Nak Hyun Choi, Sara Cherny, Charles I Berul, William R Goodyer, Taylor S Howard, Anna Joong, Leonardo Liberman, Eric S Silver, Chet R Villa, Teresa M Lee, Warren A Zuckerman
{"title":"Desmoplakin Cardiomyopathy in Pediatric Patients: A Distinct, Underrecognized Cohort of Arrhythmogenic Cardiomyopathy.","authors":"Nak Hyun Choi, Sara Cherny, Charles I Berul, William R Goodyer, Taylor S Howard, Anna Joong, Leonardo Liberman, Eric S Silver, Chet R Villa, Teresa M Lee, Warren A Zuckerman","doi":"10.1161/CIRCEP.124.013114","DOIUrl":"10.1161/CIRCEP.124.013114","url":null,"abstract":"<p><strong>Background: </strong><i>DSP</i> cardiomyopathy is a distinct subset of arrhythmogenic cardiomyopathy, reported primarily in adults, that has predominantly left ventricular involvement and features of myocarditis. Clinical characteristics, risk stratification, and management of pediatric patients with <i>DSP</i> variants are not well known. We sought to identify phenotypic features and prognosis of pediatric patients with <i>DSP</i> pathogenic or likely pathogenic variants.</p><p><strong>Methods: </strong>Multicenter, retrospective study of patients <21 years of age with <i>DSP</i> variants from 6 tertiary pediatric hospitals.</p><p><strong>Results: </strong>Thirty-four patients, including 10 probands with clinical disease and 24 genotype-positive phenotype-negative patients, were included in the study. The majority of probands were initially diagnosed with myocarditis (50%) and had biventricular (60%) or left ventricular predominant (40%) disease. Chest pain was the most common symptom at presentation (30%), and all had troponin elevation. Probands with homozygous or compound heterozygous <i>DSP</i> variants were likely to present at an early age (<13 years) with symptoms of heart failure, severe biventricular involvement, and dermatologic abnormalities. Low-voltage QRS was the most prominent ECG abnormality. Of those who underwent implantable cardioverter defibrillator implantation, 50% received appropriate implantable cardioverter defibrillator therapy and were found to have significant biventricular involvement in addition to severe left ventricular dysfunction with an ejection fraction <35%.</p><p><strong>Conclusions: </strong><i>DSP</i> cardiomyopathy in children and adolescents has varied phenotypic manifestations based on age and genotype and often can be diagnosed as myocarditis. Severe left ventricular dysfunction and biventricular involvement may be associated with a higher likelihood of malignant ventricular tachyarrhythmia.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013114"},"PeriodicalIF":9.1,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142614836","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}
Marek Jastrzębski, Paul Foley, Badrinathan Chandrasekaran, Zachary Whinnett, Pugazhendhi Vijayaraman, Gaurav A Upadhyay, Robert D Schaller, Rafał Gardas, Travis Richardson, D'Anne Kudlik, Robert W Stadler, Patrick Zimmerman, James Burrell, Robert Waxman, Richard N Cornelussen, Jonathan Lyne, Bengt Herweg
{"title":"Multicenter Hemodynamic Assessment of the LOT-CRT Strategy: When Does Combining Left Bundle Branch Pacing and Coronary Venous Pacing Enhance Resynchronization?: Primary Results of the CSPOT Study.","authors":"Marek Jastrzębski, Paul Foley, Badrinathan Chandrasekaran, Zachary Whinnett, Pugazhendhi Vijayaraman, Gaurav A Upadhyay, Robert D Schaller, Rafał Gardas, Travis Richardson, D'Anne Kudlik, Robert W Stadler, Patrick Zimmerman, James Burrell, Robert Waxman, Richard N Cornelussen, Jonathan Lyne, Bengt Herweg","doi":"10.1161/CIRCEP.124.013059","DOIUrl":"10.1161/CIRCEP.124.013059","url":null,"abstract":"<p><strong>Background: </strong>Left bundle branch area pacing (LBBAP) may be an alternative to biventricular pacing (BVP) for cardiac resynchronization therapy (CRT). We sought to compare the acute hemodynamic and ECG effects of LBBAP, BVP, and left bundle-optimized therapy CRT (LOT-CRT) in CRT candidates with advanced conduction disease.</p><p><strong>Methods: </strong>In this multicenter study, 48 patients with either nonspecific interventricular conduction delay (n=29) or left bundle branch block (n=19) underwent acute hemodynamic testing to determine the change in left ventricular pressure maximal first derivative (LV d<i>P</i>/d<i>t</i><sub>max</sub>) from baseline atrial pacing to BVP, LBBAP, or LOT-CRT.</p><p><strong>Results: </strong>Atrioventricular-optimized increases in LV d<i>P</i>/d<i>t</i><sub>max</sub> for LOT-CRT (mean, 25.8% [95% CI, 20.9%-30.7%]) and BVP (26.4% [95% CI, 20.2%-32.6%]) were greater than unipolar LBBAP (19.3% [95% CI, 15.0%-23.7%]) or bipolar LBBAP (16.4% [95% CI, 12.7%-20.0%]; <i>P</i>≤0.005). QRS shortening was greater in LOT-CRT (29.5 [95% CI, 23.4-35.6] ms) than unipolar LBBAP (11.9 [95% CI, 6.1-17.7] ms), bipolar LBBAP (11.7 ms [95% CI, 6.4-17.0]), or BVP (18.5 [95% CI, 11.0-25.9] ms), all <i>P</i>≤0.005. Compared with patients with left bundle branch block, patients with interventricular conduction delay experienced less QRS reduction (<i>P</i>=0.026) but similar improvements in LV d<i>P</i>/d<i>t</i><sub>max</sub> (<i>P</i>=0.29). Bipolar LBBAP caused anodal capture in 54% of patients and resulted in less LV d<i>P</i>/d<i>t</i><sub>max</sub> improvement than unipolar LBBAP (18.6% versus 23.7%; <i>P</i><0.001). Subclassification of LBBAP capture (European Heart Rhythm Association criteria) indicated LBBAP or LV septal pacing in 27 patients (56%) and deep septal pacing in 21 patients (44%). The hemodynamic benefit of adding left ventricular coronary vein pacing to LBBAP depended on baseline QRS duration (<i>P</i>=0.031) and success of LBBAP (<i>P</i><0.004): LOT-CRT provided 14.5% (5.0%-24.1%) greater LV d<i>P</i>/d<i>t</i><sub>max</sub> improvement and 20.8 (12.8-28.8) ms greater QRS shortening than LBBAP in subjects with QRS ≥171 ms and deep septal pacing capture type.</p><p><strong>Conclusions: </strong>In a CRT cohort with advanced conduction disease, LOT-CRT and BVP provided greater acute hemodynamic benefit than LBBAP. Subjects with wider QRS or deep septal pacing are more likely to benefit from the addition of a left ventricular coronary vein lead to implement LOT-CRT.</p><p><strong>Registration: </strong>URL: https://www.clinicaltrials.gov; Unique identifier: NCT04905290.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013059"},"PeriodicalIF":9.1,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11575906/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142496197","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Shaan Khurshid, Samuel F Friedman, Shinwan Kany, Rahul Mahajan, Ashby C Turner, Steven A Lubitz, Mahnaz Maddah, Patrick T Ellinor, Christopher D Anderson
{"title":"Electrocardiogram-Based Artificial Intelligence to Discriminate Cardioembolic Stroke and Stratify Risk of Atrial Fibrillation After Stroke.","authors":"Shaan Khurshid, Samuel F Friedman, Shinwan Kany, Rahul Mahajan, Ashby C Turner, Steven A Lubitz, Mahnaz Maddah, Patrick T Ellinor, Christopher D Anderson","doi":"10.1161/CIRCEP.124.012959","DOIUrl":"10.1161/CIRCEP.124.012959","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012959"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11479813/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alvise Del Monte, Domenico Giovanni Della Rocca, Luigi Pannone, Giampaolo Vetta, Ioannis Doundoulakis, María Cespón Fernández, Lorenzo Marcon, Cinzia Monaco, Antonio Sorgente, Gezim Bala, Erwin Ströker, Juan Sieira, Alexandre Almorad, Andrea Sarkozy, Carlo de Asmundis, Gian-Battista Chierchia
{"title":"Coronary Vasospasm During Isthmus Pulsed Field Ablation With Wide Area Focal Catheter.","authors":"Alvise Del Monte, Domenico Giovanni Della Rocca, Luigi Pannone, Giampaolo Vetta, Ioannis Doundoulakis, María Cespón Fernández, Lorenzo Marcon, Cinzia Monaco, Antonio Sorgente, Gezim Bala, Erwin Ströker, Juan Sieira, Alexandre Almorad, Andrea Sarkozy, Carlo de Asmundis, Gian-Battista Chierchia","doi":"10.1161/CIRCEP.124.012923","DOIUrl":"10.1161/CIRCEP.124.012923","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012923"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079417","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":"Incidental Premature Ventricular Contractions in Young Athletes: Shape and Size (of Premature Ventricular Contractions burden) Matter.","authors":"Marius I Chukwurah, Eugene H Chung","doi":"10.1161/CIRCEP.124.013345","DOIUrl":"10.1161/CIRCEP.124.013345","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013345"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142281271","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":"Pulsed Field Ablation: The Temptation for More While Keeping It Safe.","authors":"Julia H Indik","doi":"10.1161/CIRCEP.124.013354","DOIUrl":"10.1161/CIRCEP.124.013354","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013354"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342749","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}
Thomas A Boyle, Naga Venkata K Pothineni, Melissa Austin, Poojita Shivamurthy, Timothy Markman, Gustavo Guandalini, Matthew Hyman, Andres Enriquez, Michael G Fradley, Robert Schaller, Gregory Supple, Rajat Deo, Vincent Y See, Michael Riley, Fermin Garcia, Saman Nazarian, David Lin, Sanjay Dixit, Andrew E Epstein, David Callans, Francis E Marchlinski, David S Frankel
{"title":"Incidence and Predictors of Pacing-Induced Right Ventricular Cardiomyopathy.","authors":"Thomas A Boyle, Naga Venkata K Pothineni, Melissa Austin, Poojita Shivamurthy, Timothy Markman, Gustavo Guandalini, Matthew Hyman, Andres Enriquez, Michael G Fradley, Robert Schaller, Gregory Supple, Rajat Deo, Vincent Y See, Michael Riley, Fermin Garcia, Saman Nazarian, David Lin, Sanjay Dixit, Andrew E Epstein, David Callans, Francis E Marchlinski, David S Frankel","doi":"10.1161/CIRCEP.124.013070","DOIUrl":"10.1161/CIRCEP.124.013070","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013070"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342747","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}
Sanghamitra Mohanty, Michela Casella, Paolo Compagnucci, Prem Geeta Torlapati, Vincenzo Mirco La Fazia, Carola Gianni, Bryan MacDonald, Angel Quintero Mayedo, Domenico Giovanni Della Rocca, John Allison, Mohamed Bassiouny, G Joseph Gallinghouse, John D Burkhardt, Rodney Horton, Amin Al-Ahmad, Luigi Di Biase, Antonio Dello Russo, Andrea Natale
{"title":"Oral Anticoagulation and Factor VIII Replacement Therapy in Patients With Hemophilia Undergoing Pulsed-Field or Radiofrequency Catheter Ablation for Atrial Fibrillation.","authors":"Sanghamitra Mohanty, Michela Casella, Paolo Compagnucci, Prem Geeta Torlapati, Vincenzo Mirco La Fazia, Carola Gianni, Bryan MacDonald, Angel Quintero Mayedo, Domenico Giovanni Della Rocca, John Allison, Mohamed Bassiouny, G Joseph Gallinghouse, John D Burkhardt, Rodney Horton, Amin Al-Ahmad, Luigi Di Biase, Antonio Dello Russo, Andrea Natale","doi":"10.1161/CIRCEP.124.012921","DOIUrl":"10.1161/CIRCEP.124.012921","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012921"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142342748","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}
Xiaodong Peng, Yukun Li, Nian Liu, Shijun Xia, Xin Li, Yiwei Lai, Liu He, Caihua Sang, Jianzeng Dong, Changsheng Ma
{"title":"Plasma Proteomic Insights for Identification of Novel Predictors and Potential Drug Targets in Atrial Fibrillation: A Prospective Cohort Study and Mendelian Randomization Analysis.","authors":"Xiaodong Peng, Yukun Li, Nian Liu, Shijun Xia, Xin Li, Yiwei Lai, Liu He, Caihua Sang, Jianzeng Dong, Changsheng Ma","doi":"10.1161/CIRCEP.124.013037","DOIUrl":"10.1161/CIRCEP.124.013037","url":null,"abstract":"<p><strong>Background: </strong>Currently, there are no reliable methods for predicting and preventing atrial fibrillation (AF) in its early stages. This study aimed to identify plasma proteins associated with AF to discover biomarkers and potential drug targets.</p><p><strong>Methods: </strong>The UK Biobank Pharma Proteomics Project examined 2923 circulating proteins using the Olink platform, forming the basis of this prospective cohort study. The UK Biobank Pharma Proteomics Project included a randomly selected discovery cohort and the consortium-selected replication cohort. The study's end point was incident AF, identified using <i>International Classification of Diseases, Tenth Revision</i> codes. The association between plasma proteins and incident AF was evaluated using Cox proportional hazard models in both cohorts. Proteins present in both cohorts underwent Mendelian randomization analysis to delineate causal connections, utilizing <i>cis</i>-protein quantitative trait loci as genetic tools. The predictive efficacy of the identified proteins for AF was assessed using the area under the receiver operating characteristic curve, and their druggability was explored.</p><p><strong>Results: </strong>Data from 38 784 participants were included in this study. Incident AF cases were identified in the discovery cohort (1894; 5.5%) within a median follow-up of 14.5 years and in the replication cohort (451; 10.6%) within a median follow-up of 14.4 years. Twenty-one proteins linked to AF were identified in both cohorts. Specifically, COL4A1 (collagen IV α-1; odds ratio, 1.11 [95% CI, 1.04-1.19]; false discovery rate, 0.016) and RET (proto-oncogene tyrosine-protein kinase receptor Ret; odds ratio, 0.96 [95% CI, 0.94-0.98]; false discovery rate, 0.013) demonstrated a causal link with AF, and RET is druggable. COL4A1 improved the short- and long-term predictive performance of established AF models, as evidenced by significant enhancements in the area under the receiver operating characteristic, integrated discrimination improvement, and net reclassification index, all with <i>P</i> values below 0.05.</p><p><strong>Conclusions: </strong>COL4A1 and RET are associated with the development of AF. RET is identified as a potential drug target for AF prevention, while COL4A1 serves as a biomarker for AF prediction. Future studies are needed to evaluate the effectiveness of targeting these proteins to reduce AF risk.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e013037"},"PeriodicalIF":9.1,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142361225","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}