Search Results (66)

Introduction: Sudden cardiac death in children and adolescents is a devastating yet potentially preventable event. There is, therefore, an urgent need for early recognition of young patients at elevated cardiac risk. Electrocardiographic screening may play a central role in this effort. However, interpreting paediatric ECGs requires advanced diagnostic skills. Checklists have been advocated to mitigate errors in a number of complex fields, both medical and non-medical; however, their effectiveness in interpreting paediatric ECGs remains uncertain. Objective: To evaluate whether the use of a structured checklist improves primary care paediatricians’ performance in interpreting paediatric ECGs, and to assess the influence of professional experience on diagnostic accuracy. Methods: We conducted a prospective, parallel-group study involving primary care paediatricians in the Balearic Islands (Spain). Participants were randomly assigned to interpret paediatric ECGs, either using routine unstructured interpretation or with checklist support. Outcomes included diagnostic validity ratios, and appropriateness of referral to paediatric cardiologists. Performance was analysed according to checklist use or years of clinical experience. It was also analysed whether diagnostic accuracy varied according to whether the ECG was classified as normal, or abnormal with or without an increased risk of sudden cardiac death. Results: Thirty-one paediatricians completed the study, generating 310 ECG interpretations. Checklist use did not significantly improve sensitivity, specificity, or likelihood ratios for detecting ECG abnormalities associated with sudden cardiac death risk, nor did it increase appropriate referral rates. We report a trend towards higher specificity and likelihood ratios among paediatricians with fewer than 20 years of professional experience than among their more senior counterparts. Significantly, this study found true positive rates of normal ECGs, and of abnormal ECGs with risk of sudden cardiac death, to be around 90%, as opposed to true positive rates of around 60% for abnormal ECGs without risk of sudden cardiac death (91% vs. 85% vs. 59%, respectively). Conclusions: In this study, checklist support did not enhance diagnostic performance in paediatric ECG interpretation. This finding highlights the need for targeted efforts to improve diagnostic accuracy in this sensitive subset. We also report that mid-career paediatricians appear to achieve the highest ECG diagnostic accuracy, and we show a particularly high rate of correct interpretation of both simple ECGs and high-risk abnormal ECGs. Full article

Objectives: Sudden cardiac death (SCD) is a leading cause of mortality, accounting for approximately 50% of all cardiovascular deaths and 20% of all-natural deaths in Western countries. In individuals over 50 years of age, coronary artery disease (CAD) is responsible for more than 80% of cases, whereas in younger subjects SCD is more frequently associated with non-ischemic myocardial diseases, including hypertrophic cardiomyopathy (HCM), arrhythmogenic cardiomyopathy (ACM), dilated cardiomyopathy (DCM), and myocarditis. Additional causes in young adults include coronary artery anomalies and primary arrhythmic disorders related to channelopathies. This study evaluated the diagnostic performance of post-mortem cardiac magnetic resonance imaging (PM-CMR) in identifying morphological substrates underlying SCD in formalin-fixed explanted hearts, with particular attention to the concordance between PM-CMR findings and autopsy results in cases of sudden coronary death. Material and Methods: We retrospectively reviewed 110 PM-CMR examinations from the Regional Register of Sudden Cardiac Death of Friuli-Venezia Giulia, of which 101 were included in the final analysis. Results: PM-CMR detected pathological findings in 60 hearts (59%), including acute ischemic lesions in 39 cases and other conditions, such as hypertrophic cardiomyopathy, chronic fibrotic ischemic changes, and adipose metaplasia in 21 cases. A good agreement between PM-CMR and autopsy findings was observed (Cohen’s kappa = 0.8). Conclusions: Overall, PM-CMR proved effective in identifying relevant morphological and signal alterations, supporting conventional autopsy. Despite some limitations, particularly in hyperacute ischemic lesions, PM-CMR appears to play a promising role in the diagnostic work-up of SCD and in supporting family screening programs for primary prevention. Full article

Hypertrophic Cardiomyopathy (HCM) is one of the most common inherited cardiomyopathies and remains an important cause of ventricular arrhythmias and sudden cardiac death (SCD), particularly in younger individuals. Although the annual incidence of arrhythmic death is relatively low in contemporary cohorts, identifying those patients who may benefit from primary prevention with an implantable cardioverter-defibrillator (ICD) remains a major clinical challenge. Current risk stratification strategies rely on two principal paradigms. The European approach is centered on the HCM Risk-SCD score, whereas the American approach is mainly based on major clinical risk markers. Both strategies have important strengths and limitations, reflecting the persistent difficulty of accurately predicting arrhythmic events in such a heterogeneous disease. The HCM Risk-SCD score has demonstrated robust external validation and high specificity for identifying patients at higher risk, but it may fail to recognize some vulnerable individuals who remain below conventional treatment thresholds. For this reason, several additional risk modifiers have gained increasing relevance in contemporary practice. Among them, extensive late gadolinium enhancement, left ventricular systolic dysfunction, apical aneurysm, and clinically meaningful genetic findings may provide important incremental prognostic information beyond traditional models. Emerging disease-modifying therapies, in particular Mavacamten, may also influence future risk assessment. However, whether these improvements translate into a true reduction in SCD risk remains uncertain. Importantly, the decision to implant an ICD should not depend on numerical risk alone. It should arise from a process of shared decision-making integrating estimated risk, treatment burden, competing comorbidities, age, lifestyle, and patient values. In this context, the concept of an individualized threshold of “acceptable risk” becomes central. In conclusion, prevention of SCD in HCM is moving beyond conventional scores toward a personalized and dynamic framework in which predictive tools, advanced phenotyping, evolving therapies, clinical expertise, and patient preferences are combined to guide individualized care. Full article

(1) Background and Objectives: Current risk stratification strategies for primary prevention of sudden cardiac death (SCD) have limited sensitivity and specificity. Artificial intelligence (AI) applied to electrocardiograms (ECGs) has emerged as a promising tool to predict the risk of future cardiac arrhythmias. This scoping review synthesizes evidence from original studies evaluating AI models trained on ECGs for risk stratification of SCD/malignant ventricular arrhythmias. (2) Materials and Methods: A comprehensive search of MEDLINE, Embase, Web of Science, Scopus and IEEE Xplore was conducted to identify peer-reviewed studies from inception to February 2026. Eligible studies included original investigations in which the model input was an ECG, recorded at baseline or during monitoring, and the outcome was either short-term or long-term SCD/malignant ventricular arrhythmia risk prediction. Extracted variables included study characteristics, ECG data, AI model data, model performance metrics, and the validation strategy. Risk of bias was assessed using PROBAST. (3) Results: Twenty studies met the inclusion criteria. High-risk cardiovascular subgroups (e.g., heart failure cohort, ICD cohort, etc.) or datasets from admitted patients, and conventional machine learning models or deep learning models were used in most studies. AI-ECG algorithms achieved moderate-to-high discriminative performance for identifying patients at an increased risk for imminent SCD/malignant ventricular arrhythmias (nine studies, AUROC ≈ 0.77–0.96) or future SCD/malignant ventricular arrhythmias (eleven studies, AUROC ≈ 0.66–0.94). However, multiple methodological limitations were identified, including limited sample sizes, susceptibility to overfitting, data imbalance-related bias, heterogeneity in dataset and endpoint definitions, inadequate external validation, and incomplete assessment and reporting of model calibration. (4) Conclusions: AI-ECG models demonstrate potential for risk stratification of SCD and malignant ventricular arrhythmias. However, the current evidence base is constrained by several methodological limitations, and further research is required to determine the clinical utility of AI-ECG for predicting SCD. Full article

Inherited cardiomyopathies represent a major cause of ventricular arrhythmias (VA) and sudden cardiac death (SCD), frequently occurring in the absence of advanced systolic dysfunction. Traditional strategies for the primary prevention of SCD have relied predominantly on left ventricular ejection fraction (LVEF), an approach that fails to capture the substantial biological and clinical heterogeneity of non-ischemic cardiomyopathies. Over the past decade, advances in cardiac genetics and cardiac magnetic resonance imaging have identified specific genotypes associated with a disproportionate arrhythmic risk, which often precedes overt ventricular remodeling. The 2023 European Society of Cardiology (ESC) Guidelines on cardiomyopathies formalize this paradigm shift by integrating etiology, myocardial substrate, and electrical phenotype into contemporary risk stratification. In this narrative review, we focus on cardiomyopathy-associated genotypes consistently linked to high arrhythmic risk—LMNA, truncating variants in FLNC, RBM20, PLN p.Arg14del, and desmosomal genes—and examine their molecular mechanisms, phenotypic trajectories, and arrhythmogenic profiles. We discuss how genotype-specific patterns of myocardial fibrosis, conduction disease, and VA inform implantable cardioverter-defibrillator (ICD) decision-making beyond LVEF-based thresholds. By synthesizing genetic, imaging, and clinical evidence in light of ESC 2023 recommendations, this review highlights the evolving role of genotype-informed strategies in the personalized prevention of SCD and underscores remaining gaps in evidence and risk prediction. Full article

Background/Objectives: Low image quality reduces diagnostic accuracy. We wanted to develop a framework for assessing transthoracic echocardiography (TTE) image quality in apical 2-, 3-, and 4-chamber views, and to use this framework to characterise segment-level visualisation patterns in patients with heart failure (HF). Methods: In this cross-sectional study, 268 TTE examinations from 230 patients qualified for ICD/CRT implantation in primary prevention of sudden cardiac death were analysed. Patient demographic, electrocardiographic, echocardiographic, and clinical characteristics were collected, and apical 2-, 3-, and 4-chamber views were extracted for image quality evaluation. Mean scores for each segment were calculated. The proportion of well-visualised segments per view was also evaluated. Risk factors for poor image quality were assessed. Results: We internally assessed the reliability of the framework (intra-class correlation coefficient > 0.9). The anterior and anterolateral walls consistently demonstrated the poorest quality, and the inferior segments the best. Clear inner-edge-to-outer-edge delineation of ≥5 segmental borders was achieved in only 30% of studies, while ≥5 endocardial border segments were visualised in 65% of cases. Reduced quality was frequently observed in patients with higher BMI and BSA, presence of HF risk factors (diabetes, prior myocardial infarction, and atrial fibrillation), and heart abnormalities (increased left ventricular end-diastolic value and hypokinesis). Conclusions: The prevalence of imaging challenges in TTE examinations performed in patients qualified for CRT-D/ICD implantation is high. These findings underscore the need for thorough training of echocardiographers and for sustained attention to technical details affecting image quality to achieve consistently high-quality images in routine practice. Full article

Sudden cardiac death (SCD) remains a major clinical challenge, with implantable cardioverter-defibrillators (ICDs) serving as the primary preventive intervention. Current patient selection guidelines rely on limited and imperfect risk markers. This study explores the potential of machine learning (ML) models to improve SCD risk prediction using tabular clinical data that include features derived from medical sensing devices such as electrocardiograms (ECGs) and ICDs. Several ML models, including tree-based models, Naive Bayes (NB), logistic regression (LR), and voting classifiers (VC), were trained on demographic, clinical, laboratory, and device-derived variables from patients who underwent ICD implantation at a Croatian tertiary center. The target variable was the activation of the ICD device (appropriate or inappropriate/missed), serving as a surrogate for high-risk SCD detection. Models were optimized for the F2-score to prioritize high-risk patient detection, and interpretability was achieved with post hoc SHAP value analysis, which confirmed known and revealed additional potential SCD predictors. The random forest (RF) model achieved the highest F2-score (F2-score 0.74, AUC-ROC 0.73), demonstrating a recall of 97.30% and meeting the primary objective of high true positive detection, while the VC classifier achieved the highest overall discrimination (F2-score 0.71, AUC-ROC 0.76). The predictive performance of multiple ML models, particularly the high recall they achieved, demonstrates the promising potential of ML to refine ICD patient selection. Full article

Spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic cause of acute coronary syndrome, characterized by the development of a false lumen within the coronary arterial wall, leading to narrowing or complete occlusion of the true lumen. This underrecognized condition accounts for a substantial proportion of sudden cardiac death (SCD), particularly among young, otherwise healthy women. Macroscopically, SCAD is defined by intramural hematoma and focal thickening of the arterial wall, while histological examination demonstrates separation of the tunica media, elastic fiber degeneration, and variable inflammatory infiltrates. Proposed pathogenic mechanisms include primary intimal tear and primary intramural hematoma, frequently associated with predisposing conditions such as fibromuscular dysplasia, connective tissue disorders, and specific hormonal states. In cases of myocardial infarction, the myocardium exhibits acute ischemic necrosis and early hypoperfusion injury. Postmortem diagnosis requires meticulous coronary dissection, adjunctive histochemical and immunohistochemical staining, and, when indicated, molecular autopsy (MA). The purpose of this review is to provide an updated synthesis of current knowledge on SCAD as a cause of SCD, integrating pathogenetic, morphological, and genetic perspectives, and to emphasize the role of MA as both a diagnostic and preventive tool. Full article

Genetic background is a critical determinant of disease expression, arrhythmic vulnerability, and therapeutic response in inherited cardiomyopathies. Implantable cardioverter-defibrillators (ICD) remain the cornerstone for primary prevention of sudden cardiac death, yet conventional selection based on left ventricular ejection fraction does not adequately reflect the heterogeneity of genetic substrates. Increasing evidence demonstrates that pathogenic variants differ not only in prevalence across cardiomyopathy subtypes but also in prognostic impact. Truncating variants, particularly in genes encoding structural proteins, are often associated with severe remodeling, progressive dysfunction, and high arrhythmic risk, whereas missense variants may confer variable expressivity, ranging from aggressive arrhythmogenic phenotypes to milder or late-onset disease. This variability underscores the importance of distinguishing variant classes in clinical decision-making. Integrating genetic information with advanced imaging markers, such as late gadolinium enhancement, allows refinement of arrhythmic risk stratification beyond static thresholds and supports more tailored ICD allocation. Nevertheless, translation into routine practice is limited by challenges in variant interpretation, phenotypic overlap between cardiomyopathy subtypes, and the lack of prospective validation of genotype-based models. In the precision medicine era, evolving strategies should move toward dynamic, multimodal approaches that combine genotype, phenotype, and imaging biomarkers, enabling more accurate prediction of arrhythmic risk and more cost-effective use of ICD therapy. Full article

Background: Mitral valve prolapse is a common valvular heart disorder, usually associated with a benign prognosis in the absence of significant mitral regurgitation. However, a subset of patients is at increased risk for complex ventricular arrhythmias and sudden cardiac death. Identifying these high-risk individuals remains a major clinical challenge. Case Summary: We present the case of a 71-year-old female patient with recurrent syncopal episodes, a strong family history of sudden cardiac death, and complex ventricular ectopy. Multimodality imaging revealed bileaflet mitral valve prolapse, severe mitral regurgitation, mitral annular disjunction, and the Pickelhaube sign, with no evidence of myocardial fibrosis on cardiac magnetic resonance imaging. The patient underwent minimally invasive mitral valve repair and received an implantable cardioverter-defibrillator for primary prevention of sudden cardiac death. Follow-up revealed significant reverse cardiac remodeling, marked reduction in arrhythmic burden, and restoration of mitral valve function. Family screening identified mitral annular disjunction in both of her daughters, who were asymptomatic and without arrhythmias. Discussion: Mitral annular disjunction has emerged as a potentially arrhythmogenic substrate, especially in patients with familial clustering, raising the possibility of a genetic predisposition. Risk stratification remains difficult, as no individual clinical, electrocardiographic, or imaging marker has demonstrated consistent predictive value. Surgical correction of mitral valve prolapse with associated mitral annular disjunction may lead to a reduction in arrhythmic risk and promote favorable structural remodeling. Conclusions: This case-based review emphasizes the importance of advanced imaging techniques in the identification and management of high-risk mitral valve prolapse phenotypes. Early surgical intervention and close arrhythmic surveillance may improve outcomes, although further research is necessary to define risk assessment tools and explore the genetic background of arrhythmogenic mitral valve disease. Full article

Background: Medical clearance for competitive sports is vital to safeguarding athletes’ health, particularly in high-intensity disciplines like football. In Italy, fitness assessments follow stringent protocols set by the Commissione di Vigilanza per il controllo dell’Idoneità Sportiva (COCIS), with a strong focus on cardiovascular screening. The primary goal is to prevent sudden cardiac death (SCD), a rare but catastrophic event in athletes. Methods: This paper provides an in-depth narrative review of the 2023 COCIS guidelines, examining the cardiological screening process, required diagnostic tests, management of identified cardiovascular conditions, and the protocols’ role in reducing SCD risk. Results: Comparisons with international standards underscore the effectiveness of the Italian approach. Conclusions: The COCIS 2023 guidelines provide clear, evidence-based protocols for cardiovascular risk assessment, significantly enhancing athlete safety and reducing the incidence of SCD in high-intensity sports. Full article

Background/Objectives: Sodium-glucose cotransporter-2 inhibitors (SGLT2i) have revolutionized heart failure (HF) therapies and are an essential component of guideline-directed medical therapy (GDMT); however, their significance in arrhythmia prevention is still uncertain. This meta-analysis evaluates the benefits of SGLT2i on arrhythmias in HF. Methods: A comprehensive examination was performed with PubMed, ScienceDirect, PLOS One, Cochrane, Google Scholar, and ClinicalTrials.gov from January 2014 to March 2025, complying with PRISMA guidelines. Randomized controlled trials (RCTs) comparing SGLT2i with placebo were incorporated. Primary results included ventricular arrhythmias (VA), sudden cardiac death (SCD), atrial arrhythmias, and conduction disorders. Subgroup analyses investigated the effects on arrhythmias in HF with reduced ejection fraction (HFrEF) and preserved ejection fraction (HFpEF). Results: A total of 11 RCTs involving 23,701 patients, 11,848 on SGLT2i (mean age: 68.26 ± 10 yrs, 53.5% males) and 11,853 on placebo (mean age: 67.91 ± 10 yrs, 53% males), were analyzed with a mean follow-up of 2.71 yrs. No significant differences were reported between SGLT2i and placebo for VA [relative risk (RR): 1.02, 95% confidence interval (CI): 0.83–1.25], I² =0%), atrial arrhythmias (RR: 0.92 [CI: 0.67–1.27], I² = 65.3%), or conduction disorders (RR:1.22 [CI: 0.86–1.73], I² = 10.4%). Notably, significant reductions in risk of SCD (RR: 0.68 [CI: 0.49–0.93], I² = 0%) and in the risk of atrial arrhythmias in HFrEF (RR: 0.66 [CI: 0.49–0.89], I² = 10.3%) were witnessed, although no such reduction was seen in HFpEF (RR: 1.14 [CI: 0.94–1.40], I² = 33.8%). Conclusions: SGLT2i do not reduce overall arrhythmia or conduction disorder risk in HF but significantly reduce the risk of SCD and atrial arrhythmias in HFrEF patients. These results highlight potential arrhythmia prevention benefits in HFrEF, warranting further targeted studies. Full article

Background/Objectives:Pacing treatment of bradyarrhythmias is both to reduce symptoms and to prevent syncope and sudden cardiac death. The aim of our study was to analyze left bundle branch area pacing (LBBAP) in the prevention of new-onset AF and the improvement of echocardiographic parameters in patients with mildly reduced left ventricular ejection fraction (LVEF) compared to patients with bradyarrhythmias but preserved LVEF who underwent mid-septal right ventricular pacing. Methods: This research was structured as a retrospective observational cohort study that included 186 patients with LBBAP and 186 patients with RVP, enrolled for 3 years until March 2024 with a follow-up time of 1 year. The primary endpoint of our study was new-onset atrial fibrillation after pacemaker implantation. The secondary endpoint was the improvement of echocardiographic parameters. Results: We observed in the LBBAP group a mean QRS complex duration of 108.7 ± 8.83 ms (after pacemaker implantation), compared to a much longer duration in the RVP group (143.8 ± 9.851 ms, p = <0.0001). At 1 year of follow-up, 22 (11.82%) patients in the RVP group were diagnosed with new-onset atrial fibrillation, compared to 6 (3.22%) patients out of 186 included in the LBBAP group (p = 0.0017). Regarding LVEF, at follow-up, RVP patients had a decrease in LVEF compared to those in the LBBAP group who had an improved LVEF (54.54 ± 3.77%, p < 0.0001). Conclusions: LBBAP both prevents the onset of atrial fibrillation and improves echocardiographic parameters, especially left ventricular ejection fraction, thus contributing to significantly reducing the risk of developing/worsening advanced heart failure through pacing-induced cardiomyopathy. Full article

Background/Objectives: Risk stratification for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) remains challenging, especially in high-risk cohorts. This study evaluated the predictive utility of the ESC HCM Risk Score and the additive value of myocardial fibrosis assessment via cardiac magnetic resonance (CMR) in HCM patients with implantable cardioverter-defibrillators (ICDs) for primary prevention. Methods: A retrospective analysis was conducted on 108 HCM patients (mean age 49.4 ± 14.2 years; 30.6% female; 63.9% with LVOT obstruction) with ICDs for primary SCD prevention. The primary endpoint was a composite of all-cause mortality or appropriate ICD therapy for ventricular arrhythmia over a mean follow-up of 69.5 ± 22.8 months. ESC HCM Risk Scores, the presence of fibrosis on CMR, and clinical outcomes were analyzed using univariate and multivariate models, ROC curves, and Kaplan–Meier survival estimates. Results: The primary endpoint occurred in 25 patients (23.1%; 3.1%/year). An ESC HCM Risk Score ≥ 4% was common (81.5%) but did not significantly predict the primary outcome (the c-statistic 0.54; p = 0.08) and demonstrated low positive (25%) and high negative predictive values (85%). Severe fibrosis on CMR was significantly associated with events in univariate analysis (p = 0.04), and its inclusion improved the model’s predictive accuracy (the c-statistic increased to 0.65; p = 0.03). Kaplan–Meier analysis revealed worse event-free survival in patients with both elevated ESC scores and more than mild fibrosis (p = 0.028). Conclusions: In this high-risk HCM cohort with ICDs, the ESC risk score showed limited predictive performance, while myocardial fibrosis on CMR added significant prognostic value. Incorporating the fibrosis assessment into future risk models may enhance SCD prediction and refine ICD decision-making in HCM. Further multicenter studies are needed to validate these findings. Full article

Background: Cardiovascular diseases, including sudden cardiac death (SCD), are the leading cause of mortality among kidney transplant recipients (KTRs). While implantable cardioverter defibrillators (ICDs) are established for SCD prevention in the general population, data on the benefits in patients with CKD is scarce and controversial, and there is no established general consensus on their use in this group of patients. Furthermore, data for KTRs are lacking. The aim of this study is to present our experience with ICDs in KTRs and evaluate the outcomes in this population. Methods: We retrospectively analyzed medical records of KTRs who received a kidney allograft between October 1973 and December 2023 and received ICDs for the prevention of SCD. Results: Of 2282 KTRs, 10 patients (0.44%) underwent an ICD implantation with an average age of 60.6 years at the time of implantation; 9 were male. Primary prevention of SCD was the most common indication, with only one patient receiving an ICD following sudden cardiac arrest. The female patient received an ICD while on dialysis, and the rest of the patients received ICDs in the posttransplant period with an average time of 9.1 years after KT. Kidney allograft function was reduced in all patients at the time of the ICD implantation with an average estimated glomerular filtration rate (eGFR) of 44 mL/min/1.73 m². No ICD-related complications were recorded. Six patients are alive with an average follow-up of 5.2 years. Conclusions: ICD implantation in carefully selected KTRs may offer survival benefits and can be a valuable tool in preventing SCD. Larger studies are needed to confirm these findings and establish clear guidelines for ICD use in this specific population. Full article