micaela Ebert, Marta de Riva, Adrianus P Wijnmaalen, Daniela Q C M Barge-Schaapveld, Marianne Bootsma, Jarieke Hoogendoorn, Daniela Husser, J Peter van Tintelen, Jan D H Jongbloed, Sergio Richter, Antonio Berruezo, Gerhard Hindricks, William G Stevenson, Katja Zeppenfeld
{"title":"The Relevance of the Type of Ventricular Arrhythmia in Titin-Related Dilated Cardiomyopathy: A Multicenter Study.","authors":"Micaela Ebert, Marta de Riva, Adrianus P Wijnmaalen, Daniela Q C M Barge-Schaapveld, Marianne Bootsma, Jarieke Hoogendoorn, Daniela Husser, J Peter van Tintelen, Jan D H Jongbloed, Sergio Richter, Antonio Berruezo, Gerhard Hindricks, William G Stevenson, Katja Zeppenfeld","doi":"10.1016/j.jacep.2025.01.010","DOIUrl":"https://doi.org/10.1016/j.jacep.2025.01.010","url":null,"abstract":"<p><strong>Background: </strong>Truncating titin variants (TTNtvs) are the most prevalent cause of inherited dilated cardiomyopathy. Occurrence of different ventricular arrhythmia (VA) subtypes, including premature ventricular complexes (PVCs), nonsustained ventricular tachycardia (NSVT), and sustained monomorphic VT (SMVT), has been reported.</p><p><strong>Objectives: </strong>The aim of this study was to analyze the prognostic relevance of distinct VA subtypes among TTNtv carriers and their underlying arrhythmogenic substrates.</p><p><strong>Methods: </strong>Twenty-two TTNtv carriers referred for ablation of SMVT (n = 14) or frequent PVCs (n = 8) from 5 centers were included (mean age 56 ± 11 years; left ventricular ejection fraction 38% ± 13%; 77% male). Detailed phenotyping was performed, including Holter monitoring, cardiac imaging, and electroanatomical mapping. Patients were followed up for a median of 44 months.</p><p><strong>Results: </strong>Demographic characteristics, including age, comorbidities, and left ventricular ejection fraction, were similar. NSVTs were frequent in both groups but faster in patients with SMVT (cycle length: 350 milliseconds [Q1-Q3: 315-403 milliseconds] vs 427 milliseconds [Q1-Q3: 395-469 milliseconds]). Although substrates for SMVT extended in a basal ring-like fashion with septal predominance, PVC sites of origin were limited to the basal anterior left ventricular segment. In the SMVT group, acute complete procedural success was achieved for 36%; during follow-up, 86% had recurrent VT, and 50% died of progressive heart failure. In the PVC group, complete abolition of PVCs was achieved in only 13%; at 3 months, median PVC burden was 1%, and there were no deaths or sustained VT during follow-up. VA subtype and NSVT cycle length were associated with mortality and poor VT-free survival.</p><p><strong>Conclusions: </strong>In TTNtv carriers, SMVTs but not frequent PVCs are associated with high mortality due to heart failure. Occurrence of SMVT may identify a subgroup at risk for rapid, progressive adverse remodeling. The prognostic significance of different VA subtypes needs to be confirmed in a larger cohort.</p>","PeriodicalId":14573,"journal":{"name":"JACC. Clinical electrophysiology","volume":" ","pages":""},"PeriodicalIF":8.0,"publicationDate":"2025-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634016","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}