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Background: Trajectories of mortality after primary prevention implantable cardioverter-defibrillator (ICD) placement for older patients with heart failure during or soon after acute hospitalization have not been assessed., Objective: The purpose of this study was to compare trajectories of mortality after primary prevention ICD placement during or soon after acute cardiac or non-cardiac hospitalization., Methods: We identified older patients with heart failure undergoing primary prevention ICD placement using 20% Medicare data (2008-2018). Placement settings were as follows: (1) Current-H-during current hospitalization, (2) Recent-H-within 90 days of hospitalization, or (3) Chronic stable. Hospitalization was categorized as cardiac vs non-cardiac. Interval mortality rates and hazard ratios (HRs) using Cox regression were estimated at 0-30, 31-90, and 91-365 days after ICD placement., Results: Of the 61,710 patients (mean age 76 years; 35% female; 85% white), 19% (11,947), 25% (15,147), and 56% (34,616) had ICDs in Current-H, Recent-H, and Chronic stable settings. Mortality rates (per 100 person-years) were highest during 0-30 days, with 38 (34-42) and 22 (19-24) for Current-H and Recent-H, which declined to 21 (20-22) and 16 (15-17) during 91-365 days, respectively. Compared to Chronic stable, HRs were highest during 0-30 days post-ICD placement (5.5 [4.5-6.8] for Current-H and 3.4 [2.8-4.2] for Recent-H) and decreased during 91-365 days (2.0 [1.8-2.1] for Current-H and 1.6 [1.5-1.7] for Recent-H). HR pattens were similar for cardiac and non-cardiac hospitalizations., Conclusion: Primary prevention ICD placement during or soon after hospitalization for any reason was associated with worse mortality with diminishing risks after 90 days. Hospitalization likely identifies a sicker population in whom early mortality with or without ICD may be higher. Our results support careful consideration regarding ICD placement during the 90 days after hospitalization., Competing Interests: Disclosures Dr Setoguchi discloses having received research funding from Pfizer Inc, Pfizer Japan, Bristol Myers Squibb, and Daiichi Sankyo and served as a consultant for Pfizer Japan and Merck Co. Inc. All other coauthors have no disclosures., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Sudden cardiac arrest (SCA) is a global health concern, imposing a substantial mortality burden. However, the understanding of the impact of various extreme temperature events, when accounting for the effect of daily average temperature on SCA, remains incomplete. Additionally, the assessment of SCA mortality burden associated with temperatures from an individual-level design is limited. This nationwide case-crossover study collected individual SCA death records across all (2844) county-level administrative units in the Chinese Mainland from 2013 to 2019. Four definitions for hot nights and ten for both cold spells and heatwaves were established using various temperature thresholds and durations. Conditional logistic regression models combined with distributed lag nonlinear models were employed to estimate the cumulative exposure-response relationships. Based on 887,662 SCA decedents, this analysis found that both hot nights [odds ratio (OR): 1.28; attributable fraction (AF): 1.32 %] and heatwaves (OR: 1.40; AF: 1.29 %) exhibited significant added effects on SCA mortality independent of daily average temperatures, while cold spells were not associated with an elevated SCA risk after accounting for effects of temperatures. Cold temperatures [below the minimum mortality temperature (MMT)] accounted for a larger mortality burden than high temperatures (above the MMT) [AF: 12.2 % vs. 1.5 %]. Higher temperature-related mortality risks and burdens were observed in patients who experienced out-of-hospital cardiac arrest compared to those with in-hospital cardiac arrest. This nationwide study presents the most compelling and comprehensive evidence of the elevated mortality risk and burden of SCA associated with extreme temperature events and ambient temperatures amid global warming., 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., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background: Because of differences in chest wall anatomy, female patients may have higher rates of subcutaneous implantable cardioverter-defibrillator (S-ICD) pocket-related complications., Objective: We sought to evaluate sex-based outcomes after S-ICD implantation., Methods: Patients implanted with an S-ICD at Emory Healthcare between 2010 and 2023 were included in the analysis. Patients' clinical characteristics and post-S-ICD implantation complications were collected., Results: There were 429 male patients (68%) and 199 female patients (32%) observed for a median duration of 2.3 years (0.6-4.4 years). Male and female patients had comparable rates of diabetes (28%), end-stage renal disease (29.5%), ejection fraction (30.2% ± 13.4%), and body mass index (29.1 ± 6.6 kg/m 2 ). There was no statistical difference in the incidence of shocks between men and women (26.3% vs 20.1%; P = .09), including appropriate shocks (14.7% vs 12%; P = .98) and inappropriate shocks (11.7% vs 9.5%; P = .98). Pocket-related complications occurred in 21 patients; these included pocket infection (n = 12), wound dehiscence (n = 7), and hematoma requiring drainage (n = 2). Female patients had a significantly higher pocket-related complication rate compared with male patients (7.2% vs 2.5%; P = .016). In controlling for age, body mass index, diabetes, and end-stage renal disease, female patients had higher odds of pocket-related complications compared with male patients (odds ratio, 3.15; 95% confidence interval, 1.27-7.75). Pocket-related complications decreased after 2018 compared with before 2018 (6% vs 2.6%, P = .052), mainly driven by reduction in complications in women (12.3% vs 3.2%; P = .034) but not in men (2.8% vs 2.4%; P = 1)., Conclusion: In this cohort of S-ICD patients, women had a higher rate of post-S-ICD pocket-related complications that could be explained by sex-based differences in anatomy., Competing Interests: Disclosures V.J. received funding from the Katz foundation. B.R.R. received funding from NIH and AHRQ. D.B.D. is a consultant for Boston Scientific, Abbott, and AtriCure. M.S.L. is a consultant for Medtronic and Boston Scientific. M.F.E. is a consultant for Medtronic and Boston Scientific., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: The 2014 Heart Rhythm Society consensus statement defines histological (definite) and clinical (probable) diagnostic categories of cardiac sarcoidosis (CS), but few studies have compared their arrhythmic phenotypes and outcomes., Objective: The purpose of this study was to evaluate the electrophysiological/arrhythmic phenotype and outcomes of patients with definite and probable CS., Methods: We analyzed the arrhythmic/electrophysiological phenotype in a single-center North American cohort of 388 patients (median age 56 years; 39% female, n = 151) diagnosed with definite (n = 58) or probable (n = 330) CS (2000-2022). The primary composite outcome was survival to first ventricular tachycardia/fibrillation (VT/VF) event or sudden cardiac death. Key secondary outcomes were also assessed., Results: At index evaluation, in situ cardiac implantable electronic devices and antiarrhythmic drug use were more common in definite CS. At a median follow-up of 3.1 years, the primary outcome occurred in 22 patients with definite CS (38%) and 127 patients with probable CS (38%) (log-rank, P = .55). In multivariable analysis, only a higher ratio of the 18 F-fluorodeoxyglucose maximum standardized uptake value of the myocardium to the maximum standardized uptake value of the blood pool (hazard ratio 1.09; 95% confidence interval 1.03-1.15; P = .003, per 1 unit increase) was associated with the primary outcome. During follow-up, patients with definite CS had a higher burden of device-treated VT/VF events (mean 2.86 events per patient-year vs 1.56 events per patient-year) and a higher rate of progression to heart transplant/left ventricular assist device implantation but no difference in all-cause mortality compared with patients with probable CS., Conclusion: Patients with definite and probable CS had similarly high risks of first sustained VT/VF/sudden cardiac death and all-cause mortality, though patients with definite CS had a higher overall arrhythmia burden. Both CS diagnostic groups as defined by the 2014 Heart Rhythm Society criteria require an aggressive approach to prevent arrhythmic complications., Competing Interests: Disclosures No relevant disclosures by any of the authors., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: The association between alcohol consumption and the risk of sudden cardiac death and/or fatal ventricular arrhythmia remains controversial., Objective: We analyzed the association between alcohol consumption, genetic traits for alcohol metabolism, and the risk of sudden cardiac death and/or fatal ventricular arrhythmia., Methods: We identified 397,164 individuals enrolled between 2006 and 2010 from the UK Biobank database and followed them until 2021. Alcohol consumption was categorized as current nondrinkers (nondrinkers and ex-drinkers), mild drinkers, moderate drinkers, or heavy drinkers. Genetic traits of alcohol metabolism were stratified according to the polygenic risk score tertiles. The primary and secondary outcomes were a composite of sudden cardiac death and fatal ventricular arrhythmia as well as their individual components., Results: During follow-up (median 12.5 years), 3543 cases (0.89%) of clinical outcomes occurred. Although mild, moderate, and heavy drinkers showed deceased risks of outcomes compared with current nondrinkers, there was no prognostic difference among nondrinkers, mild drinkers, moderate drinkers, and heavy drinkers. Ex-drinkers showed an increased risk in univariate analysis, but the significance was attenuated after adjusting covariates (hazard ratio 1.19; 95% confidence interval 0.94-1.50). As a continuous variable, alcohol consumption was not associated with clinical outcomes (hazard ratio 1.01; 95% confidence interval 0.99-1.02). Consistent with these findings, there was no association between genetic traits for alcohol metabolism and the risk of clinical outcomes., Conclusion: Alcohol consumption was neither a protective factor nor a risk factor for sudden cardiac death or fatal ventricular arrhythmia. Genetic traits of alcohol metabolism were not associated with the clinical prognosis., Competing Interests: Disclosures Dr Choi received research grants from Bayer, BMS/Pfizer, Biosense Webster, Chong Kun Dang, Daiichi-Sankyo, Samjin Pharmaceutical, Sanofi-Aventis, Seers Technology, Skylabs, and Yuhan. The other authors have nothing to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Current cohorts of patients with idiopathic ventricular fibrillation (IVF) primarily include adult-onset patients. Underlying causes of sudden cardiac arrest vary with age; therefore, underlying causes and disease course may differ for adolescent-onset vs adult-onset patients., Objective: The purpose of this study was to compare adolescent-onset with adult-onset patients having an initially unexplained cause of VF., Methods: The study included 39 patients with an index event aged ≤19 years (adolescent-onset) and 417 adult-onset patients from the Dutch Idiopathic VF Registry. Data on event circumstances, clinical characteristics, change in diagnosis, and arrhythmia recurrences were collected and compared between the 2 groups., Results: In total, 42 patients received an underlying diagnosis during follow-up (median 7 [2-12] years), with similar yields (15% adolescent-onset vs 9% adult-onset; P = .16). Among the remaining unexplained patients, adolescent-onset patients (n = 33) had their index event at a median age of 17 [16-18] years, and 72% were male. The youngest patient was aged 13 years. In comparison with adults (n = 381), adolescent-onset patients more often had their index event during exercise (P <.01). Adolescent-onset patients experienced more appropriate implantable cardioverter-defibrillator (ICD) therapy during follow-up compared with adults (44% vs 26%; P = .03). Inappropriate ICD therapy (26% vs 17%; P = .19), ICD complications (19% vs 14%; P = .41), and deaths (3% vs 4%; P = 1) did not significantly differ between adolescent-onset and adult-onset patients., Conclusion: IVF may occur during adolescence. Adolescent-onset patients more often present during exercise compared with adults. Furthermore, they are more vulnerable to ventricular arrhythmias as reflected by a higher incidence of appropriate ICD therapy., Competing Interests: Disclosures Dr Yap is a consultant for Boston Scientific; and has received lecture fees and research grants from Medtronic, Biotronik, and Boston Scientific. All other authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Sudden arrhythmic death syndrome (SADS), characterized by an unknown or inconclusive cause of death at autopsy together with a negative or nonlethal toxicology screening result, is the most common cause of sudden cardiac death in victims younger than 35 years. The complete causality of SADS remains unclear, with drugs being a potential risk factor., Objective: This study aimed to describe the toxicologic profiles of SADS victims, focusing on proarrhythmic drugs, drug levels, and polypharmacy., Methods: All deaths in Denmark of those aged 1-35 years in 2000-2019 and 36-49 years in 2007-2019 were examined through death certificates, national registries, and autopsy reports with toxicology screenings. We investigated all sudden unexpected death victims with an autopsy performed, including negative or nonlethal drug findings, where cause of death was unknown or inconclusive (SADS)., Results: We identified 477 SADS victims; 313 (66%) had a positive toxicology screening result (adjudicated nonlethal), with an average of 2.8 drugs per case. More than half of the SADS victims with a positive toxicology screening result had QT-prolonging or brugadogenic drugs present. Polypharmacy was present in 66%, psychotropic polypharmacy in 37%, and QT-prolonging polypharmacy in 22%, with the most frequent overall and QT-prolonging drug combination being an antipsychotic and a psychoanaleptic drug. QT-prolonging drugs were more often present at suprapharmacologic levels than non-QT-prolonging drugs., Conclusion: The majority of the SADS population had a positive toxicology screening result, with a notably large proportion having proarrhythmic drugs and polypharmacy. This highlights the need for future focus on drugs as a risk factor for SADS., Competing Interests: Disclosures J.T.-H. reports relationships with Johnson & Johnson, MicroPort, Boston Medical Products, Cytokinetics, and Leo Pharma., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background and Aims: Vitamin D is known to influence the risk of cardiovascular disease, which is a recognized risk factor for sudden cardiac arrest (SCA). However, the relationship between vitamin D and SCA is not well understood. Therefore, this study aims to investigate the association between vitamin D and SCA in out-of-hospital cardiac arrest (OHCA) patients compared to healthy controls., Methods and Results: Using the Phase II Cardiac Arrest Pursuit Trial with Unique Registration and Epidemiologic Surveillance (CAPTURES II) registry, a 1:1 propensity score-matched case-control study was conducted between 2017 and 2020. Serum 25-hydroxyvitamin D (vitamin D) levels in patients with OHCA (454 cases) and healthy controls (454 cases) were compared after matching for age, sex, cardiovascular risk factors, and lifestyle behaviors. The mean vitamin D levels were 14.5 ± 7.6 and 21.3 ± 8.3 ng/mL among SCA cases and controls, respectively. Logistic regression analysis was used adjusting for cardiovascular risk factors, lifestyle behaviors, corrected serum calcium levels, and estimated glomerular filtration rate (eGRF). The adjusted odds ratio (aOR) for vitamin D was 0.89 (95% confidence interval [CI] 0.87-0.91). The dose-response relationship demonstrated that vitamin D deficiency was associated with SCA incidence (severe deficiency, aOR 10.87, 95% CI 4.82-24.54; moderate deficiency, aOR 2.24, 95% CI 1.20-4.20)., Conclusion: Vitamin D deficiency was independently and strongly associated with an increased risk of SCA, irrespective of cardiovascular and lifestyle factors, corrected calcium levels, and eGFR., (Copyright © 2024 The Italian Diabetes Society, the Italian Society for the Study of Atherosclerosis, the Italian Society of Human Nutrition and the Department of Clinical Medicine and Surgery, Federico II University. Published by Elsevier B.V. All rights reserved.)

Background: Sports medical examinations are a tool to detect potentially life-threatening situations during physical activity, but their usefulness and protocols in young athletes remain controversial. The aim was to describe the diagnostic performance of a 2-step ECG and echocardiogram screening model in a large sample of healthy young-adolescent athletes (aged 11-16 years), evaluating its cost-effectiveness for cardiovascular disease (CVD) detection., Methods: Cross-sectional study of 2617 individuals (81 % male, mean age 15 years), over 20 federated sports (La Rioja-Spain). A cardiological examination included family and personal history, physical examination, ECG, and transthoracic echocardiogram. The previous ECG, conducted at 11-13 years old, was reviewed in 1589 individuals (60 %). ECGs were interpreted according to 2017 international criteria. The prevalence of structural cardiac disease (CD) was investigated, and the diagnostic performance of both tests and the cost of screening was evaluated., Results: CVD was diagnosed in 63 athletes (2.4 %) and 16 (0.6 %) with pathology related to sudden cardiac death (SCD). In 3 cases (0.11 %), cessation of sporting activity was indicated, and in 2 cases (0.07 %) treatment for congenital CD was indicated. ECG alterations were infrequent (2.5 %). Eighty percent of the structural CD diagnosed by echocardiogram had a normal ECG. The cost per CVD detected was €3,080, and for CVD associated with SCD, it was €12,323., Conclusions: Our study shows the diagnostic cost-effectiveness of a two-step cardiac screening protocol, including ECG, and highlights the role of echocardiography in young adolescent athletes, which could be implemented at a low and reasonable cost., 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., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Despite being extremely rare in children, primary benign cardiac tumors can cause malignant ventricular arrhythmias (VA) or even sudden cardiac death. To assess the predictors of cardiovascular death and malignant VAs, we designed a retrospective single-center study enrolling paediatric patients. We defined as primary outcome a composite of cardiovascular death, sustained VT, ventricular fibrillation and rapid, symptomatic non-sustained VT. Our secondary endpoint was to assess the prevalence of clinically significant arrhythmias in our population. METHODS AND RESULTS: We fitted a multivariate Cox regression model to assess the predictors of the primary outcome. Over a period of 38 years, a total of 97 children were enrolled in the study. Among them, there were 73 rhabdomyomas, 13 fibromas, 3 myxomas, 3 teratomas, 1 lipoma, 2 haemangiomas and 2 fibroelastomas. Over a median follow up of 10.53 years, 16 patients met the primary outcome. Kaplan Meier unadjusted survival estimates showed that tumor dimensions larger than 2.3 cm and diagnosis of fibroma predicted worse outcomes compared with smaller tumors or other histotypes, (log rank p < 0.0002 and < 0.0001 respectively). In multivariate Cox proportional hazards analysis, diagnosis of fibroma and tumor dimensions were independently associated to the primary endpoint (HR: 5.06, 95 %CI (1.3-19); and 1.26 • (1.05-11), respectively). Clinically significant arrhythmias were reported in 24.5 % of the study population. CONCLUSIONS: Among paediatric primary cardiac tumors, type and dimensions may predict the hazard of malignant VAs and cardiac death., Competing Interests: Declaration of competing interest The Authors declare no conflict of interest., (Copyright © 2024. Published by Elsevier B.V.)

Background: In patients with Hypertrophic Cardiomyopathy (HCM) S-ICD is usually the preferred option as pacing is generally not indicated. However, limited data are available on its current practice adoption and long-term follow-up., Methods: Consecutive HCM patients with S-ICD implanted between 2013 and 2021 in 3 international centers were enrolled in this observational study. Baseline, procedural and follow-up data were regularly collected. Efficacy and safety were compared with a cohort of HCM patients implanted with a tv-ICD., Results: Seventy patients (64% males) were implanted with S-ICD at 41 ± 15 years, whereas 168 patients with tv-ICD at 49 ± 16 years. For S-ICD patients, mean ESC SCD risk score was 4,5 ± 1.9%: 25 (40%) at low-risk, 17 (27%) at intermediate and 20 (33%) at high-risk. Patients were followed-up for 5.1 ± 2.3 years. Two patients (0.6 per 100-person-years, vs 0.4 per 100 person-years with tv-ICD, p = 0.45) received an appropriate shock on VF, 17 (24%) were diagnosed with de-novo AF. Inappropriate shocks occurred in 4 patients (1.2 per 100-person-years, vs 0.9 per 100 person-years with tv-ICD, p = 0.74), all before Smart-Pass algorithm implementation. Four patients experienced device-related adverse events (1.2 per 100-person-years, vs 1 per 100 person-years with tv-ICD, p = 0.35%)., Conclusions: S-ICDs were often implanted in patients with an overall low-intermediate ESC SCD risk, reflecting both the inclusion of additional risk markers and a lower decision threshold. S-ICDs in HCM patients followed for over 5 years showed to be effective in conversion of VF and safe. Greater scrutiny may be required to avoid overtreatment in patients with milder risk profiles., Competing Interests: Conflict of interest The authors report no conflict of interest regarding the topic discussed in the present manuscript. The study was supported by an educational grant from Boston Scientific, USA. Disclosures: Dr. I. Olivotto has received grants from Brystol Myers Squibb, Cytokinetics, Amicus, Genzyme, Shire, Bayer, Boston Scientific, Menarini International, and fees (honoraria or consulting) from Bristol Myers Squibb, Cytokinetics, Amicus, Genzyme, Shire, Boston Scientific, and served or currently serving as PI on EXPLORER-HCM, MAVA-LTE, REDWOOD-HCM, REDWOOD-OLE, and SEQUOIA-HCM trial. Dr. F. Cecchi received consultancy fees from Pfizer and Bristol Myers Squibb. Dr. L. Crotti and Dr. N. Maurizi received consultancy fees from Bristol Myers Squibb., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Background: Data on the prognostic significance of temporal variability of spatial heterogeneity of electrocardiographic repolarization in coronary artery disease (CAD) are limited., Objective: The purpose of this study was to evaluate the prognostic value of temporal variability of T-wave morphology analyzed from a 5-minute resting electrocardiogram in CAD., Methods: The standard deviation (SD) of T-wave morphology dispersion (TMD-SD) and the SD of total cosine R-to-T were analyzed on a beat-to-beat basis from a 5-minute period of the standard resting 12-lead electrocardiogram obtained before the clinical stress test in 1702 patients with angiographically verified CAD and well-preserved left ventricular function., Results: During an average of 8.7 ± 2.2 years of follow-up, 60 patients experienced sudden cardiac death/arrest (SCD/SCA) (3.5%), 69 patients nonsudden cardiac death (NSCD) (4.1%), and 161 patients noncardiac death (9.5%). TMD-SD was significantly higher in patients who experienced SCD/SCA than in other patients (1.72 ± 2.00 vs 1.12 ± 1.75; P = .01) and higher in patients who succumbed to NSCD than in other patients (1.57 ± 1.74 vs 1.12 ± 1.76; P = .04), but it did not differ significantly between patients who experienced noncardiac death and those without such an event (1.16 ± 1.42 vs 1.14 ± 1.79; P = .86). In the Cox multivariable hazards model, TMD-SD retained its significant association with the risk of SCD/SCA (hazard ratio 1.119; 95% confidence interval 1.015-1.233; P = .024) but not with the risk of NSCD (hazard ratio 1.089; 95% confidence interval 0.983-1.206; P = .103)., Conclusion: TMD-SD is independently associated with the long-term risk of SCD/SCA in patients with CAD., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Energy drinks potentially can trigger life-threatening cardiac arrhythmias. It has been postulated that the highly stimulating and unregulated ingredients alter heart rate, blood pressure, cardiac contractility, and cardiac repolarization in a potentially proarrhythmic manner., Objective: The purpose of this study was to describe our experience regarding sudden cardiac arrest (SCA) occurring in proximity to energy drink consumption in patients with underlying genetic heart diseases., Methods: The electronic medical records of all SCA survivors with proven arrhythmias referred to the Mayo Clinic Windland Smith Rice Genetic Heart Rhythm Clinic for evaluation were reviewed to identify those who consumed an energy drink before their event. Patient demographics, clinical characteristics, documented energy drink consumption, and temporal relationship of energy drink consumption to SCA were obtained., Results: Among 144 SCA survivors, 7 (5%; 6 female; mean age at SCA 29 ± 8 years) experienced an unexplained SCA associated temporally with energy drink consumption. Of these individuals, 2 had long QT syndrome and 2 had catecholaminergic polymorphic ventricular tachycardia; the remaining 3 were diagnosed with idiopathic ventricular fibrillation. Three patients (43%) consumed energy drinks regularly. Six patients (86%) required a rescue shock, and 1 (14%) was resuscitated manually. All SCA survivors have quit consuming energy drinks and have been event-free since., Conclusion: Overall, 5% of SCA survivors experienced SCA in proximity to consuming an energy drink. Although larger cohort studies are needed to elucidate the incidence/prevalence and quantify its precise risk, it seems prudent to sound an early warning on this potential risk., (Copyright © 2024. Published by Elsevier Inc.)

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition with a prevalence ranging from 1 in 2000 to 1 in 5000 individuals. ARVC is a significant contributor to sudden cardiac death, particularly in young individuals and athletes, and remains challenging to diagnose definitively. We conducted a single-center retrospective study to evaluate the presentations, electrocardiogram findings, and imaging characteristics of ARVC patients evaluated at our center between 2021 and 2023. Notably, our study is the second investigation of ARVC conducted in Pakistan. We report divergent symptom prevalence as compared to the current literature and have incorporated the Task Force Criteria. Despite limited access to cardiac magnetic resonance (CMR) facilities worldwide, our findings underscore the critical role ofCMR in ARVC diagnosis. Our cohort had a mortality rate of 17 % highlighting the importance of early detection and the need for improved diagnostic facilities for ARVC in the region., Competing Interests: Declaration of competing interest None to declare, (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Hypertrophic cardiomyopathy is a condition associated with an increased risk of sudden death compared to the general population. Extended septal myectomy surgery has been suggested to impact the reduction of sudden death events according to various publications. The aim of this study was to assess changes in the prevalence of sudden death predictors in a population of patients undergoing extended septal myectomy surgery., Methods: Ninety-four consecutive patients underwent extended septal myectomy surgery due to symptomatic hypertrophic cardiomyopathy. Risk factors for sudden death, as defined by the American Heart Association and the European Society of Cardiology, were evaluated before and three months after surgery., Results: The mean age of the population was 57 ± 13 years. A significant reduction was observed in the maximum septal thickness from 21.3 to 14 mm (p<0.001), along with a decrease in the anteroposterior diameter of the left atrium from 51 to 47 mm (p=0.021). Resting intraventricular gradients decreased from 49.2 to 6.4 mmHg (p<0.001), and Valsalva-induced gradients decreased from 93.9 to 8.7 mmHg (p<0.001). Non-sustained ventricular tachycardia decreased from 6% to 2% (p<0.001), and atrial fibrillation decreased from 30% to 15% (p<0.001). Ischemic behavior during exercise stress echo decreased from 6% to 0%, and the European Society of Cardiology sudden death risk score reduced from 3.32 to 1.44 (p<0.001)., Conclusions: In this cohort of hypertrophic cardiomyopathy patients, extended septal myectomy surgery was associated with a reduction in the number and magnitude of sudden death predictors, potentially explaining the reduced mortality reported in the literature., 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., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Risk assessment tools and effective prevention strategies for sudden cardiac death (SCD) in pediatric patients with hypertrophic cardiomyopathy (HCM) have not been established. This study aimed to evaluate the efficacy of beta-blockers and exercise restriction for SCD prevention in this population., Methods: We retrospectively reviewed the medical records of patients aged <18 years who were diagnosed with HCM at our center between January 1996 and December 2021. SCD and aborted SCD were defined as SCD equivalents. We divided patients based on whether they were prescribed beta-blockers or exercise restriction and compared the outcomes among the groups. The primary outcome was the overall survival (OS), and the secondary outcome was the cumulative SCD equivalent rate. Outcomes were analyzed using Kaplan-Meier curves and Cox proportional hazard analysis. We also compared patients according to the occurrence of SCD equivalents to identify SCD risk predictors., Results: Among the 43 included patients [mean age, 7.7 (1.6-12.1) years; 23 male individuals], SCD equivalents occurred in 13 patients over 11.2 (4.5-15.6) years of follow-up, among whom 12 were resuscitated and 1 died. The OS rate was significantly higher in the beta-blocker and exercise restriction groups than in the non-beta-blocker and non-exercise restriction groups (81.3 % vs. 19.1 %, p < 0.01 and 57.4 % vs. 12.7 %, p < 0.01, respectively). Among the 13 patients with SCD equivalents, 5 had 9 recurrent SCD equivalents. A significant difference was observed between the SCD equivalent and non-SCD equivalent groups in the history of suspected arrhythmogenic syncope (p < 0.01) in the univariable but not in the multivariable analysis., Conclusions: Beta-blockers and exercise restriction may decrease the risk of SCD in pediatric patients with HCM and should be considered for SCD prevention in this population, particularly because predicting SCD in these patients remains challenging., Competing Interests: Declaration of competing interest The authors declare that there is no conflict of interest., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background-Aims: Sudden death in a young adult who showed no prodrome or complaint during his lifetime is a tragedy. The death often remains unexplained by doctors and is often the subject of a judicial investigation following which an autopsy is ordered. Our study joins several studies around the world, where the results have linked sudden death in adults to a cardiac origin., Methods: Through a series of 305 autopsies carried out in the forensic medicine department of the Frantz Fanon hospital in the city of Bejaia in Algeria over a period of two years, 57 cases corresponded to unexplained sudden deaths, i.e. an incidence of 3 cases per 100,000 inhabitants per year., Results: Sudden death was of cardiac origin in 50.8% of cases (N=28). Two epidemiologic profiles emerge in our study: the first is that of a man aged between 50 and 60 years of age, with several deleterious lifestyle habits (in particular smoking) with a cardiovascular history, previously followed by a cardiologist, who died suddenly out-of-hospital, from ischemic heart disease. The second is that of a young adult under 40 years of age, of average build, with no particular medical history, having not previously consulted a cardiologist, who died suddenly of hypertrophic cardiomyopathy., Conclusions: In many instances, we observed major anatomical lesion, which had not motivated any prior medical consultation either with a general practitioner or with a cardiologist., Competing Interests: Déclaration de liens d'intérêts Les auteurs déclarent ne pas avoir de liens d'intérêts., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Background: There are limited real-world data on the extended prognosis of patients with drug-induced type 1 Brugada electrocardiogram (ECG)., Objective: We assessed the clinical outcomes and predictors of life-threatening arrhythmias in patients with drug-induced type 1 Brugada ECG., Methods: This multicenter retrospective study, conducted at 21 Italian and Swiss hospitals from July 1997 to May 2021, included consecutive patients with drug-induced type 1 ECG. The primary outcome, a composite of appropriate ICD therapies and sudden cardiac death, was assessed along with the clinical predictors of these events., Results: A total of 606 patients (mean age 49.7 ± 14.7 years; 423 [69.8%] men) were followed for a median of 60.3 months (interquartile range 23.0-122.4 months). Nineteen patients (3.1%) experienced life-threatening arrhythmias, with a median annual event rate of 0.5% over 5 years and 0.25% over 10 years. The SCN5A mutation was the only predictor of the primary outcome (hazard ratio 4.54; P = .002), whereas a trend was observed for unexplained syncope (hazard ratio 3.85; P = .05). In patients who were asymptomatic at presentation, the median annual rate of life-threatening arrhythmias is 0.24% over 5 years and increases to 1.2% if they have inducible ventricular fibrillation during programmed ventricular stimulation., Conclusion: In patients with drug-induced type 1 Brugada ECG, the annual risk of life-threatening arrhythmias is low, with the SCN5A mutation as the only independent predictor. Unexplained syncope correlated with worse clinical outcomes. Ventricular fibrillation inducibility at programmed ventricular stimulation significantly increases the median annual rate of life-threatening arrhythmias from 0.24% to 1.2% over 5 years., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

The American approach to predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy diverges from the European method in that it relies on major risk factors independently justifying the implantation of an implantable cardioverter-defibrillator for primary prevention, whereas the European approach uses a mathematical equation to estimate a 5-year risk percentage. The aim of this review is to outline the differences between the American and European guidelines and to show how they have arisen. Furthermore, it will provide insight into the future of SCD risk prediction in patients with hypertrophic cardiomyopathy. The American SCD risk prediction method has high sensitivity but limited specificity, whereas the European method has the opposite. These differences in sensitivity and specificity likely contribute to the fact that primary prevention implantable cardioverter-defibrillator utilization is twofold higher in the United States. It is highly likely that new insights and new imaging modalities will enhance prediction models in the near future. Genotyping could potentially assume a significant role. Left ventricular global longitudinal strain was recently shown to be an independent predictor of SCD. Furthermore, after late gadolinium enhancement, additional cardiac magnetic resonance techniques such as T1 mapping and diffusion tensor imaging are showing encouraging outcomes in predicting SCD. Ultimately, it is conceivable that integrating diverse morphological and genetic characteristics through deep learning will yield novel insights and enhance SCD prediction methods., (Copyright © 2024 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

The use of subcutaneous ICDs (S-ICD) is growing over years despite increasing alerts on premature battery depletion (PBD) and lead fractures leading to unanticipated device replacements. In our single-centre study including 192 patients, per year analysis demonstrated that incidence of PBD is higher than previously reported with overall greatest battery replacement requirements around the fifth year of follow-up. The underlying issue appears to be limited to old series devices, but only a longer follow-up will clarify the real impact of this phenomenon on patient outcomes. PBD is an underestimated S-ICDs issue and if the hereby demonstrated trend were to be confirmed in latest device series, this would bring significant concerns to patient safety and huge economic expense to health system., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Outcome comparisons among subcutaneous implantable cardioverter-defibrillator (S-ICD) recipients with nonischemic cardiomyopathies are scarce., Objective: The aim of this study was to evaluate differences in device-related outcomes among S-ICD recipients with different structural substrates., Methods: Patients enrolled in the i-SUSI (International SUbcutaneouS Implantable cardioverter defibrillator registry) project were grouped according to the underlying substrate (ischemic vs nonischemic) and subgrouped into dilated cardiomyopathy, hypertrophic cardiomyopathy, Brugada syndrome (BrS), arrhythmogenic right ventricular cardiomyopathy (ARVC). The main outcome of our study was to compare the rates of appropriate and inappropriate shocks and device-related complications., Results: Among 1698 patients, the most common underlying substrate was ischemic (31.7%), followed by dilated cardiomyopathy (20.5%), BrS (10.8%), hypertrophic cardiomyopathy (8.5%), and ARVC (4.4%). S-ICD for primary prevention was more common in the nonischemic cohort (70.9% vs 65.4%; P = .037). Over a median (interquartile range) follow-up of 26.5 (12.6-42.8) months, no differences were observed in appropriate shocks between ischemic and nonischemic patients (4.8%/y vs 3.9%/y; log-rank, P = .282). ARVC (9.0%/y; hazard ratio [HR] 2.492; P = .001) and BrS (1.8%/y; HR 0.396; P = .008) constituted the groups with the highest and lowest rates of appropriate shocks, respectively. Device-related complications did not differ between groups (ischemic: 6.4%/y vs nonischemic: 6.1%/y; log-rank, P = .666), nor among underlying substrates (log-rank, P = .089). Nonischemic patients experienced higher rates of inappropriate shocks than did ischemic S-ICD recipients (4.4%/y vs 3.0%/y; log-rank, P = .043), with patients with ARVC (9.9%/y; P = .001) having the highest risk, even after controlling for confounders (adjusted HR 2.243; confidence interval 1.338-4.267; P = .002)., Conclusion: Most S-ICD recipients were primary prevention nonischemic cardiomyopathy patients. Among those, patients with ARVC tend to receive the most frequent appropriate and inappropriate shocks and patients with BrS the least frequent appropriate shocks., Competing Interests: Disclosures Dr Gasperetti has received consultant and speaker fees from Boston Scientific. Dr Martinek received speaker’s honoraria from Boston Scientific. Dr Kaiser worked as a proctor for Boston Scientific. Dr Tilz is a consultant for Boston Scientific and received speaker’s honoraria from Boston Scientific. Dr Tondo is a member of Boston Scientific Advisory Board. The other authors do not report disclosures regarding this article., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Sudden death is the leading cause of mortality in medically refractory epilepsy. Middle-aged persons with epilepsy (PWE) are under investigated regarding their mortality risk and burden of cardiovascular disease (CVD)., Methods: Using UK Biobank, we identified 7786 (1.6%) participants with diagnoses of epilepsy and 6,171,803 person-years of follow-up (mean 12.30 years, standard deviation 1.74); 566 patients with previous histories of stroke were excluded. The 7220 PWE comprised the study cohort with the remaining 494,676 without epilepsy as the comparator group. Prevalence of CVD was determined using validated diagnostic codes. Cox proportional hazards regression was used to assess all-cause mortality and sudden death risk., Results: Hypertension, coronary artery disease, heart failure, valvular heart disease, and congenital heart disease were more prevalent in PWE. Arrhythmias including atrial fibrillation/flutter (12.2% vs 6.9%; P < 0.01), bradyarrhythmias (7.7% vs 3.5%; P < 0.01), conduction defects (6.1% vs 2.6%; P < 0.01), and ventricular arrhythmias (2.3% vs 1.0%; P < 0.01), as well as cardiac implantable electric devices (4.6% vs 2.0%; P < 0.01) were more prevalent in PWE. PWE had higher adjusted all-cause mortality (hazard ratio [HR], 3.9; 95% confidence interval [CI], 3.01-3.39), and sudden death-specific mortality (HR, 6.65; 95% CI, 4.53-9.77); and were almost 2 years younger at death (68.1 vs 69.8; P < 0.001)., Conclusions: Middle-aged PWE have increased all-cause and sudden death-specific mortality and higher burden of CVD including arrhythmias and heart failure. Further work is required to elucidate mechanisms underlying all-cause mortality and sudden death risk in PWE of middle age, to identify prognostic biomarkers and develop preventative therapies in PWE., (Copyright © 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: Patients ≥ 80 years of age are underrepresented in major implantable cardioverter-defibrillator (ICD) trials, and real-world data are lacking. In this study, we sought to assess ICD utilisation, outcomes, and their predictors, in an unselected statewide population including patients ≥ 80 years old., Methods: We extracted details of ICDs implanted from 2009 to 2018 in New South Wales (NSW), Australia from the Centre for Health Record Linkage administrative data sets. Analysis was stratified into age groups of < 60 years, 60-79 years, and ≥ 80 years., Results: A total of 9304 patients (mean age 66.1 ± 13.1 years; 12.1% ≥ 80 years) had de novo ICD placement at an average rate of 1163 ± 122 patients per annum, with more implants in men in all age groups. After adjusting for NSW population size by sex, age group, and calendar year, mean implantation rates were 5.5 ± 0.6, 63.2 ± 8.6, and 52.7 ± 10.8 per 100,000 persons per annum in patients aged < 60 years, 60-79 years, and ≥ 80 years, respectively. In-hospital mortality was 0.4% and did not differ among age groups. However, 1-year mortality was 2.1%, 5.9%, and 10.7%, in those < 60 years, 60-79 years, and ≥ 80 years of age, respectively (P < 0.001), with hazard ratios for those aged ≥ 80 years of 4.3 (95% confidence interval [CI] 3.1-6.0) and those aged 60-79 years of 2.6 (95% CI 1.9-3.5) relative to those aged < 60 years (both P < 0.001) after adjusting for ICD indications, sex, implantation year, referral source, and comorbidities. In those aged ≥ 80 years, age > 83 years, congestive cardiac failure, chronic renal failure, neurodegenerative disease, and a higher Charlson comorbidity index score were each independent predictors of 1-year mortality., Conclusions: ICD use in patients aged ≥ 80 years and 60-79 years was 10-fold that in patients aged < 60 years, and perioperative outcomes were good in all ages, but there was substantially increased 1-year mortality in those aged ≥ 80 years. Careful selection based on age and comorbidity may further reduce 1-year mortality in patients ≥ 80 years old receiving ICDs., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Case reports suggest that quetiapine or haloperidol use is associated with severe QT prolongation (SQTP) and torsades de pointes., Objective: The purpose of this study was to examine the incidences, risk factors, and outcomes of SQTP in quetiapine and haloperidol users., Methods: This study accessed electronic medical records from a multicenter health-care hospital system in Taiwan and included patients who received quetiapine or haloperidol therapy and had both baseline and follow-up electrocardiograms. SQTP was defined as a posttreatment corrected QT (QTc) interval exceeding 500 ms or an increase in QTc interval of >60 ms compared with the baseline value. We analyzed the risk factors and outcomes of SQTP using multivariate logistic regression., Results: Mean increases in QTc interval were +8.3 ± 51.8 and +8.9 ± 44.0 ms after the administration of quetiapine (n = 8832) and haloperidol (n = 2341). Among these users, 1149 (13.0%) and 333 (14.2%) developed SQTP, respectively. Common risk factors for SQTP included old age, heart failure, hypokalemia, amiodarone use, and baseline QTc interval. SQTP in quetiapine users was significantly associated with ventricular arrhythmias (odds ratio 2.84; 95% confidence interval 1.95-4.13) and sudden cardiac death (odds ratio 2.29; 95% confidence interval 1.44-3.66)., Conclusion: More than 10% of patients receiving quetiapine or haloperidol therapy developed SQTP, and many of them were exposed to risk factors for SQTP. SQTP in quetiapine users was significantly associated with increased risks of ventricular arrhythmias and sudden cardiac death. Clinicians should be vigilant for ventricular arrhythmias in quetiapine users who have risk factors for SQTP., Competing Interests: Disclosures All authors have no conflicts to disclose., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Ischemic and nonischemic cardiomyopathy (NICM) are one of the leading causes of sudden cardiac death (SCD). Evidence supporting Implantable Cardioverter Defibrillator (ICD) for the prevention of SCD and mortality has shown conflicting results to date., Objective: We aim to evaluate the impact of ICD therapy with conventional care for the primary prevention of death of various causes in adults with ICM and NICM., Methods: We performed a systematic literature search on the electronic database for relevant articles from inception until 30th May 2023. Pooled odds ratios (OR) were calculated using a random effect model, and a p-value of <0.05 was considered statistically significant., Results: A total of 13 randomized controlled trials involving 7857 patients were included in the study. Pooled analysis showed that ICD therapy was associated with a significant reduction in the incidence of all-cause mortality (OR, 0.69 (95%CI:0.55-0.87), P = 0.001), with a similar trend among ICM and NICM compared with the control group. ICD therapy also reduces the incidence of SCD (OR, 0.32(95%CI: 0.24-0.43), P<0.00001) with a similar trend in ICM and NICM, as well as death due to arrhythmia (OR, 0.35(95%CI: 0.19-0.64), P<0.001). However, the incidence of cardiovascular mortality in the ICD group (OR, 0.77(95%CI: 0.58-1.02), P=0.07) was comparable to the control group., Conclusion: ICD therapy was associated with a reduction in the incidence of all-cause mortality, sudden cardiac death, and death due to arrhythmia among ischemic and nonischemic cardiomyopathy patients., Competing Interests: Declaration of Competing Interest Vikash Jaiswal serves as social media editor in the European Heart Journal Imaging Method and Practice, and Associate Editor in the European Journal of Medical Research (Cardiology Section), (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Several investigations have shown that existing risk stratification processes remain insufficient for stratifying sudden cardiac death risk in arrhythmogenic right ventricular cardiomyopathy (ARVC). Multiple auxiliary parameters are investigated to offer a more precise prognostic model. Our aim was to assess the association between several ECG markers (epsilon waves, prolonged terminal activation duration (TAD) of QRS, fragmented QRS (fQRS), late potentials on signal-averaged electrocardiogram (SA-ECG), T-wave inversion (TWI) in right precordial leads, and extension of TWI in inferior leads) with the risk of developing poor outcomes in ARVC., Methods: A systematic literature search from several databases was conducted until September 9th, 2023. Studies were eligible if it investigated the relationship between the ECG markers with the risk of developing ventricular arrhythmic events., Results: This meta-analysis encompassed 25 studies with a total of 3767 participants. Our study disclosed that epsilon waves, prolonged TAD of QRS, fQRS, late potentials on SA-ECG, TWI in right precordial leads, and extension of TWI in inferior leads were associated with the incremental risk of ventricular arrhythmias, implantable cardioverter-defibrillator shock, and sudden cardiac death, with the risk ratios ranging from 1.46 to 2.11. In addition, diagnostic test accuracy meta-analysis stipulated that the extension of TWI in inferior leads had the uppermost overall area under curve (AUC) value amidst other ECG markers apropos of our outcomes of interest., Conclusion: A multivariable risk assessment strategy based on the previously stated ECG markers potentially enhances the current risk stratification models in ARVC patients, especially extension of TWI in inferior leads., 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., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Hypertrophic cardiomyopathy (HCM) is the commonest genetic cardiomyopathy and may result in sudden cardiac death (SCD). Clinical risk stratification scores are utilised to estimate SCD risk and determine potential utility of a primary prevention implantable cardioverter defibrillator (ICD)., Methods: Patients with a confirmed diagnosis of HCM from a quaternary HCM service were defined according to clinical characteristics, genetic profiles and cardiac imaging results. European Risk-SCD score and American Heart Association / American College of Cardiology (AHA/ACC) Score were calculated. The primary outcome was cardiac arrest., Results: 380 patients with HCM were followed up for a median of 6.4 years. 18 patients (4.7%) experienced cardiac arrest, with predictive factors being younger age (37.2 vs 54.4 years, p = 0.0041), unexplained syncope (33.3% vs 9.4%, p = 0.007), non-sustained ventricular tachycardia (50.0% vs 12.7%, p < 0.0001), increased septal thickness (21.5 vs 17.5 mm, p = 0.0003), and presence of a sarcomeric gene mutation (100.0% vs 65.8%, p = 0.038). The Risk-SCD and AHA/ACC scores had poor agreement (kappa coefficient 0.38). Risk-SCD score had poor sensitivity (44.4%), classifying 55.6% of patients with cardiac arrest as low-risk but was highly specific (93.7%). AHA/ACC risk score did not discriminate between groups significantly. 20 patients (5.3%) died, with most >60-year-olds having a non-cardiac cause of death (p = 0.0223)., Conclusion: This study highlights limited (38%) agreement between the Risk-SCD and AHA/ACC scores. Most cardiac arrests occurred in ostensibly low or medium-risk patients under both scores. Appropriate ICD selection remains challenging. Incorporating newer risk markers such as HCM genotyping and myocardial fibrosis quantification by cardiac MRI may assist future risk refinement., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to disclose., (Crown Copyright © 2023. Published by Elsevier B.V. All rights reserved.)

Competing Interests: Disclosures Dr Balaji has received an external research grant from Medtronic to study sudden death in children with hypertrophic cardiomyopathy. Dr Balaji is a consultant to Milestone Pharmaceuticals and AltaThera Pharmaceuticals.

Background: Arrhythmogenic cardiomyopathy (ACM) is a very rare condition among pediatric patients. Sudden cardiac death (SCD) is the main complication and often requires ICD implantation. Aim of the study is the evaluation of the outcomes of ICD implanted ACM pediatric patients in terms of safety, efficacy and complications., Methods: All pediatric patients (<18 y.o.) diagnosed with ACM and who were implanted with ICD since 2009 in Our Institution were collected. Implantation was decided according to current recommendations/ guidelines, and outcome was recorded during follow-up., Results: Nineteen consecutive ACM patients were implanted with ICD. Subcutaneous ICDs (S-ICD) were implanted in 15 patients (79%) while transvenous ICDs (TV-ICD) in 4 patients (21%). Mean age at implantation was 14.3 ± 2.1 y.o. ICDs were implanted for secondary prevention in 4 (21%) patients, and for primary prevention in 15 (79%). During the follow-up (5.59 ± 3.4 years), appropriate ICD interventions were delivered in 4 (21%) patients for sustained VTs, [2 implanted in primary prevention (13%) and 2 in secondary prevention (50%)]. No defibrillation failures occurred. Inappropriate shocks occurred in 2 cases (10.5%). Device-related complications requiring device revision occurred in 3 (16%): lead dislodgement, surgical skin erosion and sensing defect., Conclusions: In a pediatric ACM cohort, appropriate ICD therapies occurred in a minority of primary prevention patients and frequently in secondary prevention patients. The rate of inappropriate shocks and device-related complications were even more rare and mostly wound related. Therefore, ICD therapy in pediatric ACM is effective and safe., Competing Interests: Declaration of Competing Interest The authors have no conflicts to disclose., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Commotio cordis is a rare but life-threatening condition characterized by sudden cardiac arrest resulting from a blunt chest impact. While commotio cordis has traditionally been associated with sports-related activities, a significant proportion of cases occur in non-sport-related settings, such as assaults, motor vehicle accidents (MVAs), and daily activities. This critical review examines the epidemiology, clinical characteristics, and outcomes of non-sports-related commotio cordis cases, highlighting the need for increased awareness and improved management in these contexts. The review analyzes existing literature, drawing attention to the demographics of non-sports-related cases, which predominantly affect adolescents and young adults, with males being the primary demographic. In contrast to sport-related cases, non-sports-related commotio cordis cases exhibit a wider age range and a higher proportion of female subjects. Mortality rates are significantly higher in non-sports-related commotio cordis cases, largely due to lower rates of cardiopulmonary resuscitation (CPR), limited access to automated external defibrillators (AEDs), and delayed initiation of resuscitative efforts compared to sport-related incidents. This underscores the critical importance of increasing awareness and preparedness in non-sport-related settings. To mitigate the risks associated with non-sports-related commotio cordis, efforts should focus on early recognition of the condition, timely administration of CPR, and the widespread availability and accessibility of AEDs in various environments. Enhanced awareness and education can potentially lead to a reduction in mortality and improved outcomes for individuals affected by commotio cordis outside of sports-related activities. In conclusion, commotio cordis is not exclusive to sports and presents a significant health risk in non-sport-related scenarios. This review emphasizes the urgent need for increased awareness, preparedness, and resuscitation measures in non-sports contexts to address the higher mortality associated with these cases., 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., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: RASopathies account for nearly 20% of cases of childhood hypertrophic cardiomyopathy (HCM). Sudden cardiac death (SCD) occurs in patients with RASopathy-associated HCM, but the risk factors for SCD have not been systematically evaluated., Aim: To validate the HCM Risk-Kids SCD risk prediction model in children with RASopathy-associated HCM and investigate potential specific SCD predictors in this population., Methods: Validation of HCM Risk-Kids was performed in a retrospective cohort of 169 patients with a RASopathy-associated HCM from 15 international paediatric cardiology centres. Multiple imputation by chained equations was used for missing values related to the HCM Risk-Kids parameters., Results: Eleven patients (6.5%) experienced a SCD or equivalent event at a median age of 12.5 months (IQR 7.7-28.64). The calculated SCD/equivalent event incidence was 0.78 (95% CI 0.43-1.41) per 100 patient years. Six patients (54.54%) with an event were in the low-risk category according to the HCM Risk-Kids model. Harrell's C index was 0.60, with a sensitivity of 9.09%, specificity of 63.92%, positive predictive value of 1.72%, and negative predictive value of 91%; with a poor distinction between the different risk groups. Unexplained syncope (HR 42.17, 95% CI 10.49-169.56, p < 0.001) and non-sustained ventricular tachycardia (HR 5.48, 95% CI 1.58-19.03, p < 0.007) were predictors of SCD on univariate analysis., Conclusion: Unexplained syncope and the presence of NSVT emerge as predictors for SCD in children with RASopathy-associated HCM. The HCM Risk-Kids model may not be appropriate to use in this population, but larger multicentre collaborative studies are required to investigate this further., Competing Interests: Declaration of Competing Interest Wolf CM: consultancy with Day One Biopharmaceuticals, Inc., BioMarin Pharmaceuticals, Adrenomed AG, and Pliant Therapeutics; ownership interest: Preventage Therapeutics. Zenker M: consultancy with Day One Biopharmaceuticals, Inc. and Novo Nordisk., (Copyright © 2023. Published by Elsevier B.V.)

Accurate identification of hypertrophic cardiomyopathy (HCM) patients at high risk of sudden cardiac death (SCD) event is challenging. The objective of this study was to validate the three SCD risk stratifications recommended by the 2014 European Society of Cardiology (ESC) guideline, the 2020 American Heart Association /American College of Cardiology (AHA/ACC) guideline, and the 2022 ESC guideline in Chinese patients with HCM. Our study population are made up of a cohort of 856 HCM patients without prior SCD events. The endpoint was defined as SCD or equivalent events (successful resuscitation after cardiac arrest or appropriate ICD shock for ventricular tachycardia or ventricular fibrillation). During a median follow-up of 43 months, SCD endpoints occurred in 44 (5.1%) patients. A total of 34 (77.3%) patients suffering from SCD events were classified correctly into high-risk groups by the 2020 AHA/ACC guideline, 27(61.4%) by the 2022 ESC guideline, and 13 (29.6%) by the 2014 ESC guideline. The C-statistic of the 2020 AHA/ACC guideline was 0.68 (95% CI, 0.60-0.76), which performed better than the 2022 ESC guideline (0.65: 95% CI, 0.56-0.73), and the 2014 ESC guideline (0.58: 95% CI, 0.48-0.67). The 2020 AHA/ACC guideline displayed better discrimination for SCD risk stratification in Chinese HCM patients than the other two guidelines, with a higher sensitivity but lower specificity., 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., (Copyright © 2023. Published by Elsevier Inc.)

We used the Artificial Neural Network (ANN) model to identify predictors of Sudden Cardiac Arrest (SCA) in a national cohort of young Asian patients in the United States. The National Inpatient Sample (2019) was used to identify young Asians (18-44-year-old) who were hospitalized with SCA. The neural network's predicted criteria for SCA were selected. After eliminating missing data, young Asians (n = 65,413) were randomly divided into training (n = 45,094) and testing (n = 19347) groups. Training data (70%) was used to calibrate ANN while testing data (30%) was utilized to assess the algorithm's accuracy. To determine ANN's performance in predicting SCA, we compared the frequency of incorrect prediction between training and testing data and measured the area under the Receiver Operating Curve (AUC). The 2019 young Asian cohort had 327,065 admissions (median age 32 years; 84.2% female), with SCA accounting for 0.21%. The exact rate of error in predictions vs. tests was shown by training data (0.2% vs 0.2%). In descending order, the normalized importance of predictors to accurately predict SCA in young adults included prior history of cardiac arrest, sex, age, diabetes, anxiety disorders, prior coronary artery bypass grafting, hypertension, congenital heart disease, income, peripheral vascular disease, and cancer. The AUC was 0.821, indicating an excellent ANN model for SCA prediction. Our ANN models performed excellently in revealing the order of important predictors of SCA in young Asian American patients. These findings could have a considerable impact on clinical practice to develop risk prediction models to improve the survival outcome in high-risk patients., 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., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: More than half of all sudden cardiac deaths (SCDs) are unwitnessed, but the composition of the unwitnessed SCD population is poorly described., Objective: The purpose of this study was to compare clinical and autopsy characteristics of young unwitnessed SCD subjects, based on the time from last contact to being found dead., Methods: All unwitnessed SCD subjects aged 1-35 years in Denmark from 2000-2014 identified through a multisource approach were included. Time from last seen alive to being found dead was dichotomized to <1 hour or 1-24 hours. Clinical characteristics and autopsy results were compared, and predictors of autopsy were assessed by logistic regression., Results: Of 440 unwitnessed SCD subjects, 366 (83%) had not been seen alive within 1 hour of being found dead. Comorbidities differed between the groups, with more epilepsy (17% vs 5%) and psychiatric diseases (13% vs 7%) in the 24-hour group. Patients in the 24-hour group died more frequently during sleep (64% vs 23%), the autopsy rate was higher (75% vs 61%), and deaths were more often unexplained after autopsy (69% vs 53%). Having been seen within 1 hour of death independently decreased the chance of being autopsied (odds ratio 0.51; 95% confidence interval 0.27-1.00; P = .0497)., Conclusion: The majority of unwitnessed SCD subjects had not been seen alive within 1 hour of being found dead. Clinical- and autopsy-related characteristics differed between the 2 groups. Differences were mainly attributable to death-related circumstances and comorbidities. Excluding SCD cases not seen alive within 1 hour of being found dead would severely underestimate the burden of SCD., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Implantable cardioverter-defibrillators (ICDs) reduce mortality in patients with reduced left ventricular ejection fraction (LVEF). We investigated sex disparities in a contemporary Canadian population for utilization of primary prevention ICDs., Methods: This was a retrospective cohort study on patients with reduced LVEF admitted to hospitals from 2010 to 2020 in Nova Scotia (population = 971,935)., Results: There were 4406 patients eligible for ICDs: 3108 (71%) men and 1298 (29%) women. The mean follow-up time was 3.9 ± 3.0 years. Rates of coronary disease were similar between men and women (45.8% vs 44.0%; P = 0.28), but men had lower LVEF (26.6 ± 5.9% vs 27.2 ± 5.8%; P = 0.0017). The referral rate for ICD was 11% (n = 487), with 13% of men (n = 403) and 6.5% of women (n = 84) referred (P < 0.001). The ICD implantation rate in the population was 8% (n = 358), with 9.5% of men (n = 296) and 4.8% of women (n = 62) (P < 0.001) receiving the device. Men were more likely than women to receive an ICD (odds ratio 2.08, 95% confidence interval 1.61-2.70; P < 0.0001)). There was no significant difference in mortality between men and women (P = 0.2764). There was no significant difference in device therapies between men and women (43.8% vs 31.1%; P = 0.0685)., Conclusions: A significant disparity exists in the utilization of primary prevention ICDs between men and women in a contemporary Canadian population., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Although the 12‑lead electrocardiogram (ECG) is abnormal in most patients with hypertrophic cardiomyopathy (HCM), some present normal ECG. This study aimed to analyse the baseline characteristics, clinical presentation and outcomes of HCM patients with normal ECG and to compare them with those with abnormal ECG., Methods and Results: Baseline characteristics, clinical presentation, data from complementary exams and clinical outcomes of 1070 consecutive patients included in the Portuguese Registry of HCM (Pro-HCM registry) were compared between two groups of patients: normal Vs. abnormal ECG. Among this population, 98 (9.2%) patients had normal ECG at presentation; they were significantly younger and had lower frequency of hypertension, symptoms at presentation, heart failure, angina, cardiac and non-cardiac diseases. ESC and AHA risk scores for Sudden Cardiac Death (SCD) were not significantly different between the two groups. Patients with normal ECG had higher prevalence of family history of SCD and lower degree of left ventricular (LV) hypertrophy, LV systolic dysfunction, LV outflow tract obstruction and myocardial fibrosis. The combined endpoint of cardiac death, SCD, cardiac arrest, appropriate ICD shocks or evolution to systolic dysfunction, during a mean follow-up of 5 years was significantly less frequent in patients with normal ECG (2.1% Vs. 6.5%; p = 0.043)., Conclusions: A normal ECG is not a marker of an overall benign profile in HCM patients. Though a normal ECG at presentation is associated with a less severe phenotype and a lower probability of evolution to heart failure at 5-years, this finding did not show a protective effect in other clinical outcomes., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Hypertrophic cardiomyopathy (HCM) carries an increased risk of sudden cardiac death. Ventricular fibrillation (VF) is thought to be the common culprit arrhythmia., Objective: The purpose of this study was to describe the incidence and predictors of sustained ventricular arrhythmias (VTAs) in HCM patients., Methods: We retrospectively analyzed all patients with HCM and an implantable cardioverter-defibrillator (ICD) from a prospectively derived registry in 2 tertiary medical centers. Clinical, electrocardiographic, echocardiographic, ICD interrogation, and genetic data were collected and compared, first between patients with and without VTAs and then between patients with only VF and those with ventricular tachycardia (VT) with or without VF., Results: Of the 1328 HCM patients, 207 (145 [70%] male; mean age 33 ± 16 years) were implanted with ICDs. Over a mean follow-up of 10 ± 6 years, 37 patients with ICDs (18%) developed sustained VTAs. These were associated with a family history of sudden cardiac death and a personal history of VTAs (P = .036 and P = .001, respectively). Sustained monomorphic VT was the most common arrhythmia (n = 26, 70%) and was linked to decreased left ventricular (LV) ejection fraction and increased LV end-systolic and end-diastolic diameters. Antitachycardia pacing (ATP) successfully terminated 258 (79%) of the 326 VT events. Mortality rates were comparable between patients with and without VTAs (4 [11%] vs 29 [17%]; P = .42) and between those with and without ICDs (24 [16%] vs 85 [20%]; P = .367)., Conclusion: VT rather than VF is the most common arrhythmia in patients with HCM; it is amenable to ATP and is associated with lower LV ejection fraction and higher LV diameters. Therefore, ATP-capable devices may be considered in HCM patients with these LV features., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Illicit drug use may accelerate coronary disease and cardiac hypertrophy or stimulate arrhythmias. Rates of illicit drug use in young patients with sudden cardiac death (SCD) are uncertain., Objective: The purpose of this study was to identify rates of illicit drug use in young patients with SCD., Methods: A prospective statewide registry identified out-of-hospital patients with cardiac arrest aged 18-50 years from April 2019 to April 2021. Clinical characteristics were compared between patients with and without illicit drug use (defined by toxicological results or reported regular use). Illicit drugs included amphetamine-type substances, cocaine, heroin, cannabis, and other drugs., Results: A total of 554 (40.2%) of 1378 patients had confirmed cardiac cause of out-of-hospital cardiac arrest, with 523 undergoing toxicological assessment. There were 170 patients (32.5%) having either positive toxicology for illicit drugs (n = 138) or negative toxicology but reported regular drug use (n = 32). Patients with SCD and illicit drug use were more commonly male (81.2% vs 72.3%; P = .028), smokers (38.8% vs 19.8%; P ≤ .0001), and excess alcohol drinkers (30.6% vs 20.6%; P = .012) and had a psychiatric diagnosis (38.8% vs 25.7%; P = .002), lower body mass index (29.4 kg/m 2 vs 31.7 kg/m 2 ; P = .0063), and lower rates of hypertension (10.6% vs 18.6%; P = .019). Death commonly occurred while sedentary (47.5%) or during sleep (45.8%). Accounting for these baseline differences, there were no differences in rates of coronary disease or cardiomyopathy. Cannabis (n = 106) was the most common illicit drug identified and polysubstance abuse occurred frequently (n = 25)., Conclusion: Approximately one-third of young patients with SCD have positive toxicology at the time of death or reported frequent use of illicit drugs, with high rates of polysubstance abuse., (Crown Copyright © 2023. Published by Elsevier Inc. All rights reserved.)

Sarcoidosis, amyloidosis, hemochromatosis and scleroderma are the most forms of infiltrative/nonischemic cardiomyopathy (NICM) associated with sudden cardiac death. In patients who undergo in-hospital cardiac arrest, a high index of suspicion is required to rule out NICM as an underlying contributor. We aimed to analyze the prevalence of NICM among patients with in-hospital cardiac arrest and identify factors associated with increased mortality. We analyzed data from the National Inpatient Sample, and identified patients who were hospitalized across 10 years from 2010 to 2019 with a diagnosis of cardiac arrest and NICM. The total number of patients with in-hospital cardiac arrest was 19,34,260. The total number with NICM was 14,803 (0.77%). Mean age was 63 years. Overall prevalence of NICM across the years ranged between 0.75% to 0.9%, with a significant temporal increase (P < 0.01). Incidence of in-hospital mortality ranged between 61% to 76% for females and 30% to 38% for males. The following comorbidities were more prevalent in patients with NICM than those without: heart failure, chronic obstructive pulmonary disease (COPD), chronic kidney disease, anemia, malignancy, coagulopathy, ventricular tachycardia, acute kidney injury and stroke. The following factors were independent predictors of in-hospital mortality-age, female gender, Hispanic race, history of COPD and presence of malignancy (P = 0.042). The prevalence of infiltrative cardiomyopathy in patients with in-hospital cardiac arrest is increasing. Females, older patients and Hispanic population are at an increased risk of mortality. Sex and race-based disparities in the prevalence of NICM in patients with in-hospital cardiac arrest is an area of further research., 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., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Arrhythmic mitral valve prolapse (MVP) has gained great interest recently because of the increasing recognition of its potential role in unexplained cardiac arrest. Although evidence has accumulated to show the association of arrhythmic MVP (AMVP) with sudden cardiac death (SCD), risk stratification and management remain unclear. Physicians are faced with the challenges of screening for AMVP among MVP patients and the dilemma of when and how to intervene to prevent SCD in these patients. In addition, there is little guidance to help approach MVP patients who present with an otherwise unexplained cardiac arrest to know whether MVP was the primary cause of cardiac arrest or just an innocent bystander. Herein we review the epidemiology and definition of AMVP, the risk and mechanisms of SCD, and summarize the clinical evidence behind risk markers of SCD and therapeutic interventions that could potentially prevent it. We also propose an algorithm that provides guidance as to how to screen for AMVP and what therapeutic interventions to use. Last, we propose a diagnostic algorithm for approaching patients with otherwise unexplained cardiac arrest who are shown to have MVP., (Copyright © 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Background: The burden of cardiac death after transcatheter aortic valve replacement (TAVR), particularly from advanced heart failure (HF) and sudden cardiac death (SCD), remains largely unknown., Objectives: This study sought to evaluate the incidence and predictors of SCD and HF-related death in TAVR recipients treated with newer-generation devices., Methods: This study included a total of 5,421 consecutive patients who underwent TAVR with newer-generation devices using balloon (75.7%) or self-expandable (24.3%) valves., Results: After a median follow-up of 2 (IQR: 1-3) years, 976 (18.0%) patients had died, 50.8% from cardiovascular causes. Advanced HF and SCD accounted for 11.6% and 7.5% of deaths, respectively. Independent predictors of HF-related death were atrial fibrillation (HR: 2.17; 95% CI: 1.47-3.22; P < 0.001), prior pacemaker (HR: 1.79; 95% CI: 1.10-2.92; P = 0.01), reduced left ventricular ejection fraction (HR: 1.08 per 5% decrease; 95% CI: 1.01-1.14; P = 0.02), transthoracic approach (HR: 2.50; 95% CI: 1.37-4.55; P = 0.003), and new-onset persistent left bundle branch block (HR: 1.85; 95% CI: 1.14-3.02; P = 0.01). Two baseline characteristics (diabetes, HR: 1.81; 95% CI: 1.13-2.89; P = 0.01; and chronic kidney disease, HR: 1.72; 95% CI: 1.02-2.90; P = 0.04) and 3 procedural findings (valve in valve, HR: 2.17; 95% CI: 1.01-4.64; P = 0.04; transarterial nontransfemoral approach, HR: 2.23; 95% CI: 1.23-4.48; P = 0.01; and periprocedural ventricular arrhythmia, HR: 7.19; 95% CI: 2.61-19.76; P < 0.001) were associated with an increased risk of SCD after TAVR., Conclusions: Advanced HF and SCD accounted for a fifth of deaths after TAVR in contemporary practice. Potentially treatable factors leading to increased risk of HF deaths and SCD were identified, such as arrhythmia/dyssynchrony factors for HF and valve-in-valve TAVR or periprocedural ventricular arrhythmias for SCD., Competing Interests: Funding Support and Author Disclosures Dr Mesnier was supported by a research grant from Fédération Française de Cardiologie. Dr Belahnech was supported by a research grant from Aula Vall d’Hebron. Dr Rodés-Cabau holds the Research Chair “Fondation Famille Jacques Larivière” for the Development of Structural Heart Disease Interventions; and has received institutional research grants and consultant/speaker fees from Edwards Lifesciences and Medtronic. Dr Ternacle has served as a consultant for Abbott. Dr Modine has served as a consultant for Abbott, Edwards Lifesciences, and Medtronic. Dr Nombela-Franco has served as a proctor for Abbott and Edwards Lifesciences. Dr Reguiero has served as a proctor for Abbott. Dr Himbert has served as a proctor for Abbott and Edwards Lifesciences. Dr Asmarats has received speaker fees from Edwards Lifesciences. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)