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Background and Aims Incidence and types of secondary tricuspid regurgitation (TR) are not well defined in atrial fibrillation (AFib) and sinus rhythm (SR). Atrial secondary TR (A-STR) is associated with pre-existing AFib; however, close to 50% of patients with A-STR do not have AFib. The aim of this study was to assess incidence, types, and outcomes of ≥ moderate TR in AFib vs. SR. Methods Adults with and without new-onset AFib without structural heart disease or ≥ moderate TR at baseline were followed for the development of ≥ moderate TR. Tricuspid regurgitation types were pacemaker, left-sided valve disease, left ventricular (LV) dysfunction, pulmonary hypertension (PH), isolated ventricular, and A-STR. Results Among 1359 patients with AFib and 20 438 in SR, 109 and 378 patients developed ≥ moderate TR, respectively. The individual types of TR occurred more frequently in AFib related to the higher pacemaker implantation rates (1.12 vs. 0.19 per 100 person-years, P <.001), larger right atrial size (median 78 vs. 53 mL, P <.001), and higher pulmonary pressures (median 30 vs. 28 mmHg, P <.001). The most common TR types irrespective of rhythm were LV dysfunction-TR and A-STR. Among patients in SR, those with A-STR were older, predominantly women with more diastolic abnormalities and higher pulmonary pressures. All types of secondary TR were associated with all-cause mortality, highest in PH-TR and LV dysfunction-TR. Conclusions New-onset AFib vs. SR conferred a higher risk of the individual TR types related to sequelae of AFib and higher pacemaker implantation rates, although the distribution of TR types was similar. Secondary TR was universally associated with increased mortality. [ABSTRACT FROM AUTHOR]

Key Points: Question: Cardiac arrest frequently occurs without explanation, even after a thorough clinical evaluation. Can a simple maneuver clinically diagnose calcium release deficiency syndrome (CRDS), a newly described cause of sudden death? Findings: In this international, multicenter, case-control study, a provoked measure of T-wave amplitude on an electrocardiogram ascertained cases of CRDS with high accuracy. The genetic mouse models recapitulated the human findings and suggested a pathologically large systolic calcium release from the sarcoplasmic reticulum was responsible. Meaning: These preliminary results suggest that the repolarization response on an electrocardiogram to brief tachycardia followed by a pause may effectively diagnose CRDS. Given the frequency of unexplained cardiac arrest, should these findings be confirmed in larger studies, this readily available maneuver may provide clinically actionable information. Importance: Sudden death and cardiac arrest frequently occur without explanation, even after a thorough clinical evaluation. Calcium release deficiency syndrome (CRDS), a life-threatening genetic arrhythmia syndrome, is undetectable with standard testing and leads to unexplained cardiac arrest. Objective: To explore the cardiac repolarization response on an electrocardiogram after brief tachycardia and a pause as a clinical diagnostic test for CRDS. Design, Setting, and Participants: An international, multicenter, case-control study including individual cases of CRDS, 3 patient control groups (individuals with suspected supraventricular tachycardia; survivors of unexplained cardiac arrest [UCA]; and individuals with genotype-positive catecholaminergic polymorphic ventricular tachycardia [CPVT]), and genetic mouse models (CRDS, wild type, and CPVT were used to define the cellular mechanism) conducted at 10 centers in 7 countries. Patient tracings were recorded between June 2005 and December 2023, and the analyses were performed from April 2023 to December 2023. Intervention: Brief tachycardia and a subsequent pause (either spontaneous or mediated through cardiac pacing). Main Outcomes and Measures: Change in QT interval and change in T-wave amplitude (defined as the difference between their absolute values on the postpause sinus beat and the last beat prior to tachycardia). Results: Among 10 case patients with CRDS, 45 control patients with suspected supraventricular tachycardia, 10 control patients who experienced UCA, and 3 control patients with genotype-positive CPVT, the median change in T-wave amplitude on the postpause sinus beat (after brief ventricular tachycardia at ≥150 beats/min) was higher in patients with CRDS (P <.001). The smallest change in T-wave amplitude was 0.250 mV for a CRDS case patient compared with the largest change in T-wave amplitude of 0.160 mV for a control patient, indicating 100% discrimination. Although the median change in QT interval was longer in CRDS cases (P =.002), an overlap between the cases and controls was present. The genetic mouse models recapitulated the findings observed in humans and suggested the repolarization response was secondary to a pathologically large systolic release of calcium from the sarcoplasmic reticulum. Conclusions and Relevance: There is a unique repolarization response on an electrocardiogram after provocation with brief tachycardia and a subsequent pause in CRDS cases and mouse models, which is absent from the controls. If these findings are confirmed in larger studies, this easy to perform maneuver may serve as an effective clinical diagnostic test for CRDS and become an important part of the evaluation of cardiac arrest. Case-control study including individual cases of calcium release deficiency syndrome (CRDS), 3 patient control groups, and genetic mouse models assesses the cardiac repolarization response on an electrocardiogram after brief tachycardia and a pause as a clinical diagnostic test for CRDS. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy (HCM) affects an estimated 1:500 individuals and is largely considered to be a disease of the sarcomere. However, disease-causative genetic variants in PLN -encoded phospholamban and JPH2 -encoded junctophilin-2 cause HCM via perturbations in intracellular calcium handling (ICH). Given recent case reports describing asymmetric left ventricular hypertrophy (LVH) in patients with arrhythmogenic cardiomyopathy (ACM)-predisposing genetic variants in PKP2 -encoded plakophilin-2, a gene known to play a role in ICH, we sought to examine the prevalence and clinical impact of an HCM phenotype in PKP2-ACM patients. In this retrospective study, review of 771 genotype-positive ACM patients evaluated between 01/2015 and 04/2024 was used to identify those with a pathogenic/likely pathogenic (P/LP) variant in PKP2. Pertinent demographic, electrocardiographic, imaging and outcome data was extracted from the electronic medical record. HCM was defined by the presence of maximal end-diastolic wall thickness of >15 mm anywhere in the left ventricle. Advanced heart failure (HF) and major VA outcomes [sudden cardiac arrest (SCA), sustained VT, and appropriate implantable cardioverter-defibrillator (ICD) shocks] were analyzed. Collectively, an HCM phenotype was observed in 9 (7.8%) of the 115 P/LP variant-positive patients (mean age 38±36; 51 (44%) male, and 57 with a ACM phenotype). Of note sigmoid subtype HCM was noted in 8/9 (89%) and left ventricular outflow tract obstruction present in 7/9 (78%; mean gradient of 57±48 mmHg). In comparison to PKP2-ACM patients without HCM, PKP2-HCM patients with HCM were older and less likely to have a family history of any cardiomyopathy (Table). Interestingly, in the patients with a positive phenotype, non-sustained VT and major VA outcomes were less prevalent in the HCM patients (Table). Nearly 8% of individuals with a P/LP variant in PKP2 have a concomitant HCM phenotype. Whether this observation reflects abnormal calcium handling in the setting of PKP2 haploinsufficiency or selection bias in the setting of the combined prevalence of HCM (1:500) and PKP2 loss-of-function (1:695) requires further studies. [ABSTRACT FROM AUTHOR]

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy. Mavacamten, the first-in-class drug approved by the FDA for the treatment of HCM, has shown efficacy in improving clinical symptoms. However, the impact of mavacamten on arrhythmic events remains inadequately explored. We previously reported an increase in atrial fibrillation risk in the weeks following mavacamten initiation and now seek to assess other arrhythmic events. This retrospective study included patients with HCM treated with mavacamten at a multi-site tertiary referral center. To assess the impact of mavacamten on the frequency of arrhythmic events, data from Holter monitoring was assessed at three time points: baseline (at treatment initiation), an intermediate follow-up, and the most recent follow-up available. The frequencies of premature ventricular contractions (PVCs), supraventricular premature contractions (SVPCs), supraventricular tachycardia (SVT), and non-sustained ventricular tachycardia (NSVT) were recorded and corrected for the total duration of Holter monitoring. The rates of arrhythmic events at different time points were analyzed using the Wilcoxon signed-rank test. Statistical significance was set at p<0.05. Eighteen patients were enrolled, 50% female, with median age 66.3 years (IQR 61.7-73 years). Median duration to intermediate follow-up was 3.7 months (IQR 0-7 months), while median duration to the most recent follow-up was 11.3 months (IQR 3-24 months). The number of SVT runs was higher during the intermediate and most recent follow-up periods compared to baseline (3 [0.12 – 6.3] and 1 [0 – 2.6] vs. 0.25 [0 – 3], respectively), however, these differences were not statistically significant (p = 0.753 and p = 0.645, respectively). Duration of SVT was significantly higher at the intermediate follow-up compared to baseline (10 [IQR 7-24] beats vs 1.5 [IQR 0-9] beats, p= 0.028). However, there was no significant difference in SVT duration between the most recent follow-up and baseline (4.5 [IQR 0-9.5] beats vs 1.5 [IQR 0-5.75] beats, p= 0.14). Rates of PVCs, SVPCs, and NSVT trended higher at intermediate follow-up but did not reach statistical significance. Conversely, at the most recent follow-up, the rates of all arrhythmic outcomes trended lower compared to baseline, but again lacked statistical significance. The data indicates an initial rise in arrhythmic events upon starting mavacamten treatment, followed by a subsequent decline. Despite the small sample size, these findings provide important insights into potential patterns of response to the medication. Larger studies are necessary to confirm these observations and deepen our understanding of mavacamten's impact on arrhythmic events. [ABSTRACT FROM AUTHOR]

Introduction Methods Results Conclusion Little is known regarding the prevalence and prognostic implications of non‐sustained ventricular tachycardia (NSVT) in patients with wild‐type transthyretin cardiac amyloidosis (ATTRwt‐CA). We aimed to investigate the prevalence of NSVT in patients with ATTRwr‐CA, and the association of NSVT with sustained ventricular arrhythmias (VA) and all‐cause mortality.In a cohort of ATTRwt‐CA patients from 2011 to 2022 without prior sustained VA, we ascertained the presence and characteristics of NSVT during clinically indicated ambulatory Holter monitoring. Patients were stratified based on the presence of NSVT at baseline. The primary and secondary endpoints of sustained VA and all‐cause mortality, respectively, were assessed during follow‐up.The cohort included 217 patients with ATTRwt‐CA (95% males, median age 75 years; median NYHA class 2). Baseline Holter monitoring demonstrated NSVT in 116 (53%) patients. During a median follow‐up of 27 months (IQR 16–45) after the index Holter monitor, 11 (5.1%) patients reached the primary endpoint of sustained VA (incidence 1.8 per 100 person‐years, all monomorphic VT), and 46 (21%) patients died. In univariable Cox proportional hazard model, NYHA class 3 or 4 heart failure (p = .048), the presence of NSVT (p = .04), the duration of longest NSVT run (p = .029), and the percentage of ventricular ectopy (p < .001) were associated with the primary outcome. When adjusting for age and NYHA class 3 or 4 heart failure, the presence of NSVT remained associated with the primary outcome (p = .03). All‐cause mortality was not significantly different between patients with and without NSVT. A cutoff for ventricular ectopy burden of 0.5%, the median for all patients in this study, was associated with increased risk in sustained VA (log‐rank p = .004) and mortality (log‐rank p = .02).NSVT is highly prevalent among ATTRwt‐CA patients undergoing clinically indicated Holter monitoring and may confer an increased risk of incident sustained VA. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis is a disease characterized by non-caseating granulomas and may affect any organ system. Cardiac involvement may lead to conduction abnormalities, heart failure, or malignant ventricular arrhythmias. As sarcoidosis may present with heterogeneous manifestations, a detailed past medical history may provide clues that help guide further workup. We present a rare case of a patient with undiagnosed oral sarcoidosis who subsequently experienced cardiac arrest from cardiac involvement., Case Summary: A 43-year-old male with a history of palpitations and periodontitis consistent with oral sarcoidosis presents after experiencing sudden cardiac arrest. He was subsequently diagnosed with cardiac and pulmonary sarcoidosis. With contemporary management (both immunosuppression and antiarrhythmics), he has not experienced any recurrent arrhythmias., Discussion: In the setting of cardiac arrest and non-ischaemic cardiomyopathy, a careful clinical history and targeted cardiac testing may help clinicians determine when to consider cardiac sarcoidosis as a diagnosis. While oral sarcoidosis is a very rare condition, this case highlights how infrequent manifestations of sarcoidosis may be encountered in the clinical setting., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Background and Aims: Current guidelines suggest that asymptomatic atrial fibrillation (AF) is independently associated with increased risks of stroke and mortality compared with symptomatic AF. Considering that recent investigations have provided conflicting results, the present study aimed to evaluate the association between symptom status and clinical outcomes in patients with AF., Methods: Medline, Cochrane Library, and Scopus were searched until 25 March 2024. Triple-independent study selection, data extraction and quality assessment were performed. Evidence was pooled using random-effects meta-analyses., Results: Thirty-six studies (217 850 participants) were included. Based on the frequentist analysis, symptomatic individuals had no significant difference in the risk of all-cause mortality [hazard ratio (HR) .97, 95% confidence interval (CI) .80-1.17], cardiovascular mortality (HR 1.04, 95% CI .72-1.49), thromboembolism (HR 1.06, 95% CI .87-1.28), stroke (HR 1.06, 95% CI .84-1.34), hospitalization (HR 1.34, 95% CI .89-2.02), and myocardial infarction (HR .98, 95% CI .70-1.36), compared to the asymptomatic group. Symptomatic patients had a 33% increased risk of new-onset heart failure (HR 1.33, 95% CI 1.19-1.49) and a 30% lower risk of progression to permanent AF (HR .70, 95% CI .54-.89). The Bayesian analysis yielded comparable results, yet the association between symptom status and new-onset heart failure was not significant (HR 1.27, 95% credible interval .76-1.93; Bayes factor = 1.2). Symptomatic patients had higher odds of receiving antiarrhythmic drugs (odds ratio [OR] 1.64, 95% CI 1.33-2.03) and ablation therapy (OR 1.47, 95% CI 1.06-2.05) compared to asymptomatic cases., Conclusions: The risk of major clinical outcomes did not differ between individuals with and without AF-related symptoms. Asymptomatic patients had a greater hazard of progression to permanent AF., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Background: Atrioventricular (AV) conduction ablation has been achieved by targeting the area of penetration of the conduction axis as defined by recording a His bundle potential. Ablation of the His bundle may reduce the possibility of a robust junctional escape rhythm. It was hypothesised that specific AV nodal ablation is feasible and safe., Methods: The anatomical position of the AV node in relation to the site of penetration of the conduction axis was identified as described in dissections and histological sections of human hearts. Radiofrequency (RF) ablation was accomplished based on the anatomical criteria., Results: Specific anatomical ablation of the AV node was attempted in 72 patients. Successful AV nodal ablation was accomplished in 63 patients (87.5%), following 60 minutes (IQR 50-70 minutes) of procedure time, 3.4 minutes (IQR 2.4-5.5 minutes) of fluoroscopy time, and delivery of 4 (IQR 3-6) RF lesions. An escape rhythm was present in 45 patients (71%), and the QRS complex was similar to that before ablation in all 45 patients. Atropine was administered in six patients after the 10-min waiting period and did not result in restoration of conduction. In nine patients, AV conduction could not be interrupted, and AV block was achieved with ablation of the His after delivery of 12 (IQR 8-15) RF lesions. No cases of sudden death were encountered, and all patients had persistent AV block during a median 10.5 months (IQR 5-14 months) of follow-up., Conclusion: Anatomical ablation of the AV node is feasible and safe, and results in an escape rhythm similar to that before ablation., Competing Interests: Disclosure: HC and RHA are the Section Editors, JEM, KCS are on the editorial board and DGK is Editor-in-chief of Arrhythmia & Electrophysiology Review, this did not influence peer review. All other authors have no conflicts of interest to declare., (Copyright © The Author(s), 2024. Published by Radcliffe Group Ltd.)

Background: Artificial intelligence (AI) is increasingly utilized in interventional cardiology (IC) and holds the potential to revolutionize the field., Methods: We conducted a global, web-based, anonymous survey of IC fellows and attendings to assess the knowledge and perceptions of interventional cardiologists regarding AI use in IC., Results: A total of 521 interventional cardiologists participated in the survey. The median age range of participants was 36 to 45 years, most (51.5%) practice in the United States, and 7.5% were women. Most (84.7%) could explain well or somehow knew what AI is about, and 63.7% were optimistic/very optimistic about AI in IC. However, 73.5% believed that physicians know too little about AI to use it on patients and most (46.1%) agreed that training will be necessary. Only 22.1% were currently implementing AI in their personal clinical practice, while 60.6% estimated implementation of AI in their practice during the next 5 years. Most agreed that AI will increase diagnostic efficiency, diagnostic accuracy, treatment selection, and healthcare expenditure, and decrease medical errors. The most tried AI-powered tools were image analysis (57.3%), ECG analysis (61.7%), and AI-powered algorithms (45.9%). Interventional cardiologists practicing in academic hospitals were more likely to have AI tools currently implemented in their clinical practice and to use them, women had a higher likelihood of expressing concerns regarding AI, and younger interventional cardiologists were more optimistic about AI integration in IC., Conclusions: Our survey suggests a positive attitude of interventional cardiologists regarding AI implementation in the field of IC.