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Background There is an increase in the sale of legal drugs in our country. One of these substances is kratom. Kratom (Mitragyna speciosa) is a partial agonist of the opioid kappa, mu, and delta receptors. It acts as a stimulant at low concentrations, making users feel more energetic and euphoric. It has sedative and antinociceptive effects at higher doses. Case summary An 18-year-old man collapsed during football training and required cardiopulmonary resuscitation; the initial rhythm was ventricular fibrillation managed by defibrillation. Laboratory parameters were unremarkable. Blood samples sent for toxicological evaluation were positive for kratom and caffeine. Echocardiographic examination, coronary computed tomography angiography, and cardiac magnetic resonance imaging did not prove the cause. Genetic testing did not find a pathogenic gene variant associated with familial ventricular fibrillation, but a variant of unknown significance was found in MYOM1. Given this situation, we implanted an implantable cardioverter-defibrillator (ICD) from the secondary prevention of sudden cardiac death (SCD) according to the guidelines of the European Society of Cardiology (ESC). No recurrence of ventricular arrhythmia has been reported by ambulatory ICD memory checks on our patient. Discussion In some country, kratom is freely available and sold as a plant, not a drug. Only incident cases of ventricular fibrillation after kratom use are described in the literature. There is insufficient scientific evidence linking kratom to ventricular fibrillation. This is an absolutely crucial case report of this type, which has not yet been published in similar circumstances in the world. Therefore, the development of ventricular fibrillation was assumed to be due to a combination of kratom, caffeine, and exercise. The safety profile and effects of kratom should be the subject of future research. We would like to stress the importance of reporting further case series for more scientific evidence and thus increasing the pressure for stricter availability and regulation of kratom in some countries, especially where it is over-the-counter. [ABSTRACT FROM AUTHOR]

Aims Primary prevention patients with ischaemic cardiomyopathy and chronic total occlusion of an infarct-related coronary artery (CTO) are at a particularly high risk of implantable cardioverter-defibrillator (ICD) therapy occurrence. The trial was designed to evaluate the efficacy of preventive CTO-related substrate ablation strategy in ischaemic cardiomyopathy patients undergoing primary prevention ICD implantation. Methods and results The PREVENTIVE VT study was a prospective, multicentre, randomized trial including ischaemic patients with ejection fraction ≤40%, no documented ventricular arrhythmias (VAs), and evidence of scar related to the coronary CTO. Patients were randomly assigned 1:1 to a preventive substrate ablation before ICD implantation or standard therapy with ICD implantation only. The primary outcome was a composite of appropriate ICD therapy or unplanned hospitalization for VAs. Secondary outcomes included the primary outcome's components, the incidence of appropriate ICD therapies, cardiac hospitalization, electrical storm, and cardiovascular (CV) mortality. Sixty patients were included in the study. During the mean follow-up of 44.7 ± 20.7 months, the primary outcome occurred in 5 (16.7%) patients undergoing preventive substrate ablation and in 13 (43.3%) patients receiving only ICD [hazard ratio (HR): 0.33; 95% confidence interval (CI): 0.12–0.94; P = 0.037]. Patients in the preventive ablation group also had fewer appropriate ICD therapies (P = 0.039) and the electrical storms (Log-rank: P = 0.01). While preventive ablation also reduced cardiac hospitalizations (P = 0.006), it had no significant impact on CV mortality (P = 0.151). Conclusion Preventive ablation of the coronary CTO-related substrate in patients undergoing primary ICD implantation is associated with the reduced risk of appropriate ICD therapy or unplanned hospitalization due to VAs. [ABSTRACT FROM AUTHOR]

Background and Aims Implantable cardioverter-defibrillators (ICDs) are critical for preventing sudden cardiac death (SCD) in arrhythmogenic right ventricular cardiomyopathy (ARVC). This study aims to identify cross-continental differences in utilization of primary prevention ICDs and survival free from sustained ventricular arrhythmia (VA) in ARVC. Methods This was a retrospective analysis of ARVC patients without prior VA enrolled in clinical registries from 11 countries throughout Europe and North America. Patients were classified according to whether they received treatment in North America or Europe and were further stratified by baseline predicted VA risk into low- (<10%/5 years), intermediate- (10%–25%/5 years), and high-risk (>25%/5 years) groups. Differences in ICD implantation and survival free from sustained VA events (including appropriate ICD therapy) were assessed. Results One thousand ninety-eight patients were followed for a median of 5.1 years; 554 (50.5%) received a primary prevention ICD, and 286 (26.0%) experienced a first VA event. After adjusting for baseline risk factors, North Americans were more than three times as likely to receive ICDs {hazard ratio (HR) 3.1 [95% confidence interval (CI) 2.5, 3.8]} but had only mildly increased risk for incident sustained VA [HR 1.4 (95% CI 1.1, 1.8)]. North Americans without ICDs were at higher risk for incident sustained VA [HR 2.1 (95% CI 1.3, 3.4)] than Europeans. Conclusions North American ARVC patients were substantially more likely than Europeans to receive primary prevention ICDs across all arrhythmic risk strata. A lower rate of ICD implantation in Europe was not associated with a higher rate of VA events in those without ICDs. [ABSTRACT FROM AUTHOR]

Background and Aims Intra-pocket ultrasound-guided axillary vein puncture (IPUS-AVP) for venous access in implantation of transvenous cardiac implantable electronic devices (CIED) is uncommon due to the lack of clinical evidence supporting this technique. This study investigated the efficacy and early complications of IPUS-AVP compared to the standard method using cephalic vein cutdown (CVC) for CIED implantation. Methods ACCESS was an investigator-led, interventional, randomized (1:1 ratio), monocentric, controlled superiority trial. A total of 200 patients undergoing CIED implantation were randomized to IPUS-AVP (n = 101) or CVC (n = 99) as a first assigned route. The primary endpoint was the success rate of insertion of all leads using the first assigned venous access technique. The secondary endpoints were time to venous access, total procedure duration, fluoroscopy time, X-ray exposure, and complications. Complications were monitored during a follow-up period of three months after procedure. Results IPUS-AVP was significantly superior to CVC for the primary endpoint with 100 (99.0%) vs. 86 (86.9%) procedural successes (P =.001). Cephalic vein cutdown followed by subclavian vein puncture was successful in a total of 95 (96.0%) patients, P =.21 vs. IPUS-AVP. All secondary endpoints were also significantly improved in the IPUS-AVP group with reduction in time to venous access [3.4 vs. 10.6 min, geometric mean ratio (GMR) 0.32 (95% confidence interval, CI, 0.28–0.36), P <.001], total procedure duration [33.8 vs. 46.9 min, GMR 0.72 (95% CI 0.67–0.78), P <.001], fluoroscopy time [2.4 vs. 3.3 min, GMR 0.74 (95% CI 0.63–0.86), P <.001], and X-ray exposure [1083 vs. 1423 mGy.cm², GMR 0.76 (95% CI 0.62–0.93), P =.009]. There was no significant difference in complication rates between groups (P =.68). Conclusions IPUS-AVP is superior to CVC in terms of success rate, time to venous access, procedure duration, and radiation exposure. Complication rates were similar between the two groups. Intra-pocket ultrasound-guided axillary vein puncture should be a recommended venous access technique for CIED implantation. [ABSTRACT FROM AUTHOR]

Aims The validated HCM Risk-Kids model provides accurate individualized estimates of sudden cardiac death risk in children with hypertrophic cardiomyopathy (HCM). A second validated model, PRIMaCY, also provides individualized estimates of risk, but its performance and clinical impact has not been independently investigated. The aim of this study was to investigate the clinical impact of using the PRIMaCY sudden cardiac death (SCD) risk model in childhood HCM. Methods and results The estimated 5-year SCD risk was calculated for children meeting diagnostic criteria for HCM in a large single-centre cohort using PRIMaCY (clinical and genetic) and HCM Risk-Kids model, and model performance was assessed. Three hundred one patients [median age 10 (interquartile range 4–14)] were followed up for an average of 4.9 (±3.8) years, during which 30 (10.0%) reached the SCD or equivalent event endpoint. Harrell's C -statistic for the clinical and genetic models was 0.66 [95% confidence interval (CI) 0.52–0.8] and 0.66 (95% CI 0.54–0.80) with a calibration slope of 0.19 (95% CI 0.04–0.54) and 0.26 (95% CI −0.03–0.62), respectively. The number needed to treat to potentially treat one life-threatening arrhythmia for the PRIMaCY clinical, PRIMaCY genetic, and HCM Risk-Kids models was 13.7, 14.5, and 9.4, respectively. Conclusion Although PRIMaCY has a similar discriminatory ability to that reported for HCM Risk-Kids, estimated risk estimates did not correlate well with observed risk. A higher proportion of patients met implantable cardioverter-defibrillator thresholds using PRIMaCY model compared with HCM Risk-Kids. This has important clinical implications as these patients will be exposed to a lifetime risk of complications and inappropriate therapies. [ABSTRACT FROM AUTHOR]

Aims This study assessed associations of minimum final extrastimulus coupling interval utilized within electrophysiology study (EPS) after myocardial infarction (MI) and possible site of origin of induced ventricular tachycardia (VT) with long-term occurrence of spontaneous ventricular tachyarrhythmia and long-term survival. Methods and results This prospective study recruited consecutive patients with left ventricular ejection fraction (LVEF) ≤ 40% who underwent EPS days 3–5 after MI between 2004 and 2017. Positive EPS was defined as sustained monomorphic VT cycle length ≥200 ms for ≥10 s or shorter duration if haemodynamic compromise occurred. Each of the four extrastimuli was shortened by 10 ms at a time, until it failed to capture the ventricle (ventricular refractoriness) or induced ventricular tachyarrhythmia. Outcomes included spontaneous ventricular tachyarrhythmia occurrence and all-cause mortality. Shorter coupling interval length of final extrastimulus that induced VT was associated with higher risk of spontaneous ventricular tachyarrhythmia (P < 0.001). Significantly higher rates of spontaneous ventricular tachyarrhythmia (65.2% vs. 23.2%; P < 0.001) were observed for final coupling interval at EPS <200 ms vs. >200 ms. Right bundle branch block (RBBB) morphology of induced VT, with possible site of origin from the left ventricle, was associated with all-cause mortality [hazard ratio (HR) 3.2, P = 0.044] and a composite of spontaneous ventricular tachyarrhythmia recurrence or mortality (HR 1.8, P = 0.043). Conclusion Ventricular tachycardia induced with shorter coupling intervals was associated with higher risk of spontaneous ventricular tachyarrhythymia on follow-up, indicating that the final extrastimulus coupling interval at EPS early after MI should be determined by ventricular refractoriness. Induced VT with possible origin from left ventricle was associated with increased risk of spontaneous ventricular tachyarrhythmia recurrence or death. [ABSTRACT FROM AUTHOR]

Cardiac sarcoidosis (CS) results from epithelioid cell granulomas infiltrating the myocardium and predisposing to conduction disturbances, ventricular tachyarrhythmias, and heart failure. Manifest CS, however, constitutes only the top of an iceberg as advanced imaging uncovers cardiac involvement 4 to 5 times more commonly than what is clinically detectable. Definite diagnosis of CS requires myocardial biopsy and histopathology, but a sufficient diagnostic likelihood can be achieved by combining extracardiac histology of sarcoidosis with clinical manifestations and findings on cardiac imaging. CS can appear as the first or only organ manifestation of sarcoidosis or on top of pre-existing extracardiac disease. Due to the lack of controlled trials, the care of CS is based on observational evidence of low quality. Currently, the treatment involves corticosteroid-based, tiered immunosuppression to control myocardial inflammation with medical and device-based therapy for symptomatic atrioventricular block, ventricular tachyarrhythmias, and heart failure. Recent outcome data indicate 90% to 96% 5-year survival in manifest CS with the 10-year figures ranging from 80% to 90%. Major progress in the care of CS awaits the key to its molecular–genetic pathogenesis and large-scale controlled clinical trials. [ABSTRACT FROM AUTHOR]

There is an increasing proportion of the general population surviving to old age with significant chronic disease, multi-morbidity, and disability. The prevalence of pre-frail state and frailty syndrome increases exponentially with advancing age and is associated with greater morbidity, disability, hospitalization, institutionalization, mortality, and health care resource use. Frailty represents a global problem, making early identification, evaluation, and treatment to prevent the cascade of events leading from functional decline to disability and death, one of the challenges of geriatric and general medicine. Cardiac arrhythmias are common in advancing age, chronic illness, and frailty and include a broad spectrum of rhythm and conduction abnormalities. However, no systematic studies or recommendations on the management of arrhythmias are available specifically for the elderly and frail population, and the uptake of many effective antiarrhythmic therapies in these patients remains the slowest. This European Heart Rhythm Association (EHRA) consensus document focuses on the biology of frailty, common comorbidities, and methods of assessing frailty, in respect to a specific issue of arrhythmias and conduction disease, provide evidence base advice on the management of arrhythmias in patients with frailty syndrome, and identifies knowledge gaps and directions for future research. [ABSTRACT FROM AUTHOR]

Aims There is little evidence of the impact of syncope in implantable cardioverter-defibrillator (ICD) patients in routine community hospital care. This single-centre retrospective study sought to evaluate the incidence and prognostic significance of syncope in consecutive ICD patients. Methods and results Data were collected on consecutive patients undergoing first ICD implantation between January 2009 and December 2019. The primary endpoints were the first occurrence of all-cause syncope, all-cause mortality, and all-cause hospitalization. Multivariate Cox proportional hazard models were used to identify risk factors associated with syncope and to analyse the subsequent risk of mortality and hospitalization. 1003 patients (58% primary prevention) were included in the final analysis. During a mean follow-up of 1519 ± 1055 days, 106 (10.6%) experienced syncope, 304 died (30.3%), and 477 (47.5%) were hospitalized for any cause. In an analysis adjusted for baseline variables, the first occurrence of syncope was associated with a significantly increased risk of mortality (HR 2.82, P < 0.001) and the first occurrence of hospitalization (HR 2.46, P = 0.002). Conclusion Syncope in ICD recipients is common and associated with a poor prognosis irrespective of baseline variables and ICD programming. The occurrence of syncope is associated with a significant increase in the risk of mortality and hospitalization. [ABSTRACT FROM AUTHOR]

Aims To analyze and compare the effectiveness and safety of transvenous lead extraction (TLE) of implantable cardioverter-defibrillator (ICD) leads with a dwell time of >10 years (Group A) vs. younger leads (Group B) using mechanical extraction systems. Methods and results Between October 2011 and July 2022, we performed TLE in 318 patients. Forty-six (14.4%) extracted ICD leads in 46 (14.5%) patients that had been implanted for >10 years. The median dwell time of all extracted ICD leads was 5.9 years. Cardiovascular implantable electronic device-related infection was an indication for TLE in 31.8% of patients. Complete ICD leads removal and complete procedural success in both groups were similar (95.7% in Group A vs. 99.6% in Group B, P = 0.056% and 95.6% in Group A vs. 99.6% in Group B, P = 0.056, respectively). We did not find a significant difference between major and minor complication rates in both groups (6.5% in Group A vs. 1.5% in Group B and 2.2% in Group A vs. 1.8% in Group B, P = 0.082, respectively). One death associated with the TLE procedure was recorded in Group B. Conclusion The TLE procedures involving the extraction of old ICD leads were effective and safe. The outcomes of ICD lead removal with a dwell time of >10 years did not differ significantly compared with younger ICD leads. However, extraction of older ICD leads required more frequent necessity for utilizing multiple extraction tools, more experience and versatility of the operator, and increased surgery costs. [ABSTRACT FROM AUTHOR]

Background Subcutaneous implantable cardioverter-defibrillators (S-ICDs) are increasingly used in patients without a pacing indication, and may reduce venous complications, endocarditis and extraction morbidity. Subcutaneous implantable cardioverter-defibrillator displacements may be less obvious than their transvenous counterparts. Case summary A 59-year-old man was found to have dilated cardiomyopathy associated with heavy alcohol intake following investigation for a stroke. Despite 2 years of alcohol cessation and optimal drug therapy, his ejection fraction remained severely impaired, and he received an S-ICD using the manufacturer recommended screening and implant method, and by an experienced operator. Ten months later, inappropriate shocks were delivered despite optimal programming. Device displacement was demonstrated by lateral chest radiography on the second instance of inappropriate therapy. On the first admission, a lateral film was not performed, and simple device programming was undertaken which failed to prevent the second occurrence. The patient requested an explant; as ventricular function had improved following initiation of sacubutril/valsartan, the clinical team opted to remove the device. Discussion Careful inspection of lateral chest films and review of device indication are needed to reduce the risk of inappropriate shocks. This is the first description of inappropriate device activity following lone generator displacement—lead displacement is well described. [ABSTRACT FROM AUTHOR]

Background Risk stratification for sudden cardiac death (SCD) is a key factor in the management of patients with hypertrophic cardiomyopathy (HCM). Cardiac magnetic resonance (CMR) has a unique role in the evaluation of HCM and offers superior diagnostic and prognostic information to assess the indication for a prophylactic implantable cardioverter-defibrillator (ICD). Case summary A 39-year-old patient with non-obstructive HCM with a low ESC HCM Risk-SCD score underwent a CMR revealing a left ventricular apical aneurysm and extensive late gadolinium enhancement; a prophylactic ICD was thus implanted. A month later, the patient was admitted in refractory electrical storm with over 50 appropriate ICD shocks due to sustained ventricular tachycardia. Despite anti-arrhythmic therapy and mechanical ventilation, the evolution was unfavourable with haemodynamic instability; veno-arterial extracorporeal membrane oxygenation was implanted. The patient was submitted to CMR-guided epicardial VT catheter ablation with complications of LV thrombus and severe pericardial effusion. Discussion This case details the complex risk stratification for SCD in patients with HCM, highlighting the important role of CMR in the integrated approach to risk stratification. [ABSTRACT FROM AUTHOR]

Background Implantable cardioverter-defibrillator (ICD) implantation is a key therapeutic option in arrhythmogenic right ventricular cardiomyopathy (ARVC) to prevent sudden cardiac death due to ventricular tachycardia (VT) and fibrillation (VF). However, sub-optimized R-wave sensing due to myocardium loss interferes with VT/VF identification and appropriate therapy. We tried to implant a 3830 lead to the left ventricular septum (LVS) to facilitate ICD sensing in an ARVC patient. Case summary A 68-year-old woman diagnosed with ARVC was scheduled to undergo ICD implantation. Initially, no sites with suitable R-wave amplitudes were found in the right ventricle (RV) to deploy the defibrillation lead (<3.0 mV). It was likely due to severe RV involvement, but the LVS myocardium was more preserved based on cardiac magnetic resonance imaging. Therefore, we implanted a 3830 lead into the deep area of the septum to facilitate R-wave sensing. During the procedure from the right to left septum, the R-wave amplitude significantly increased (2.6 to 4.3–7.1 mV). Left ventricular septum pacing was finally achieved with favourable R-wave sensing (9.9 mV 24 h post-operation). The 3830 lead was plugged into the IS-1 port, while the defibrillation lead was plugged into the DF-1 port. After a 4-month follow-up, the R-wave amplitude of the 3830 lead was 11.1 mV. Discussion When the R-wave sensing is not acceptable for ICD implantation in ARVC patients, it is critical to assess myocardial conditions comprehensively. If the septal myocardium is preserved, implanting a 3830 lead to the deep or LVS is feasible to improve R-wave sensing. [ABSTRACT FROM AUTHOR]

Aims: Most patients who receive implantable cardioverter defibrillators (ICDs) for primary prevention do not receive therapy during the lifespan of the ICD, whilst up to 50% of sudden cardiac death (SCD) occur in individuals who are considered low risk by conventional criteria. Machine learning offers a novel approach to risk stratification for ICD assignment.Methods and Results: Systematic search was performed in MEDLINE, Embase, Emcare, CINAHL, Cochrane Library, OpenGrey, MedrXiv, arXiv, Scopus, and Web of Science. Studies modelling SCD risk prediction within days to years using machine learning were eligible for inclusion. Transparency and quality of reporting (TRIPOD) and risk of bias (PROBAST) were assessed. A total of 4356 studies were screened with 11 meeting the inclusion criteria with heterogeneous populations, methods, and outcome measures preventing meta-analysis. The study size ranged from 122 to 124 097 participants. Input data sources included demographic, clinical, electrocardiogram, electrophysiological, imaging, and genetic data ranging from 4 to 72 variables per model. The most common outcome metric reported was the area under the receiver operator characteristic (n = 7) ranging between 0.71 and 0.96. In six studies comparing machine learning models and regression, machine learning improved performance in five. No studies adhered to a reporting standard. Five of the papers were at high risk of bias.Conclusion: Machine learning for SCD prediction has been under-applied and incorrectly implemented but is ripe for future investigation. It may have some incremental utility in predicting SCD over traditional models. The development of reporting standards for machine learning is required to improve the quality of evidence reporting in the field. [ABSTRACT FROM AUTHOR]

Aims: The GermAn Laser Lead Extraction RegistrY: GALLERY is a retrospective, national multicentre registry, investigating the safety and efficacy of laser lead extraction procedures in Germany.Methods and Results: Twenty-four German centres that are performing laser lead extraction have participated in the registry. All patients, treated with a laser lead extraction procedure between January 2013 and March 2017, were consecutively enrolled. Safety and efficacy of laser lead extraction were investigated. A total number of 2524 consecutive patients with 6117 leads were included into the registry. 5499 leads with a median lead dwell time of 96 (62-141) months were treated. The mean number of treated leads per patient was 2.18 ± 1.02. The clinical procedural success rate was 97.86% and the complete lead removal was observed in 94.85%. Additional extraction tools were used in 6.65% of cases. The rate of procedural failure was 2.14% with lead age  ≥10 years being its only predictor. The overall complication rate was 4.32%, including 2.06% major and 2.26% minor complications. Procedure-related mortality was 0.55%. Female sex and the presence of abandoned leads were predictors for procedure-related complications. The all-cause in-hospital mortality was 3.56% with systemic infection being the strongest predictor, followed by age ≥75 years and chronic kidney disease.Conclusion: In the GALLERY, a high success- and low procedure-related complication rates have been demonstrated. In multivariate analysis, female sex and the presence of abandoned leads were predictors for procedure-related complications, while the presence of systemic infection, age ≥75 years, and chronic kidney disease were independent predictors for all-cause mortality. [ABSTRACT FROM AUTHOR]

Aims: While electrocardiogram (ECG) characteristics have been associated with life-threatening ventricular arrhythmias (LTVA) in dilated cardiomyopathy (DCM), they typically rely on human-derived parameters. Deep neural networks (DNNs) can discover complex ECG patterns, but the interpretation is hampered by their 'black-box' characteristics. We aimed to detect DCM patients at risk of LTVA using an inherently explainable DNN.Methods and Results: In this two-phase study, we first developed a variational autoencoder DNN on more than 1 million 12-lead median beat ECGs, compressing the ECG into 21 different factors (F): FactorECG. Next, we used two cohorts with a combined total of 695 DCM patients and entered these factors in a Cox regression for the composite LTVA outcome, which was defined as sudden cardiac arrest, spontaneous sustained ventricular tachycardia, or implantable cardioverter-defibrillator treated ventricular arrhythmia. Most patients were male (n = 442, 64%) with a median age of 54 years [interquartile range (IQR) 44-62], and median left ventricular ejection fraction of 30% (IQR 23-39). A total of 115 patients (16.5%) reached the study outcome. Factors F8 (prolonged PR-interval and P-wave duration, P < 0.005), F15 (reduced P-wave height, P = 0.04), F25 (increased right bundle branch delay, P = 0.02), F27 (P-wave axis P < 0.005), and F32 (reduced QRS-T voltages P = 0.03) were significantly associated with LTVA.Conclusion: Inherently explainable DNNs can detect patients at risk of LTVA which is mainly driven by P-wave abnormalities. [ABSTRACT FROM AUTHOR]

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC), an inherited heart muscle abnormality, is a major cause of sudden cardiac death (SCD). However, the burden of SCD and risk factors in ARVC are not clearly described. Thus, we estimated the rates and predictors of SCD in ARVC in a meta-analysis.Methods and Results: PubMed, Embase, and Web of Science were searched through 7 April 2021. Prospective studies reporting SCD from ARVC cohorts were included. Data were independently extracted by two reviewers and pooled in a random-effects meta-analysis. Fifty-two studies (n = 5485 patients) with moderate-to-low risk of bias were included. The pooled annualized rates of SCD were 0.65 per 1000 [95% confidence interval 0.00-6.43, I2 0.00%] in those with an implantable cardioverter-defibrillator (ICD) and 7.21 (2.38-13.79, I2 0.0%) in non-ICD cohorts: 7.14 in probands and 8.44 for 2010 Task Force Criteria (TFC). Multivariable predictors of life-threatening arrhythmic events including SCD were: age at presentation [adjusted hazard ratio 0.98 (0.97-0.99)], male sex [2.08 (1.29-3.36)], right ventricular (RV) dysfunction [6.99 (2.17-22.49)], QRS fragmentation [6.55 (3.33-12.90)], T-wave inversion [1.12 (1.02-1.24)], syncope at presentation [2.83 (2.40-4.08)], previous non-sustained ventricular tachyarrhythmia [2.53 (1.44-4.45)], and the TFC score [1.96 (1.02-3.76)], (P < 0.05). Predictors of appropriate ICD therapy were RV dysfunction, syncope, and inducible ventricular arrhythmia (P < 0.01).Conclusion: This meta-analysis demonstrates a high burden of SCD in ARVC patients, especially among probands and ARVC defined by the modified TFC. Better strategies are required to improve patient management and prevent SCD in ARVC. PROSPERO ID: CRD42020211761. [ABSTRACT FROM AUTHOR]

Background The indication for implantable cardioverter-defibrillators (ICDs) to prevent sudden death is now consolidated. However, there is no unanimous agreement on how patients experience certain aspects of their lives after an ICD implant. Various clinical studies have evaluated the impact of ICDs on patient mortality, survival, and their beneficial effects. Given the growing number of qualitative studies, a synthesis of qualitative evidence became desirable. Aims To explore and synthesize the results of qualitative studies addressing the experiences of patients with an ICD. Methods We employed a systematic review and meta-synthesis. PubMed, CINAHL, and PsycINFO were systematically searched to identify qualitative studies published between January 2005 and October 2020, which investigate the experience of patients living with an ICD. Selected studies were assessed for quality by two reviewers independently. Results A total of 24 papers were selected. Six themes emerged: fear and insecurity, the need for information, new impacts on life, living with ICD shocks, gender differences, and the role of the family. These results could help clinicians and researchers to develop new strategies and interventions to improve the quality of life in these patients. Conclusion This meta-synthesis underlines the difficulties experienced by patients with ICD implants. Despite the advanced therapeutic management programmes for this type of intervention, it is necessary to adopt and implement the current post-discharge care plans, where the family's involvement is essential to help patients adapt to their new lifestyle. [ABSTRACT FROM AUTHOR]

The implantable cardioverter-defibrillator (ICD) is a life-saving therapy in patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden cardiac death. The heterogeneity of clinical scenarios in HCM and the availability of ICDs with distinct characteristics emphasizes the need for selecting the right device for the right patient. There is growing awareness that unnecessarily complex devices can lead to short- and long-term complications without adding significant clinical benefits. Young patients have the greatest potential years of life gained from the ICD but are also most exposed to device-related complications. This increases the complexity of decision-making of ICD prescription in these often otherwise well patients in whom device selection should be tailored to preserve survival benefit without introducing morbidity. In the light of the multiple clinical phenotypes characterizing HCM, the present article offers evidence-based perspectives helpful in predicting the individual impact of the ICD and choosing the most appropriate device. [ABSTRACT FROM AUTHOR]

Background The atrial sites suitable for lead placement are limited after complex surgical atrial procedures, and lead placement can be challenging in patients with congenitally corrected transposition of the great arteries (ccTGA) after intracardiac repair. Case summary A 34-year-old man with ccTGA, who had undergone a double-switch operation with combined Senning and Jatene operations at the age of 14 was transferred to us. He experienced faintness and suffered cardiopulmonary arrest, and electrocardiography revealed ventricular fibrillation. After conversion to sinus rhythm by urgent external defibrillation, sinus bradycardia was revealed. Electrophysiological study was done using a three-dimensional (3D) mapping system (Ensite®) to evaluate the electrical condition of atria and to decide whether atrial lead can be transvenously placed. The electrical potential of the functional right atrium was good in the lateral or posterior wall, but the threshold was high. By contrast, the roof of the functional right atrium beyond cavoatrial junction was characterized by low voltage, but in a limited region of the roof of right atrium, the threshold was satisfactory and the electrical potential was normal. Thus, 3 weeks later, we implanted a transvenous implantable cardioverter-defibrillator (ICD). We used a 3D mapping system to place the atrial lead in the limited region of the roof of the right atrium mentioned above, the threshold was 0.7 V. Discussion Electrophysiological examination using a 3D mapping system before implantation of a dual-chamber ICD is useful because atrial sites suitable for lead placement are limited in patients. [ABSTRACT FROM AUTHOR]

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) causes ventricular arrhythmias (VAs) and sudden cardiac death (SCD). In 2019, a risk prediction model that estimates the 5-year risk of incident VAs in ARVC was developed (ARVCrisk.com). This study aimed to externally validate this prediction model in a large international multicentre cohort and to compare its performance with the risk factor approach recommended for implantable cardioverter-defibrillator (ICD) use by published guidelines and expert consensus. Methods and results In a retrospective cohort of 429 individuals from 29 centres in North America and Europe, 103 (24%) experienced sustained VA during a median follow-up of 5.02 (2.05–7.90) years following diagnosis of ARVC. External validation yielded good discrimination [C-index of 0.70 (95% confidence interval-CI 0.65–0.75)] and calibration slope of 1.01 (95% CI 0.99–1.03). Compared with the three published consensus-based decision algorithms for ICD use in ARVC (Heart Rhythm Society consensus on arrhythmogenic cardiomyopathy, International Task Force consensus statement on the treatment of ARVC, and American Heart Association guidelines for VA and SCD), the risk calculator performed better with a superior net clinical benefit below risk threshold of 35%. Conclusion Using a large independent cohort of patients, this study shows that the ARVC risk model provides good prognostic information and outperforms other published decision algorithms for ICD use. These findings support the use of the model to facilitate shared decision making regarding ICD implantation in the primary prevention of SCD in ARVC. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study is to evaluate whether the MADIT-ICD benefit score can predict who benefits most from the addition of implantable cardioverter-defibrillator (ICD) to cardiac resynchronization therapy (CRT) in real-world patients with heart failure with reduced ejection fraction (HFrEF) and to compare this with selection according to a multidisciplinary expert centre approach.Methods and Results: Consecutive HFrEF patients who received a CRT for a guideline indication at a tertiary care hospital (Ziekenhuis Oost-Limburg, Genk, Belgium) between October 2008 and September 2016, were retrospectively evaluated. The MADIT-ICD benefit groups (low, intermediate, and high) were compared with the current multidisciplinary expert centre approach. Endpoints were (i) sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) and (ii) non-arrhythmic mortality. Of the 475 included patients, 165 (34.7%) were in the lowest, 220 (46.3%) in the intermediate, and 90 (19.0%) in the highest benefit group. After a median follow-up of 34 months, VT/VF occurred in 3 (1.8%) patients in the lowest, 9 (4.1%) in the intermediate, and 13 (14.4%) in the highest benefit group (P < 0.001). Vice versa, non-arrhythmic death occurred in 32 (19.4%) in the lowest, 32 (14.6%) in the intermediate, and 3 (3.3%) in the highest benefit group (P = 0.002). The predictive power for ICD benefit was comparable between expert multidisciplinary judgement and the MADIT-ICD benefit score: Uno's C-statistic 0.69 vs. 0.69 (P = 0.936) for VT/VF and 0.62 vs. 0.60 (P = 0.790) for non-arrhythmic mortality.Conclusion: The MADIT-ICD benefit score can identify who benefits most from CRT-D and is comparable with multidisciplinary judgement in a CRT expert centre. [ABSTRACT FROM AUTHOR]

Sudden cardiac death (SCD) accounts for up to 25% of deaths in patients with congenital heart disease (CHD). To date, research has largely been driven by observational studies and real-world experience. Drawbacks include varying definitions, incomplete taxonomy that considers SCD as a unitary diagnosis as opposed to a terminal event with diverse causes, inconsistent outcome ascertainment, and limited data granularity. Notwithstanding these constraints, identified higher-risk substrates include tetralogy of Fallot, transposition of the great arteries, cyanotic heart disease, Ebstein anomaly, and Fontan circulation. Without autopsies, it is often impossible to distinguish SCD from non-cardiac sudden deaths. Asystole and pulseless electrical activity account for a high proportion of SCDs, particularly in patients with heart failure. High-quality cardiopulmonary resuscitation is essential to improve outcomes. Pulmonary hypertension and CHD complexity are associated with lower likelihood of successful resuscitation. Risk stratification for primary prevention implantable cardioverter-defibrillators (ICDs) should consider the probability of SCD due to a shockable rhythm, competing causes of mortality, complications of ICD therapy, and associated costs. Risk scores to better estimate probabilities of SCD and CHD-specific guidelines and consensus-based recommendations have been proposed. The subcutaneous ICD has emerged as an attractive alternative to transvenous systems in those with vascular access limitations, prior device infections, intra-cardiac shunts, or a Fontan circulation. Further improving SCD-related outcomes will require a multidimensional approach to research that addresses disease processes and triggers, taxonomy to better reflect underlying pathophysiology, high-risk features, early warning signs, access to high-quality cardiopulmonary resuscitation and specialized care, and preventive therapies tailored to underlying mechanisms. [ABSTRACT FROM AUTHOR]

Aims To report 5-year outcomes of EFFORTLESS registry patients with early generation subcutaneous implantable cardioverter-defibrillator (S-ICD) devices. Methods and results Kaplan–Meier, trend and multivariable analyses were performed for mortality and late (years 2–5) complications, appropriate shock (AS) and inappropriate shock (IAS) rates. Nine hundred and eighty-four of 994 enrolled patients with diverse diagnoses (28% female, 48 ± 17 years, body mass index 27 ± 6 kg/m2, ejection fraction 43 ± 18%) underwent S-ICD implantation. Median follow-up was 5.1 years (interquartile range 4.7–5.5 years). All-cause mortality was 9.3% (95% confidence interval 7.2–11.3%) at 5 years; 703 patients remained in follow-up on study completion, 171 withdrew including 87 (8.8%) with device explanted, and 65 (6.6%) lost to follow-up. Of the explants, only 20 (2.0%) patients needed a transvenous device for pacing indications. First and final shock efficacy for discrete ventricular arrhythmias was consistent at 90% and 98%, respectively, with storm episode final shock efficacy at 95.2%. Time to therapy remained unaltered. Overall 1- and 5-year complication rates were 8.9% and 15.2%, respectively. Early complications did not predict later complications. There were no structural lead failures. Inappropriate shock rates at 1 and 5 years were 8.7% and 16.9%, respectively. Self-terminating inappropriately sensed episodes predicted late IAS. Predictors of late AS included self-terminating appropriately sensed episodes and earlier AS. Conclusion In this diverse S-ICD registry population, spontaneous shock efficacy was consistently high over 5 years. Very few patients underwent S-ICD replacement with a transvenous device for pacing indications. Treated and self-terminating arrhythmic episodes predict future shock events, which should encourage more personalized device optimization. [ABSTRACT FROM AUTHOR]

Implantable cardioverter-defibrillator (ICD) may impact patients' life significantly. The aim of this survey was to analyse the impact of the ICD on quality of life (QoL) metrics from the patient's perspective. 'Living with an ICD' was a prospective, multicentre study with an online questionnaire submitted to the European Heart Rhythm Association (EHRA) Research Network centres as well as patient associations from 10 European countries; it was filled-in directly and personally by the patients that were invited to participate, with a minimal interaction or influence from the healthcare professionals. Overall, the questionnaire was completed by 1809 patients (624 women, 34.5%). Patients in their 60s and 70s and from Western Europe were the most represented. The median time from first ICD implantation was 5 years (IQR 2-10). Device-related complications were reported by 505 patients (22.4%), including one or more inappropriate shocks (n = 209, 11.6%). Almost half the respondents reported improved QoL, with a more favourable impact for those receiving cardiac resynchronization therapy-defibrillator (CRT-D), and only a 10th experienced a significant decrease in QoL. The occurrence of complications remained a major predictor of deteriorated QoL (odds ratio 2.1, 95% confidence interval 1.4-3.0, P < 0.001). In conclusion, most patients have a globally positive view and acceptance of ICD therapy, reporting preserved to improved QoL after device implantation. Complications, namely inappropriate shocks, affect the expectation of living a normal life post-implant and are associated with a significant decrease in QoL. Our findings also highlight the importance of a detailed informed consent process and the involvement of the patient in the decision-making process. [ABSTRACT FROM AUTHOR]

Aims: The aim of this study is to provide a thorough, quantified assessment of the substernal space as the site of extravascular implantable cardioverter-defibrillator (ICD) lead placement using computed tomography (CT) scans and summarizing adverse events and defibrillation efficacy across anatomical findings. Subcutaneous ICDs are an alternative to transvenous defibrillators but have limitations related to ICD lead distance from the heart. An alternative extravascular system with substernal lead placement has the potential to provide defibrillation at lower energy and pacing therapies from a single device.Methods and Results: A multi-centre, non-randomized, retrospective analysis of 45 patient CT scans quantitatively and qualitatively assessing bony, cardiac, vascular, and other organ structures from two human clinical studies with substernal lead placement. Univariate logistic regression was used to evaluate 15 anatomical parameters for impact on defibrillation outcome and adjusted for multiple comparisons. Adverse events were summarized. Substernal implantation was attempted or completed in 45 patients. Defibrillation testing was successful in 37 of 41 subjects (90%) using ≥10 J safety margin. There were two intra-procedural adverse events in one patient, including reaction to anaesthesia and an episode of transient atrial fibrillation during ventricular fibrillation induction. Anatomical factors associated with defibrillation failure included large rib cage width, myocardium extending very posteriorly, and a low heart position in the chest (P-values <0.05), though not significant adjusting for multiple comparisons.Conclusion: Retrospective analysis demonstrates the ability to implant within the substernal space with low intra-procedural adverse events and high defibrillation efficacy despite a wide range of anatomical variability. [ABSTRACT FROM AUTHOR]

Aim: The association of standard 12-lead electrocardiogram (ECG) markers with benefits of the primary prophylactic implantable cardioverter-defibrillator (ICD) has not been determined in the contemporary era. We analysed traditional and novel ECG variables in a large prospective, controlled primary prophylactic ICD population to assess the predictive value of ECG in terms of ICD benefit.Methods and Results: Electrocardiograms from 1477 ICD patients and 700 control patients (EU-CERT-ICD; non-randomized, controlled, prospective multicentre study; ClinicalTrials.gov Identifier: NCT02064192), who met ICD implantation criteria but did not receive the device, were analysed. The primary outcome was all-cause mortality. In ICD patients, the co-primary outcome of first appropriate shock was used. Mean follow-up time was 2.4 ± 1.1 years to death and 2.3 ± 1.2 years to the first appropriate shock. Pathological Q waves were associated with decreased mortality in ICD patients [hazard ratio (HR) 0.54, 95% confidence interval (CI) 0.35-0.84; P < 0.01] and patients with pathological Q waves had significantly more benefit from ICD (HR 0.44, 95% CI 0.21-0.93; P = 0.03). QTc interval increase taken as a continuous variable was associated with both mortality and appropriate shock incidence, but commonly used cut-off values, were not statistically significantly associated with either of the outcomes.Conclusion: Pathological Q waves were a strong ECG predictor of ICD benefit in primary prophylactic ICD patients. Excess mortality among Q wave patients seems to be due to arrhythmic death which can be prevented by ICD. [ABSTRACT FROM AUTHOR]

Background Minimizing right ventricular (RV) pacing to reduce the progression of heart failure is an established practice. Proprietary algorithms to reduce unnecessary RV pacing have been incorporated into both simple and complex cardiac pacemaker devices, for reducing the possibility of heart failure and arrhythmias. Case summary We present a case of a 43-year-old male implanted with a dual-chamber primary prevention implantable cardioverter-defibrillator (AUTOGEN EL, Boston Scientific) for sudden cardiac death. At the time of implant, the patient had hypertrophic cardiomyopathy with mild left ventricular (LV) systolic impairment, and sinus rhythm with intact atrioventricular (AV) conduction. The patient developed progression of his disease with symptoms (dyspnoea) and LV impairment. This led to a decision to activate the minimal RV pacing algorithm (RHYTHMIQ™). A deterioration in AV conduction caused intrinsic ventricular beats to fall in the atrial blanking period, and subsequent VVI backup pacing resulted in R on T pacing. This induced ventricular arrhythmia. RHYTHMIQ™ was subsequently deactivated, and the patient has had no further device-induced arrhythmias. Discussion Numerous studies have demonstrated the adverse effect of RV pacing on LV function. Minimizing RV pacing is, therefore, encouraged in individuals with intact AV conduction. However, underlying conduction abnormalities must be assessed prior to activating algorithms designed to minimize RV pacing. This case demonstrates the importance of careful intracardiac electrogram interpretation and individual case-based device programming, to avoid device-induced complications. [ABSTRACT FROM AUTHOR]

More than 40 years after the first implantable cardioverter-defibrillator (ICD) implantation, sudden cardiac death (SCD) still accounts for more than five million deaths worldwide every year. Huge efforts in the field notwithstanding, it is now increasingly evident that the current strategy of long-term prevention based on left ventricular ejection fraction as the key selection criterion is actually of very limited impact, also because the largest absolute numbers of SCD are encountered in the general population not known to be at risk. It has been recently reemphasized that SCD is often not so sudden, with almost half of the victims experiencing typical warning symptoms preceding the event. Importantly, heeded and prompt medical attention can dramatically improve survival. Essentially, such timely action increases the chances of the SCD event being witnessed by emergency medical services and provides the opportunity for early intervention. In addition, newer technologies incorporating digital data acquisition, transfer between interconnected devices, and artificial intelligence, should allow dynamic, real-time monitoring of diverse parameters and therefore better identification of subjects at short-term SCD risk. Along with warning symptoms, these developments allow a new approach of near-term prevention based on the hours and minutes preceding SCD. In the present review, we challenge the current paradigm of mid- and long-term prevention using ICD in patients at the highest risk of SCD, and introduce a complementary concept applicable to the entire population that would aim to pre-empt SCD by timely detection and intervention within the minutes or hours prior to the event. [ABSTRACT FROM AUTHOR]

Aims: Risk stratification of patients with long QT syndrome (LQTS) represents a difficult task. In 2018, we proposed a granular estimate of the baseline 5-year risk of life-threatening arrhythmias (LAE) for patients with LQTS, based on the genotype (long QT syndrome Type 1, long QT syndrome Type 2, and long QT syndrome Type 3) and the duration of the QTc interval. We sought to externally validate a novel risk score model (1-2-3-LQTS-Risk model) in a geographically diverse cohort from the USA and to evaluate its performance and assess potential clinical implication of this novel model.Methods and Results: The prognostic model (1-2-3-LQTS-Risk model) was derived using data from a prospective, single-centre longitudinal cohort study published in 2018 (discovery cohort) and was validated using an independent cohort of 1689 patients enrolled in the International LQTS Registry (Rochester NY, USA). The validation study revealed a C-index of 0.69 [95% confidence interval (CI): 0.61-0.77] in the validation cohort, when compared with C-index of 0.79 (95% CI: 0.70-0.88) in the discovery cohort. Adopting a 5-year risk ≥5%, as suggested by the ROC curve analysis as the most balanced threshold for implantable cardioverter-defibrillator (ICD) implantation, would result in a number needed to treat (NNT) of nine (NNT = 9; 95% CI: 6.3-13.6).Conclusion: The 1-2-3-LQTS-Risk model, the first validated 5-year risk score model for patients with LQTS, can be used to aid clinicians to identify patients at the highest risk of LAE who could benefit most from an ICD implant and avoid unnecessary implants. [ABSTRACT FROM AUTHOR]

Worldwide, ∼4 million people die from sudden cardiac death every year caused in more than half of the cases by ischaemic cardiomyopathy (ICM). Prevention of sudden cardiac death after myocardial infarction by implantation of a cardioverter-defibrillator (ICD) is the most common, even though not curative, therapy to date. Optimized ICD programming should be strived for in order to decrease the incidence of ICD interventions. Catheter ablation reduces the recurrence of ventricular tachycardias (VTs) and is an important adjunct to sole ICD-based treatment or pharmacological antiarrhythmic therapy in patients with ICM, as conclusively demonstrated by seven randomized controlled trials (RCTs) in the last two decades. However, none of the conducted trials was powered to reveal a survival benefit for ablated patients as compared to controls. Whereas thorough consideration of an early approach is necessary following two recent RCTs (PAUSE-SCD, BERLIN VT), catheter ablation is particularly recommended in patients with recurrent VT after ICD therapy. In this context, novel, pathophysiologically driven ablation strategies referring to deep morphological and functional substrate phenotyping based on high-resolution mapping and three-dimensional visualization of scars appear promising. Emerging concepts like sympathetic cardiac denervation as well as radioablation might expand the therapeutical armamentarium especially in patients with therapy-refractory VT. Randomized controlled trials are warranted and on the way to investigate how these translate into improved patient outcome. This review summarizes therapeutic strategies currently available for the prevention of VT recurrences, the optimal timing of applicability, and highlights future perspectives after a PAUSE in BERLIN. [ABSTRACT FROM AUTHOR]

Patients with cardiac implantable electronic devices (CIED) undergoing radiotherapy (RT) are more common due to the ageing of the population. With newer CIEDs' implementing the complementary metal-oxide semiconductor (CMOS) technology which allows the miniaturization of CIED, it is also more susceptible to RT. Effects of RT on CIED ranges from device interference, device operational/memory errors of permanent damage. These malfunctions can cause life-threatening clinical effects. Cumulative dose is not the only component of RT that causes CIED malfunction, as neutron use and dose rate effect also affects CIEDs. The management of this patient cohort in clinical practice is inconsistent due to the lack of a consistent guideline from manufacturers and physician specialty societies. Our review will focus on the current clinical practice and the recently updated guidelines of managing patients with CIED undergoing RT. We aim to simplify the evidence and provide a simple and easy to use guide based on the recent guidelines. [ABSTRACT FROM AUTHOR]

Aims: In patients undergoing cardiac implantable electronic device (CIED) intervention, routine pre-procedure antibiotic prophylaxis is recommended. A more powerful antibiotic protocol has been suggested in patients at high risk of infection. Stratification of individual infective risk could guide the prophylaxis before CIED procedure.Methods and Results: Patients undergoing CIED surgery were stratified according to the Shariff score in low and high infective risk. Patients in the 'low-risk' group were treated with only two antibiotic administrations while patients in the 'high-risk' group were treated with a prolonged 9-day protocol, according to renal function and allergies. We followed-up patients for 250 days with clinical outpatient visit and electronic control of the CIED. As primary endpoint, we evaluated CIED-related infections. A total of 937 consecutive patients were enrolled, of whom 735 were stratified in the 'low-risk' group and 202 in the 'high-risk' group. Despite different risk profiles, CIED-related infection rate at 250 days was similar in the two groups (8/735 in 'low risk' vs. 4/202 in 'high risk', P = 0.32). At multivariate analysis, active neoplasia, haematoma, and reintervention were independently associated with CIED-related infection (HR 5.54, 10.77, and 12.15, respectively).Conclusion: In a large cohort of patients undergoing CIED procedure, an antibiotic prophylaxis based on individual stratification of infective risk resulted in similar rate of infection between groups at high and low risk of CIED-related infection. [ABSTRACT FROM AUTHOR]

Aims: Cardiac resynchronization therapy (CRT) reoperations are associated with a particularly high risk of device-related infection (DRI). An antibacterial envelope reduces the occurrence of DRIs in a broad population of moderate-tohigh-risk patients. To investigate the efficacy of an antibacterial envelope in a very high-risk population of patients undergoing CRT reoperation.Methods and Results: In this Danish two-centre, observational cohort study, we included consecutive patients who underwent a CRT pacemaker- or defibrillator reoperation procedure between January 2008 and November 2019. We obtained data from the Danish Pacemaker and ICD Register and through systematic medical chart review. Follow-up was restricted to 2 years. A total of 1943 patients were included in the study of which 736 (38%) received an envelope. Envelope patients had more independent risk factors for infection than non-envelope patients. Sixty-seven (3.4%) patients met the primary endpoint of DRI requiring device system extraction; 50 in the non-envelope group and 17 in the envelope group [4.1% vs. 2.3%, adjusted hazard ratio (HR) 0.52, 95% confidence interval (CI) 0.30-0.90; P = 0.021]. This difference persisted in propensity score analysis (HR 0.51, 95% CI 0.29-0.90; P = 0.019).Conclusion: Use of an antibacterial envelope was associated with a clinically and statistically significant reduction in DRIs in patients undergoing CRT reoperations. Our results were comparable to those recently reported from a large randomized controlled trial, which is suggestive of a proportional effect of the envelope even in very high-risk patients. [ABSTRACT FROM AUTHOR]

Background and objective Balance between benefit and burden of implantable cardioverter-defibrillator (ICD) therapy is more debatable in older patients, compared to younger patients. Of around 6000 yearly implanted ICDs in the Netherlands, 1:4 is received by patients ≥75 years. We aimed to evaluate the current clinical practice in the Netherlands for ICD implants and generator replacements, with a special focus on the older ICD patients. Research design and methods Cardiologists from all Dutch ICD implanting centres (n  = 28) were interviewed. Questions aimed to evaluate outpatient care, pre-operative patient assessment, end-of-life-care counselling, evaluation of social and cognitive wellbeing, clinical evaluation of all patients prior to ICD replacement, and the consideration of the option to downgrade or not replace a device. Results Implanting cardiologists from all 28 implanting centres were approached for an interview. Response rate was 86%. Management appeared diverse. An age ≥80 years was consistently reported as incentive for more extensive patient evaluation. Patients were invited for counselling prior to device replacements in only the minority (46%) of hospitals. Downgrade or non-replacement was performed in rare cases. End-of-life care discussions were not standard procedure in 67% of the hospitals. Evaluation of social and cognitive wellbeing of patients was based solely on the general clinical impression of the physician in 83%, or not at all assessed in 8% of the centres. Discussion and implication A structured framework for care and evaluation of cognitive and/or physical limitations is currently absent in most hospitals. At time of ICD (re-)evaluation, several factors may be considered before deciding on (continuation of) ICD therapy: patient preferences and comorbidity, the need for pacemaker therapy, primary vs. secondary prevention, procedural risks, and patient preferences. [ABSTRACT FROM AUTHOR]

Aims Implantable cardioverter-defibrillator (ICD) treatment has expanded due to its effectiveness. However, there are concerns about complications, and use in the most elderly has been questioned. There is scarce data on qualitative aspects regarding experiences of living with an ICD among patients above the age of 80 years. The aim of this study was to explore octogenarians' experience, knowledge, and attitude of living with an ICD. Method and results We used semi-structured interviews to collect data from 20 patients, aged 80–89 years (90% men). The data were then structured and analysed through deductive thematic analysis network approach using the ATLAS.ti v.8.4 software. The framework of the analysis was based on the Successful Aging Theory. Three main themes emerged regarding the patients' experiences: Life goes on; Their Health , The Janus-Faced Device; Their attitudes , and Mind the gap ; Their knowledge. None of the octogenarians regretted receiving their ICD, instead, they presented with feelings such as gratitude towards the device. However, a lack of knowledge about the devices was expressed, which can be a risk for potential complications, in turn causing suffering and unnecessary concerns. Conclusion Overall, the ICD did not pose a threat towards successful ageing. It was mainly considered a life-saving device. However, the lack of knowledge might hinder informed choices close to death and contribute to ethical dilemmas when deactivation of the ICD is a reasonable option. [ABSTRACT FROM AUTHOR]

Aims: Remote patient monitoring (RPM) systems offer a promising alternative to conventional In-Clinic check-ups, hereby reducing unnecessary clinic visits. Especially with the rise of the COVID-19 pandemic, this reduction is of paramount importance. Regarding the association between RPM and clinical outcomes, findings of previous studies have been inconsistent. The aim of this study is to elucidate the effect of partly substituting In-Clinic visits by RPM on clinical outcomes in implantable cardioverter-defibrillator (ICD) patients.Methods and Results: The study included 595 heart failure patients (LVEF ≤35%; NYHA Class II/III) implanted with an ICD compatible with the Boston Scientific LATITUDE™ system. Participants were randomized to RPM plus an annual In-Clinic visit or 3-6 months In-Clinic check-ups alone. The investigated endpoints after 2 years of follow-up included a composite of all-cause mortality and cardiac hospitalization, mortality and cardiac hospitalization as independent endpoints and ICD therapy. The incidence of mortality and hospitalization did not differ significantly as independent, nor as composite endpoint between the RPM and In-Clinic group (all Ps <0.05). The results were similar regarding ICD therapy, except for appropriate ICD therapy (odds ratio 0.50; 95% confidence interval 0.26-0.98; P = 0.04). Exploratory subgroup analyses indicated that the effect of RPM differs between patients with specific characteristics, i.e. ≥60 years and permanent atrial fibrillation (all Ps < 0.05).Conclusion: RPM is non-inferior to conventional In-Clinic visits regarding clinical outcomes. Routine In-Clinic follow-up may partly be substituted by RPM without jeopardizing safety and efficiency, and thus reducing unnecessary In-Clinic visits.Clinicaltrials.gov Identifier: NCT01691586. [ABSTRACT FROM AUTHOR]

Aims: We developed and validated an algorithm for prediction of heart failure (HF) hospitalizations using remote monitoring (RM) data transmitted by implanted defibrillators.Methods and Results: The SELENE HF study enrolled 918 patients (median age 69 years, 81% men, median ejection fraction 30%) with cardiac resynchronization therapy (44%), dual-chamber (38%), or single-chamber defibrillators with atrial diagnostics (18%). To develop a predictive algorithm, temporal trends of diurnal and nocturnal heart rates, ventricular extrasystoles, atrial tachyarrhythmia burden, heart rate variability, physical activity, and thoracic impedance obtained by daily automatic RM were combined with a baseline risk-stratifier (Seattle HF Model) into one index. The primary endpoint was the first post-implant adjudicated HF hospitalization. After a median follow-up of 22.5 months since enrolment, patients were randomly allocated to the algorithm derivation group (n = 457; 31 endpoints) or algorithm validation group (n = 461; 29 endpoints). In the derivation group, the index showed a C-statistics of 0.89 [95% confidence interval (CI): 0.83-0.95] with 2.73 odds ratio (CI 1.98-3.78) for first HF hospitalization per unitary increase of index value (P < 0.001). In the validation group, sensitivity of predicting primary endpoint was 65.5% (CI 45.7-82.1%), median alerting time 42 days (interquartile range 21-89), and false (or unexplained) alert rate 0.69 (CI 0.64-0.74) [or 0.63 (CI 0.58-0.68)] per patient-year. Without the baseline risk-stratifier, the sensitivity remained 65.5% and the false/unexplained alert rates increased by ≈10% to 0.76/0.71 per patient-year.Conclusion: With the developed algorithm, two-thirds of first post-implant HF hospitalizations could be predicted timely with only 0.7 false alerts per patient-year. [ABSTRACT FROM AUTHOR]

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients have an increased risk of ventricular arrhythmias (VA). Four implantable cardioverter-defibrillator (ICD) recommendation algorithms are available The International Task Force Consensus ('ITFC'), an ITFC modification by Orgeron et al. ('mITFC'), the AHA/HRS/ACC guideline for VA management ('AHA'), and the HRS expert consensus statement ('HRS'). This study aims to validate and compare the performance of these algorithms in ARVC.Methods and Results: We classified 617 definite ARVC patients (38.5 ± 15.1 years, 52.4% male, 39.2% prior sustained VA) according to four algorithms. Clinical performance was evaluated by sensitivity, specificity, ROC-analysis, and decision curve analysis for any sustained VA and for fast VA (>250 b.p.m.). During 6.4 [2.8-11.5] years follow-up, 282 (45.7%) patients experienced any sustained VA, and 63 (10.2%) fast VA. For any sustained VA, ITFC and mITFC provide higher sensitivity than AHA and HRS (94.0-97.8% vs. 76.7-83.5%), but lower specificity (15.9-32.0% vs. 42.7%-60.1%). Similarly, for fast VA, ITFC and mITFC provide higher sensitivity than AHA and HRS (95.2-97.1% vs. 76.7-78.4%) but lower specificity (42.7-43.1 vs. 76.7-78.4%). Decision curve analysis showed ITFC and mITFC to be superior for a 5-year sustained VA risk ICD indication threshold between 5-25% or 2-9% for fast VA.Conclusion: The ITFC and mITFC provide the highest protection rates, whereas AHA and HRS decrease unnecessary ICD placements. ITFC or mITFC should be used if we consider the 5-year threshold for ICD indication to lie within 5-25% for sustained VA or 2-9% for fast VA. These data will inform decision-making for ICD placement in ARVC. [ABSTRACT FROM AUTHOR]

Aims: Treatment with implantable cardioverter-defibrillators (ICD) is a cornerstone for prevention of sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy (ARVC). We aimed at describing the complications associated with ICD treatment in a multinational cohort with long-term follow-up.Methods and Results: The Nordic ARVC registry was established in 2010 and encompasses a large multinational cohort of ARVC patients, including their clinical characteristics, treatment, and events during follow-up. We included 299 patients (66% males, median age 41 years). During a median follow-up of 10.6 years, 124 (41%) patients experienced appropriate ICD shock therapy, 28 (9%) experienced inappropriate shocks, 82 (27%) had a complication requiring surgery (mainly lead-related, n = 75), and 99 (33%) patients experienced the combined endpoint of either an inappropriate shock or a surgical complication. The crude rate of first inappropriate shock was 3.4% during the first year after implantation but decreased after the first year and plateaued over time. Contrary, the risk of a complication requiring surgery was 5.5% the first year and remained high throughout the study period. The combined risk of any complication was 7.9% the first year. In multivariate cox regression, presence of atrial fibrillation/flutter was a risk factor for inappropriate shock (P < 0.05), whereas sex, age at implant, and device type were not (all P > 0.05).Conclusion: Forty-one percent of ARVC patients treated with ICD experienced potentially life-saving ICD therapy during long-term follow-up. A third of the patients experienced a complication during follow-up with lead-related complications constituting the vast majority. [ABSTRACT FROM AUTHOR]

Aims: Randomized data on the efficacy/safety of cardiac resynchronization therapy with vs. without defibrillator (CRT-D,-P) in heart failure with reduced ejection fraction (HFrEF) are scarce. We aimed to evaluate survival associated with use of CRT-D vs. CRT-P in a contemporary cohort with HFrEF.Methods and Results: Patients from Swedish HF Registry treated with CRT-D/CRT-P and fulfilling criteria for primary prevention defibrillator use were included. Logistic regression was used to evaluate predictors of CRT-D non-use. All-cause mortality was compared in CRT-D vs. CRT-P by Cox regression in a 1 : 1 propensity-score-matched cohort. Of 1988 patients with CRT, 1108 (56%) had CRT-D and 880 (44%) CRT-P. Older age, higher ejection fraction (EF), female sex, and the lack of referral to HF nurse-led outpatient clinic were major determinants of CRT-D non-use. After matching, 645 CRT-D patients were compared with 645 with CRT-P. The CRT-D use was associated with lower 1- and 3-year all-cause mortality [hazard ratio (HR):0.76, 95% confidence interval (CI):0.58-0.98; HR: 0.82, 95% CI: 0.68-0.99, respectively]. Results were consistent in all pre-specified subgroups except for CRT-D use being associated with lower 3-year mortality in patients with an EF < 30% but not in those with an EF ≥ 30% (HR: 0.73, 95% CI: 0.59-0.89 and HR: 1.24, 95% CI: 0.83-1.85, respectively; P-interaction = 0.02).Conclusion: In a contemporary HFrEF cohort, CRT-D was associated with lower mortality compared with CRT-P. The CRT-D use was less likely in older patients, females, and in patients not referred to HF nurse-led outpatient clinic. Our findings support the use of CRT-D vs. CRT-P in HFrEF, in particular with severely reduced EF. [ABSTRACT FROM AUTHOR]

Background Calmodulinopathy is an emerging group of primary electrical disease with various, severe, and early onset phenotype. Sudden cardiac arrest (SCA)/death can be the first symptom and current medical management seems insufficient to prevent recurrences. Implantable cardioverter-defibrillator (ICD) in the young is challenging and can be harmful. Case summary We report the management of two very young boys (aged 3.5 and 5.5 years old) who survived an SCA due to calmodulin mutation responsible of a catecholaminergic polymorphic ventricular tachycardia phenotype. In both case, SCA had an adrenergic trigger. Despite SCA, ICD implantation was denied by the parents. After thorough discussion with the family, the patients were managed with solely betablocker treatment and loop recorder implantation. At last follow-up of 30 and 23 months, respectively, there were no recurrence of any cardiac event. Discussion The benefits of ICD implantation at a very young age must be weighed against the risk complication. In the youngest, whom recreative activities are under constant supervision, the decision, jointly made with the parents, could be to postpone ICD. [ABSTRACT FROM AUTHOR]

Aims: The roles of implantable cardioverter-defibrillators (ICDs) and radiofrequency catheter ablation (RCA) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and well-tolerated monomorphic ventricular tachycardia (MVT) are debated. In this multicentre retrospective study, we aimed at reporting the outcome of selected patients with ARVC after RCA without a back-up ICD.Methods and Results: Patients with ARVC who underwent RCA of well-tolerated MVT at 10 tertiary centres across 5 countries, without an ICD before and 3 months after RCA, without syncope or electrical storm, and with left ventricular ejection fraction ≥50% were included. In total, 65 ARVC patients [mean age 44.5 ± 13.2 years, 78% males] underwent RCA of MVT between 2003 and 2016. Clinical presentation was palpitations in 51 (80%) patients. One (2%) patient had >1 clinical MVT. At the ablative procedure, clinical MVTs (mean rate 185 ± 32 b.p.m.) were inducible in 50 (81%) patients. Epicardial ablation was performed in 19 (29%) patients. Complete acute success was achieved in 47 (72%) patients. After a median follow-up of 52.4 months (range 12.3-171.4), there was no death or aborted cardiac arrest, and VT recurred in 19 (29%) patients. Survival without VT recurrence was estimated at 88%, 80%, and 68%, 12, 36, and 60 months after RCA, respectively, and was significantly associated with the approach and the procedural outcome.Conclusion : In patients with ARVC, well-tolerated MVT without a back-up ICD did not lead to fatal arrhythmic event after RCA despite VT recurrences in some. Our data suggest that RCA may be an alternative to ICD in selected ARVC patients. [ABSTRACT FROM AUTHOR]

Aim: The Prevention of Arrhythmia Device Infection Trial (PADIT) infection risk score, developed based on a large prospectively collected data set, identified five independent predictors of cardiac implantable electronic device (CIED) infection. We performed an independent validation of the risk score in a data set extracted from U.S. healthcare claims.Methods and Results: Retrospective identification of index CIED procedures among patients aged ≥18 years with at least one record of a CIED procedure between January 2011 and September 2014 in a U.S health claims database. PADIT risk factors and major CIED infections (with system removal, invasive procedure without system removal, or infection-attributable death) were identified through diagnosis and procedure codes. The data set was randomized by PADIT score into Data Set A (60%) and Data Set B (40%). A frailty model allowing multiple procedures per patient was fit using Data Set A, with PADIT score as the only predictor, excluding patients with prior CIED infection. A data set of 54 042 index procedures among 51 623 patients with 574 infections was extracted. Among patients with no history of prior CIED infection, a 1 unit increase in the PADIT score was associated with a relative 28% increase in infection risk. Prior CIED infection was associated with significant incremental predictive value (HR 5.66, P < 0.0001) after adjusting for PADIT score. A Harrell's C-statistic for the PADIT score and history of prior CIED infection was 0.76.Conclusion: The PADIT risk score predicts increased CIED infection risk, identifying higher risk patients that could potentially benefit from targeted interventions to reduce the risk of CIED infection. Prior CIED infection confers incremental predictive value to the PADIT score. [ABSTRACT FROM AUTHOR]

Background Cardiac implanted electronic devices (CIED) have significantly improved the survival and quality of life in heart failure patients. Although implantable cardioverter-defibrillators (ICD) and cardiac resynchronization therapy (CRT) have a major role in patients with moderate to severe heart failure symptoms, the role of these devices in patients with a left ventricular assist device (LVAD) is not yet well defined. The burden of CIED-related procedures in patients with an LVAD is high. The price of lead malfunctions and pocket complications requires creative approaches to tackle CIED-related issues in this patient population. Case summary Here, we describe the clinical course of a 67-year-old ventricular pacing dependent LVAD patient with an ICD indication based on recurrent monomorphic ventricular tachycardias and a CRT indication due to previous deterioration of (right-sided) heart failure in the absence of biventricular pacing. We were confronted with impending right ventricular lead failure and bilateral venous access problems due to chronic subclavian vein occlusion in a patient with a total of five transvenous leads, therapeutic anticoagulation, and pronounced thoracic collaterals. We sought for a creative solution to be able to deliver effective biventricular fusion pacing with the existing leads from two contralateral pulse generators resulting in biventricular fusion pacing. This provided the solution to deliver effective CRT. Discussion This case illustrates the complexity of care and CIED-related decision-making in pacing dependent LVAD patients, in particularly those with an ICD and CRT indication. [ABSTRACT FROM AUTHOR]

Background Magnetic resonance (MR) imaging (MRI) for patients with implantable cardiac devices is becoming more routine, with the development of MR conditional devices allowing more patients access to the imaging they need. However, for this to be performed safely, strict protocols must be followed necessitating close collaboration between cardiology and radiology departments. We present a case where mandatory device re-programming of a cardiac resynchronization therapy defibrillator device into MRI mode was not performed pre-scan leading to temporary device dysfunction with no clinical consequences. Case summary A 72-year-old man presented to a device clinic for a routine device interrogation. An atrial tachycardia response episode was recorded at the same time as the patient reported having undergone an MRI scan at a local centre. The electrogram demonstrated temporary right ventricular loss of capture with standard output programming, and a short episode of oversensing on the atrial and ventricular channel which was not sustained for long enough to meet tachycardia detection. Discussion We demonstrate two potential electrophysiological effects of MRI on pacemakers, where the device had not been appropriately re-programmed pre-procedure. This illustrates that whilst MRI in patients with implantable cardiac devices is safe, strict protocols must be followed requiring robust multidisciplinary communication. [ABSTRACT FROM AUTHOR]

Aims: Cardiac implantable electronic devices with device advisories have the potential of device malfunction. Remote monitoring (RM) of devices has been suggested to allow the identification of abnormal device performance and permit early intervention. We sought to describe the outcomes of patients with and without RM in devices subject to the Abbott Premature Battery Depletion (PBD) advisory with data from a Canadian registry.Methods and Results: Patients with an Abbott device subject to the PBD advisory from nine implantable cardioverter defibrillator (ICD) implanting centres in Canada were included in the registry. The use of RM was identified from baseline and follow-up data in the registry. The primary outcome was detection of PBD and all-cause mortality. A total of 2666 patients were identified with a device subject to the advisory. In all, 1687 patients (63.2%) had RM at baseline. There were 487 deaths during follow-up. At a mean follow-up of 5.7 ± 0.7 years, mortality was higher in those without a remote monitor compared with RM at baseline (24.7% vs. 14.5%; P < 0.001). Pre-mature battery depletion was identified in 36 patients (2.1%) with RM vs. 7 (0.7%) without RM (P = 0.004). Time to battery replacement was significantly reduced in patients on RM (median 5 vs. 13 days, P = 0.001).Conclusion: The use of RM in patients with ICD and cardiac resynchronization therapy under advisory improved detection of PBD, time to device replacement, and was associated with a reduction in all-cause mortality. The factors influencing the association with mortality are unknown and deserve further study. [ABSTRACT FROM AUTHOR]

Aims: Response to cardiac resynchronization therapy (CRT) is associated with improved survival, and reduction in heart failure hospitalization, and ventricular arrhythmia (VA) risk. However, the impact of CRT super-response [CRT-SR, increase in left ventricular ejection fraction (LVEF) to ≥ 50%] on VA remains unclear.Methods and Results: We undertook a meta-analysis aimed at determining the impact of CRT response and CRT-SR on risk of VA and all-cause mortality. Systematic search of PubMed, EMBASE, and Cochrane databases, identifying all relevant English articles published until 31 December 2019. A total of 34 studies (7605 patients for VA and 5874 patients for all-cause mortality) were retained for the meta-analysis. The pooled cumulative incidence of appropriate implantable cardioverter-defibrillator therapy for VA was significantly lower at 13.0% (4.5% per annum) in CRT-responders, vs. 29.0% (annualized rate of 10.0%) in CRT non-responders, relative risk (RR) 0.47 [95% confidence interval (CI) 0.39-0.56, P < 0.0001]; all-cause mortality 3.5% vs. 9.1% per annum, RR of 0.38 (95% CI 0.30-0.49, P < 0.0001). The pooled incidence of VA was significantly lower in CRT-SR compared with CRT non-super-responders (non-responders + responders) at 0.9% vs. 3.8% per annum, respectively, RR 0.22 (95% CI 0.12-0.40, P < 0.0001); as well as all-cause mortality at 2.0% vs. 4.3%, respectively, RR 0.47 (95% CI 0.33-0.66, P < 0.0001).Conclusions: Cardiac resynchronization therapy super-responders have low absolute risk of VA and all-cause mortality. However, there remains a non-trivial residual absolute risk of these adverse outcomes in CRT responders. These findings suggest that among CRT responders, there may be a continued clinical benefit of defibrillators. [ABSTRACT FROM AUTHOR]

Aims: The aim of this registry was to evaluate the additional prognostic value of a composite cardiac magnetic resonance (CMR)-based risk score over standard-of-care (SOC) evaluation in a large cohort of consecutive unselected non-ischaemic cardiomyopathy (NICM) patients.Methods and Results: In the DERIVATE registry (www.clinicaltrials.gov/registration: RCT#NCT03352648), 1000 (derivation cohort) and 508 (validation cohort) NICM patients with chronic heart failure (HF) and left ventricular ejection fraction <50% were included. All-cause mortality and major adverse arrhythmic cardiac events (MAACE) were the primary and secondary endpoints, respectively. During a median follow-up of 959 days, all-cause mortality and MAACE occurred in 72 (7%) and 93 (9%) patients, respectively. Age and >3 segments with midwall fibrosis on late gadolinium enhancement (LGE) were the only independent predictors of all-cause mortality (HR: 1.036, 95% CI: 1.0117-1.056, P < 0.001 and HR: 2.077, 95% CI: 1.211-3.562, P = 0.008, respectively). For MAACE, the independent predictors were male gender, left ventricular end-diastolic volume index by CMR (CMR-LVEDVi), and >3 segments with midwall fibrosis on LGE (HR: 2.131, 95% CI: 1.231-3.690, P = 0.007; HR: 3.161, 95% CI: 1.750-5.709, P < 0.001; and HR: 1.693, 95% CI: 1.084-2.644, P = 0.021, respectively). A composite clinical and CMR-based risk score provided a net reclassification improvement of 63.7% (P < 0.001) for MAACE occurrence when added to the model based on SOC evaluation. These findings were confirmed in the validation cohort.Conclusion: In a large multicentre, multivendor cohort registry reflecting daily clinical practice in NICM work-up, a composite clinical and CMR-based risk score provides incremental prognostic value beyond SOC evaluation, which may have impact on the indication of implantable cardioverter-defibrillator implantation. [ABSTRACT FROM AUTHOR]