Circulation. Arrhythmia and electrophysiology最新文献

{"title":"Long-Term Outcomes of Cardiac Resynchronization Therapy in Patients With Repaired Tetralogy of Fallot: A Multicenter Study.","authors":"Nawin L Ramdat Misier, Jeremy P Moore, Hoang H Nguyen, Michael S Lloyd, Anne M Dubin, Douglas Y Mah, Richard J Czosek, Paul Khairy, Philip M Chang, Jens C Nielsen, Alper Aydin, Thomas A Pilcher, Edward T O'Leary, Kalyanam Shivkumar, Natasja M S de Groot","doi":"10.1161/CIRCEP.123.012363","DOIUrl":"10.1161/CIRCEP.123.012363","url":null,"abstract":"<p><strong>Background: </strong>A growing number of patients with tetralogy of Fallot develop left ventricular systolic dysfunction and heart failure, in addition to right ventricular dysfunction. Although cardiac resynchronization therapy (CRT) is an established treatment option, the effect of CRT in this population is still not well defined. This study aimed to investigate the early and late efficacy, survival, and safety of CRT in patients with tetralogy of Fallot.</p><p><strong>Methods: </strong>Data were analyzed from an observational, retrospective, multicenter cohort, initiated jointly by the Pediatric and Congenital Electrophysiology Society and the International Society of Adult Congenital Heart Disease. Twelve centers contributed baseline and longitudinal data, including vital status, left ventricular ejection fraction (LVEF), QRS duration, and NYHA functional class. Outcomes were analyzed at early (3 months), intermediate (1 year), and late follow-up (≥2 years) after CRT implantation.</p><p><strong>Results: </strong>A total of 44 patients (40.3±19.2 years) with tetralogy of Fallot and CRT were enrolled. Twenty-nine (65.9%) patients had right ventricular pacing before CRT upgrade. The left ventricular ejection fraction improved from 32% [24%-44%] at baseline to 42% [32%-50%] at early follow-up (<i>P</i><0.001) and remained improved from baseline thereafter (<i>P</i>≤0.002). The QRS duration decreased from 180 [160-205] ms at baseline to 152 [133-182] ms at early follow-up (<i>P</i><0.001) and remained decreased at intermediate and late follow-up (<i>P</i>≤0.001). Patients with upgraded CRT had consistent improvement in left ventricular ejection fraction and QRS duration at each time point (<i>P</i>≤0.004). Patients had a significantly improved New York Heart Association functional class after CRT implantation at each time point compared with baseline (<i>P</i>≤0.002). The transplant-free survival rates at 3, 5, and 8 years after CRT implantation were 85%, 79%, and 73%.</p><p><strong>Conclusions: </strong>In patients with tetralogy of Fallot treated with CRT consistent improvement in QRS duration, left ventricular ejection fraction, New York Heart Association functional class, and reasonable long-term survival were observed. The findings from this multicenter study support the consideration of CRT in this unique population.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012363"},"PeriodicalIF":8.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139721854","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":"Sweetened Beverages, Genetic Susceptibility, and Incident Atrial Fibrillation: A Prospective Cohort Study.","authors":"Ying Sun, Bowei Yu, Yuefeng Yu, Bin Wang, Xiao Tan, Yingli Lu, Yu Wang, Kun Zhang, Ningjian Wang","doi":"10.1161/CIRCEP.123.012145","DOIUrl":"10.1161/CIRCEP.123.012145","url":null,"abstract":"<p><strong>Background: </strong>An association between sweetened beverages and several cardiometabolic diseases has been reported, but their association with atrial fibrillation (AF) is unclear. We aimed to investigate the associations between consumption of sugar-sweetened beverages (SSB), artificially sweetened beverages (ASB), and pure fruit juice (PJ) and risk of consumption with AF risk and further evaluate whether genetic susceptibility modifies these associations.</p><p><strong>Methods: </strong>A total of 201 856 participants who were free of baseline AF, had genetic data available, and completed a 24-hour diet questionnaire were included. Cox proportional hazard models were used to estimate the hazard ratios (HRs) and 95% confidence intervals (CIs).</p><p><strong>Results: </strong>During a median follow-up of 9.9 years, 9362 incident AF cases were documented. Compared with nonconsumers, individuals who consumed >2 L/wk of SSB or ASB had an increased risk of AF (HR, 1.10 [95% CI, 1.01-1.20] and HR, 1.20 [95% CI, 1.10-1.31]) in the multivariable-adjusted model. A negative association was observed between the consumption of ≤1 L/wk of PJ and the risk of AF (HR, 0.92 [95% CI, 0.87-0.97]). The highest HRs (95% CIs) of AF were observed for participants at high genetic risk who consumed >2 L/wk of ASB (HR, 3.51 [95% CI, 2.94-4.19]), and the lowest HR were observed for those at low genetic risk who consumed ≤1 L/wk of PJ (HR, 0.77 [95% CI, 0.65-0.92]). No significant interactions were observed between the consumption of SSB, ASB, or PJ and genetic predisposition to AF.</p><p><strong>Conclusions: </strong>Consumption of SSB and ASB at >2 L/wk was associated with an increased risk for AF. PJ consumption ≤1 L/wk was associated with a modestly lower risk for AF. The association between sweetened beverages and AF risk persisted after adjustment for genetic susceptibility to AF. This study does not demonstrate that consumption of SSB and ASB alters AF risk but rather that the consumption of SSB and ASB may predict AF risk beyond traditional risk factors.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012145"},"PeriodicalIF":8.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140027466","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":"Is Seeing Believing? Adding Another Tool to Assess PFA Durability.","authors":"Nishaki Kiran Mehta, David E Haines","doi":"10.1161/CIRCEP.124.012773","DOIUrl":"10.1161/CIRCEP.124.012773","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012773"},"PeriodicalIF":8.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139995781","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 BLISTER Score: A Novel, Externally Validated Tool for Predicting Cardiac Implantable Electronic Device Infections, and Its Cost-Utility Implications for Antimicrobial Envelope Use.","authors":"Edd Maclean, Karishma Mahtani, Shohreh Honarbakhsh, Charles Butcher, Nikhil Ahluwalia, Adam S C Dennis, Antonio Creta, Malcolm Finlay, Mark Elliott, Vishal Mehta, Nadeev Wijesuriya, Omar Shaikh, Yom Zaw, Chizute Ogbedeh, Vasu Gautam, Pier D Lambiase, Richard J Schilling, Mark J Earley, Philip Moore, Amal Muthumala, Simon C E Sporton, Ross J Hunter, Christopher A Rinaldi, Jonathan Behar, Claire Martin, Christopher Monkhouse, Anthony Chow","doi":"10.1161/CIRCEP.123.012446","DOIUrl":"10.1161/CIRCEP.123.012446","url":null,"abstract":"<p><strong>Background: </strong>Antimicrobial envelopes reduce the incidence of cardiac implantable electronic device infections, but their cost restricts routine use in the United Kingdom. Risk scoring could help to identify which patients would most benefit from this technology.</p><p><strong>Methods: </strong>A novel risk score (BLISTER [Blood results, Long procedure time, Immunosuppressed, Sixty years old (or younger), Type of procedure, Early re-intervention, Repeat procedure]) was derived from multivariate analysis of factors associated with cardiac implantable electronic device infection. Diagnostic utility was assessed against the existing PADIT score (Prior procedure, Age, Depressed renal function, Immunocompromised, Type of procedure) in both standard and high-risk external validation cohorts, and cost-utility models examined different BLISTER and PADIT score thresholds for TYRX (Medtronic; Minneapolis, MN) antimicrobial envelope allocation.</p><p><strong>Results: </strong>In a derivation cohort (n=7383), cardiac implantable electronic device infection occurred in 59 individuals within 12 months of a procedure (event rate, 0.8%). In addition to the PADIT score constituents, lead extraction (hazard ratio, 3.3 [95% CI, 1.9-6.1]; <i>P</i><0.0001), C-reactive protein >50 mg/L (hazard ratio, 3.0 [95% CI, 1.4-6.4]; <i>P</i>=0.005), reintervention within 2 years (hazard ratio, 10.1 [95% CI, 5.6-17.9]; <i>P</i><0.0001), and top-quartile procedure duration (hazard ratio, 2.6 [95% CI, 1.6-4.1]; <i>P</i>=0.001) were independent predictors of infection. The BLISTER score demonstrated superior discriminative performance versus PADIT in the standard risk (n=2854, event rate: 0.8%, area under the curve, 0.82 versus 0.71; <i>P</i>=0.001) and high-risk validation cohorts (n=1961, event rate: 2.0%, area under the curve, 0.77 versus 0.69; <i>P</i>=0.001), and in all patients (n=12 198, event rate: 1%, area under the curve, 0.8 versus 0.75, <i>P</i>=0.002). In decision-analytic modeling, the optimum scenario assigned antimicrobial envelopes to patients with BLISTER scores ≥6 (10.8%), delivering a significant reduction in infections (relative risk reduction, 30%; <i>P</i>=0.036) within the National Institute for Health and Care Excellence cost-utility thresholds (incremental cost-effectiveness ratio, £18 446).</p><p><strong>Conclusions: </strong>The BLISTER score (https://qxmd.com/calculate/calculator_876/the-blister-score-for-cied-infection) was a valid predictor of cardiac implantable electronic device infection, and could facilitate cost-effective antimicrobial envelope allocation to high-risk patients.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012446"},"PeriodicalIF":8.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10949977/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139518991","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}

{"title":"Utilizing Human-Induced Pluripotent Stem Cells to Study Cardiac Electroporation Pulsed-Field Ablation.","authors":"Leonid Maizels, Eyal Heller, Michal Landesberg, Shany Glatstein, Irit Huber, Gil Arbel, Amira Gepstein, Doron Aronson, Shirley Sharabi, Roy Beinart, Amit Segev, Elad Maor, Lior Gepstein","doi":"10.1161/CIRCEP.123.012278","DOIUrl":"10.1161/CIRCEP.123.012278","url":null,"abstract":"<p><strong>Background: </strong>Electroporation is a promising nonthermal ablation method for cardiac arrhythmia treatment. Although initial clinical studies found electroporation pulsed-field ablation (PFA) both safe and efficacious, there are significant knowledge gaps concerning the mechanistic nature and electrophysiological consequences of cardiomyocyte electroporation, contributed by the paucity of suitable human in vitro models. Here, we aimed to establish and characterize a functional in vitro model based on human-induced pluripotent stem cells (hiPSCs)-derived cardiac tissue, and to study the fundamentals of cardiac PFA.</p><p><strong>Methods: </strong>hiPSC-derived cardiomyocytes were seeded as circular cell sheets and subjected to different PFA protocols. Detailed optical mapping, cellular, and molecular characterizations were performed to study PFA mechanisms and electrophysiological outcomes.</p><p><strong>Results: </strong>PFA generated electrically silenced lesions within the hiPSC-derived cardiac circular cell sheets, resulting in areas of conduction block. Both reversible and irreversible electroporation components were identified. Significant electroporation reversibility was documented within 5 to 15-minutes post-PFA. Irreversibly electroporated regions persisted at 24-hours post-PFA. Per single pulse, high-frequency PFA was less efficacious than standard (monophasic) PFA, whereas increasing pulse-number augmented lesion size and diminished reversible electroporation. PFA augmentation could also be achieved by increasing extracellular Ca²⁺ levels. Flow-cytometry experiments revealed that regulated cell death played an important role following PFA. Assessing for PFA antiarrhythmic properties, sustainable lines of conduction block could be generated using PFA, which could either terminate or isolate arrhythmic activity in the hiPSC-derived cardiac circular cell sheets.</p><p><strong>Conclusions: </strong>Cardiac electroporation may be studied using hiPSC-derived cardiac tissue, providing novel insights into PFA temporal and electrophysiological characteristics, facilitating electroporation protocol optimization, screening for potential PFA-sensitizers, and investigating the mechanistic nature of PFA antiarrhythmic properties.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012278"},"PeriodicalIF":8.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10949974/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139721856","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}

{"title":"Artificial Sweeteners: A New Dietary Environmental Risk Factor for Atrial Fibrillation?","authors":"Robert A Koeth, Jonathan D Smith, Mina K Chung","doi":"10.1161/CIRCEP.124.012761","DOIUrl":"10.1161/CIRCEP.124.012761","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012761"},"PeriodicalIF":8.4,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10958529/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140027465","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}

{"title":"Cardiac Pulsed Field Ablation Lesion Durability Assessed by Polarization-Sensitive Optical Coherence Reflectometry.","authors":"Maria Terricabras, Raphael P Martins, Rafael Peinado, Paweł Derejko, Lluís Mont, Sabine Ernst, David Herranz, Christophe Bailleul, Atul Verma","doi":"10.1161/CIRCEP.123.012255","DOIUrl":"10.1161/CIRCEP.123.012255","url":null,"abstract":"<p><strong>Background: </strong>Pulsed field ablation uses electrical fields to cause nonthermal cell death over several hours. Polarization-sensitive optical coherence reflectometry is an optical imaging technique that can detect changes in the tissue ultrastructure in real time, which occurs when muscular tissue is damaged. The objective of this study was to evaluate the ability of a polarization-sensitive optical coherence reflectometry system to predict the development of chronic lesions based on acute changes in tissue birefringence during pulsed field ablation.</p><p><strong>Methods: </strong>Superior vena cava isolation was performed in 30 swine using a biphasic, bipolar pulsed field ablation system delivered with a nonirrigated focal tip catheter. Acute changes in tissue birefringence and voltage abatement were analyzed for each individual lesion. A high-resolution electroanatomical map was performed at baseline and 4 to 12 weeks after ablation to locate electrical gaps in the ablated area.</p><p><strong>Results: </strong>A total of 141 lesions were delivered and included in the analysis. Acute electrical isolation based on the electroanatomical map was achieved in 96% of the animals, but chronic isolation was only seen in 14 animals (46%). The mean voltage abatement of lesions that showed recovery was 82.8%±14.6% versus 84.4%±17.4% for those that showed fibrosis (<i>P</i>=0.7). The mean acute reduction in tissue birefringence in points demonstrating fibrosis was 63.8%±11.3% versus 9.1%±0.1% in the points that resulted in electrical gaps. A threshold of acute reduction of birefringence of ≥20% could predict chronic lesion formation with a sensitivity of 96% and a specificity of 83%.</p><p><strong>Conclusions: </strong>Acute tissue birefringence changes assessed with polarization-sensitive optical coherence reflectometry during pulsed field ablation can predict chronic lesion formation and guide the ablation procedure although limited by the tissue thickness.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012255"},"PeriodicalIF":9.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10949975/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139691393","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}

{"title":"Spatially Conserved Spiral Wave Activity During Human Atrial Fibrillation.","authors":"Wouter-Jan Rappel, Tina Baykaner, Junaid Zaman, Prasanth Ganesan, Albert J Rogers, Sanjiv M Narayan","doi":"10.1161/CIRCEP.123.012041","DOIUrl":"10.1161/CIRCEP.123.012041","url":null,"abstract":"<p><strong>Background: </strong>Atrial fibrillation is the most common cardiac arrhythmia in the world and increases the risk for stroke and morbidity. During atrial fibrillation, the electric activation fronts are no longer coherently propagating through the tissue and, instead, show rotational activity, consistent with spiral wave activation, focal activity, collision, or partial versions of these spatial patterns. An unexplained phenomenon is that although simulations of cardiac models abundantly demonstrate spiral waves, clinical recordings often show only intermittent spiral wave activity.</p><p><strong>Methods: </strong>In silico data were generated using simulations in which spiral waves were continuously created and annihilated and in simulations in which a spiral wave was intermittently trapped at a heterogeneity. Clinically, spatio-temporal activation maps were constructed using 60 s recordings from a 64 electrode catheter within the atrium of N=34 patients (n=24 persistent atrial fibrillation). The location of clockwise and counterclockwise rotating spiral waves was quantified and all intervals during which these spiral waves were present were determined. For each interval, the angle of rotation as a function of time was computed and used to determine whether the spiral wave returned in step or changed phase at the start of each interval.</p><p><strong>Results: </strong>In both simulations, spiral waves did not come back in phase and were out of step.\" In contrast, spiral waves returned in step in the majority (68%; <i>P</i>=0.05) of patients. Thus, the intermittently observed rotational activity in these patients is due to a temporally and spatially conserved spiral wave and not due to ones that are newly created at the onset of each interval.</p><p><strong>Conclusions: </strong>Intermittency of spiral wave activity represents conserved spiral wave activity of long, but interrupted duration or transient spiral activity, in the majority of patients. This finding could have important ramifications for identifying clinically important forms of atrial fibrillation and in guiding treatment.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012041"},"PeriodicalIF":9.1,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10950516/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139721855","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}

{"title":"Pulseless Electric Activity or Electromechanical Dissociation.","authors":"Larisa G Tereshchenko","doi":"10.1161/CIRCEP.124.012760","DOIUrl":"10.1161/CIRCEP.124.012760","url":null,"abstract":"","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012760"},"PeriodicalIF":8.4,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10922765/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139691394","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}

{"title":"Unraveling Complexities in Genetically Elusive Long QT Syndrome.","authors":"Babken Asatryan, Brittney Murray, Alessio Gasperetti, Rebecca McClellan, Andreas S Barth","doi":"10.1161/CIRCEP.123.012356","DOIUrl":"10.1161/CIRCEP.123.012356","url":null,"abstract":"<p><p>Genetic testing has become standard of care for patients with long QT syndrome (LQTS), providing diagnostic, prognostic, and therapeutic information for both probands and their family members. However, up to a quarter of patients with LQTS do not have identifiable Mendelian pathogenic variants in the currently known LQTS-associated genes. This absence of genetic confirmation, intriguingly, does not lessen the severity of LQTS, with the prognosis in these gene-elusive patients with unequivocal LQTS mirroring genotype-positive patients in the limited data available. Such a conundrum instigates an exploration into the causes of corrected QT interval (QTc) prolongation in these cases, unveiling a broad spectrum of potential scenarios and mechanisms. These include multiple environmental influences on QTc prolongation, exercise-induced repolarization abnormalities, and the profound implications of the constantly evolving nature of genetic testing and variant interpretation. In addition, the rapid advances in genetics have the potential to uncover new causal genes, and polygenic risk factors may aid in the diagnosis of high-risk patients. Navigating this multifaceted landscape requires a systematic approach and expert knowledge, integrating the dynamic nature of genetics and patient-specific influences for accurate diagnosis, management, and counseling of patients. The role of a subspecialized expert cardiogenetic clinic is paramount in evaluation to navigate this complexity. Amid these intricate aspects, this review outlines potential causes of gene-elusive LQTS. It also provides an outline for the evaluation of patients with negative and inconclusive genetic test results and underscores the need for ongoing adaptation and reassessment in our understanding of LQTS, as the complexities of gene-elusive LQTS are increasingly deciphered.</p>","PeriodicalId":10319,"journal":{"name":"Circulation. Arrhythmia and electrophysiology","volume":" ","pages":"e012356"},"PeriodicalIF":8.4,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139541762","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}