Sudden Cardiac Death In Patients Research Articles

Validation of the HCM Risk-SCD model in patients with hypertrophic cardiomyopathy and future perspectives

Abstract Background/Introduction The fact that hypertrophic cardiomyopathy (HCM) is associated with sudden cardiac death (SCD) due to malignant ventricular arrhythmias underlies its clinical importance (1). SCD in patients with HCM is often an unpredictable event, occurring without previous warning signs or symptoms, and is most common in the young middle-aged group (2). The greatest dilemma in the management of HCM patients is to identify the high-risk population for SCD. The HCM risk- SCD model provides a useful convenience in determining the risk of SCD in patients with HCM even though some patients with low-risk scores still remain at risk of SCD (3). These results have increased the interest in additional strategies to improve the existing risk model. Purpose Hence, the aim of our study was to assess the performance of HCM Risk-SCD in a large series of consecutive patients with HCM who had been followed up in a tertiary centre. Methods The study population consisted of 389 consecutive HCM patients who had been followed up between 2004 and 2021. Demographic and clinical characteristics estimated 5-year risk using the HCM Risk-SCD model were compiled and survival data were collected during follow-up. Patients were divided into 2 groups according to their long-term survival and HCM risk-SCD scores of these two groups were compared. Results The long-term mortality was observed in 47 patients out of 389 patients in the mean follow-up of 55.5 ± 12.7 months. The mean HCM Risk-SCD of surviving patients was significantly lower than that of non-survivors (1.8% vs. 3.0%, p<0.001). The HCM Risk-SCD score was above 6% in 9 (2.6%) survivors and 9 (19.1%) non-survivors (p<0.001). Considering the Kaplan-Meier curve based on the 6% HCM Risk-SCD score, patients at ≥6% risk also had a significantly higher long-term risk of death compared to those with <6% (log-rank test value: 33.72, p<0.001) (Figure 1). The ROC curve based on the HCM Risk-SCD score had 61% sensitivity and 61% specificity for risk for 2.0%, 38% sensitivity and 99% specificity for ≥4%, 17% sensitivity and 99% specificity for ≥6% (Figure 2). Conclusion(s) In conclusion, the current study demonstrates that the HCM risk-SCD model, while easily calculable and clinically useful, does not reliably identify a significant subset of patients with HCM who will potentially be at lifetime risk of SCD. Identifying patients with hypertrophic cardiomyopathy at high risk for SCD who deserve ICD therapy is a critical management priority. A new risk algorithm that can detect the SCD risk of HCM patients with higher sensitivity is needed.Figure 1Figure 2

Comparison of ventricular tachyarrhythmias in HF patients on dapagliflozin versus empagliflozin

Abstract Background Ventricular tachycardia (VT) and ventricular fibrillation (VF) are devastating tachyarrhythmias that can cause sudden cardiac death in patients with heart failure (HF) if not anticipated or treated promptly. Although post-hoc analysis in DAPA-HF showed promise in their benefit, Sodium-glucose transport protein 2 inhibitors (SGLT2i) have not yet been evaluated for their effect on these tachyarrhythmias. This retrospective analysis aims to compare the 2 most commonly used SGLT2i agents, dapagliflozin and empagliflozin, and the incidence of VT in patients with HF. Methods The TriNetX Research network was used for this study. The two initial patient cohorts were created using ICD-10 codes and medication codes from the TriNetX platform. Both cohorts consisted of patients over age 60 with a history of heart failure and either VF or VT. One cohort was on treatment with dapagliflozin and the other with empagliflozin. The cohorts were balanced by propensity score matching and the greedy nearest neighbor algorithm for age, gender, race, ethnicity, hyperkalemia, and the current use of spironolactone, amiodarone, metoprolol, and carvedilol, resulting in 9,841 patients in each arm. The cohorts were then evaluated for the development of VF or VT over the subsequent five years as well as all-cause mortality. Additional cohorts were created to evaluate dapagliflozin vs empagliflozin in diabetics, nondiabetics, and patients with ejection fractions less than 35% Results Dapagliflozin was associated with an increased risk of ventricular arrhythmia in heart failure patients compared to empagliflozin (46.0% vs. 40.8%, RR 1.13, 95% CI (1.09,1.16), P value < 0.0001). It was also associated with higher mortality at five years (RR 1.10, 95% CI (1.03,1.18), P value 0.0034). Conclusions In comparisons between dapagliflozin and empagliflozin in heart failure patients with previously documented ventricular arrhythmias, empagliflozin had 13% less risk of recurrence of ventricular arrhythmias. Secondarily, the empagliflozin cohort also exhibited lower all-cause mortality (14.8% vs 16.3%) at five years from starting the drug. Our analysis does not disprove findings in DAPA-HF, but rather may support the potential class effect of SGLT2 inhibitors in their ability to suppress VT.(1) Importantly, the overall incidence of ventricular tachyarrhythmias is high in both cohort, given we selected a high-risk population. Notably, our documented recurrence rate is similar to past reports of recurrence after VT ablation, the gold standard for treatment of VT. (2) SGLT2 inhibitors have shown clear benefit in multiple placebo-controlled studies in terms of heart failure outcomes like mortality and readmission, but their effect on arrhythmias is one more additional benefit this class may have. (3) Given these associations, empagliflozin may be a more favorable options for HF patients who have already had VT or VF.

Arrhythmia burden in patients with congenital heart disease

Abstract Background The prevalence of congenital heart defects (CHD) is rising. Many patients experience arrhythmias either due to the defect itself or necessary intervention. Sudden onset of arrhythmia poses grave risks such as heart failure, stroke, or cardiac death. Research gaps exist regarding high-risk defects, age susceptibility, and prognostic implications of arrhythmia. Purpose Delineate the burden of arrhythmias in patients with CHD. Methods Patients born in the period 1970-2017 and diagnosed with CHD were identified in Swedish nationwide medical registries. Each patient was matched by birthyear and sex with ten controls from the general population. Cox proportional hazard regression model and Fine-Gray regression model with death as competing risk were used to compute risk (hazard ratio [HR]) and cumulative incidence of arrhythmias (supraventricular and ventricular arrhythmias). Estimates were compared with the matched controls. Follow-up started at time of birth. Results We included 63,559 patients and 583,205 matched controls with median follow-up of 12.8 years. Almost two-thirds of the total CHD population were born after year 2000 and 89% (n=56,564) had a simple lesion (defined as atrial or ventricular septal defect, coarctation of the aorta or malformation of the heart valves). During follow-up, 1,632 events of arrhythmia (90% of supraventricular origin) were registered in the population of patients with CHD, yielding an incidence rate of 16.4 per 10,000 person-years (versus 1.6 in the control population). The cumulative incidence of arrhythmias in the total CHD population was slightly increased during childhood compared with the control population, but rose significantly hereafter, illustrated in Figure 1, yielding an overall risk of 10.5 (CI 95%: 9.8-11.2). Stratified by lesion severity, the risk for arrhythmias was 8.7 (CI 95%: 7.6-8.7) and 30.4 (CI 95%: 26.2-35.2) in patients with simple and complex lesions, respectively. Patients with simple CHD lesions displayed a risk of 7.7 (95% CI: 7.1-8.4) for supraventricular arrhythmias and 11.5 (95% CI: 9.2-14.5) for ventricular arrhythmias. Correspondingly, patients with complex CHD lesions displayed risks of 66.7 (95% CI: 42.7-104.2) and 28.9 (95% CI: 24.8-33.6). Conclusion Arrhythmias, irrespective of origin, are common in patients with CHD and correlate with lesion severity. The overall burden of arrhythmias increased significantly after childhood. Meticulous follow-up is vital in order to prevent, detect and treat arrhythmias as it might trigger clinical deterioration and even sudden cardiac death in patients with CHD.

Epicardial ablation in patients with Brugada syndrome: a single centre experience

Abstract Introduction Brugada syndrome (BrS) is a rare disease associated with an increased risk of ventricular fibrillation (VF), ventricular tachycardia (VT), and sudden cardiac death (SCD). The standard prevention of SCD in patients with BrS is the implantation of an implantable cardioverter-defibrillator (ICD), especially in patients who have previously suffered cardiac arrest or arrhythmogenic syncope. Catheter ablation is an effective method to prevent recurrences of VF/VT in these patients. Purpose Retrospective analysis of the results of catheter ablation by epicardial approach and genetic testing in patients with a diagnosis of BrS and repeated adequate ICD interventions. Methods In 2013-2024, catheter ablation by epicardial approach was performed in our centre in 9 patients diagnosed with Brugada syndrome (mean age 41±6 years, nine males, left ventricular ejection fraction 57±4%). All patients had an ICD implanted for secondary prevention of SCD and presented with adequate ICD therapies (mean 9±11 shocks/patient in the last six months before catheter ablation). Before catheter ablation, two patients were treated with quinidine. Patients underwent cardiogenetic testing by next-generation sequencing of 228 genes. Results Type 1 electrocardiogram (ECG) pattern was present at baseline ECG in four (44%) patients; in five (56%) patients, type 1 was induced after ajmaline administration. Genetic testing was performed in seven patients, and only one patient (14%) was found to have a relevant pathogenic mutation in the SCN5A gene. Prior to ablation, sustained VT/VF could be induced by programmed electrical stimulation in three patients (33%). Epicardial ablation was targeted at areas of abnormal electrograms located over the right ventricular outflow tract. No complications were noted. The mean follow-up period was 25±30 months. In one patient (11%), reablation was required to suppress ventricular arrhythmias successfully. After the last ablation, no recurrence of VT/VF was observed in any patient. Conclusions Epicardial ablation is an effective method that leads to the suppression of ventricular arrhythmias in patients with a diagnosis of BrS and repeated ICD interventions. Despite the malignant arrhythmic phenotype, patients in our cohort had a rather low prevalence of signs associated with a high risk of VT/VF, such as spontaneous ECG pattern of BrS, inducibility of VT/VF during electrical programmed stimulation, or the presence of a causative genetic mutation.

Effectiveness of contemporary risk stratification in patients with hypertrophic cardiomyopathy: a pilot study

Abstract Introduction Risk stratification and primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) is a challenging discipline. When using current stratification systems, there is always a compromise between sensitivity and specificity. Objective In our pilot study, we sought to determine the incidence of SCD and the effectiveness of stratification systems, i.e., the ESC HCM Risk-SCD score and the system of individual major risk factors (RF) according to ACC/AHA in patients followed in an expert center. Methods Patients with HCM were risk-stratified and followed for 10 ± 8 years at the referral HCM center. Results Five hundred and five patients were stratified, and 71 (14%) were implanted with a cardioverter-defibrillator (ICD) based on risk stratification. ICD patients were stratified at age 39 ± 17 years, of which 26 (35%) were women, maximal left ventricular wall thickness (MLWT) was 23 ± 7 mm, and left atrial dimension (LA) 46 ± 7 mm. During follow-up, 12 (17%) patients had ≥ 1 appropriate ICD therapy at age 46 ± 19 years. Patients with ≥ 1 appropriate ICD therapy had an average 5-year ESC score of 1.8 ± 0.4% and an average ACC/AHA major RF of 1.4 ± 0.8, MLWT 25 ± 6 mm, LA 47 ± 7 mm. None of the ICD patients died of SCD. Five (7%) patients suffered from ICD complications, including 4 (6%) inappropriate shock, and one patient experienced infective endocarditis requiring system extraction. During follow-up, 118 (23%) patients died, of which 28 (6%) from HCM-related causes, 12 (2%) from stroke, 11 (2%) from advanced heart failure, one perioperatively (myectomy) and 4 (1%) patients died suddenly (SCD). The mean age of SCD patients at the time of stratification was 59 ± 14 years; all were male, MLWT 18 ± 5 mm, LA 49 ± 2 mm, mean ESC score 2.0 ± 0.9%, and three (75%) had no RF present according to ACC/AHA. The ESC HCM Risk-SCD score was markedly less sensitive (sensitivity 56%; 95% CI 21-86) but more specific (specificity 98%; 95% CI 96-99) than the ACC/AHA approach (sensitivity 77%; 95% CI 46-95; and specificity 71%; 95% CI 67-75) (Figure). Conclusion With current risk stratification, albeit imperfect, SCD is rare in HCM patients managed at an expert center.

Nationwide burden of sudden cardiac death among patients with a psychiatric disorder

BackgroundPatients with psychiatric disorders have increased all-cause mortality compared with the general population. Previous research has shown that there is a fourfold increased risk of sudden cardiac death (SCD) among...

Deep learning of echocardiography distinguishes between presence and absence of late gadolinium enhancement on cardiac magnetic resonance in patients with hypertrophic cardiomyopathy

BackgroundHypertrophic cardiomyopathy (HCM) can cause myocardial fibrosis, which can be a substrate for fatal ventricular arrhythmias and subsequent sudden cardiac death. Although late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) represents myocardial fibrosis and is associated with sudden cardiac death in patients with HCM, CMR is resource-intensive, can carry an economic burden, and is sometimes contraindicated. In this study for patients with HCM, we aimed to distinguish between patients with positive and negative LGE on CMR using deep learning of echocardiographic images.MethodsIn the cross-sectional study of patients with HCM, we enrolled patients who underwent both echocardiography and CMR. The outcome was positive LGE on CMR. Among the 323 samples, we randomly selected 273 samples (training set) and employed deep convolutional neural network (DCNN) of echocardiographic 5-chamber view to discriminate positive LGE on CMR. We also developed a reference model using clinical parameters with significant differences between patients with positive and negative LGE. In the remaining 50 samples (test set), we compared the area under the receiver-operating-characteristic curve (AUC) between a combined model using the reference model plus the DCNN-derived probability and the reference model.ResultsAmong the 323 CMR studies, positive LGE was detected in 160 (50%). The reference model was constructed using the following 7 clinical parameters: family history of HCM, maximum left ventricular (LV) wall thickness, LV end-diastolic diameter, LV end-systolic volume, LV ejection fraction < 50%, left atrial diameter, and LV outflow tract pressure gradient at rest. The discriminant model combining the reference model with DCNN-derived probability significantly outperformed the reference model in the test set (AUC 0.86 [95% confidence interval 0.76–0.96] vs. 0.72 [0.57–0.86], P = 0.04). The sensitivity, specificity, positive predictive value, and negative predictive value of the combined model were 0.84, 0.76, 0.78, and 0.83, respectively.ConclusionCompared to the reference model solely based on clinical parameters, our new model integrating the reference model and deep learning-based analysis of echocardiographic images demonstrated superiority in distinguishing LGE on CMR in patients with HCM. The novel deep learning-based method can be used as an assistive technology to facilitate the decision-making process of performing CMR with gadolinium enhancement.

Early prediction of sudden cardiac death using multimodal fusion of ECG Features extracted from Hilbert–Huang and wavelet transforms with explainable vision transformer and CNN models

Background and Objective:Sudden cardiac death (SCD) is a critical health issue characterized by the sudden failure of heart function, often caused by ventricular fibrillation (VF). Early prediction of SCD is crucial to enable timely interventions. However, current methods predict SCD only a few minutes before its onset, limiting intervention time. This study aims to develop a deep learning-based model for the early prediction of SCD using electrocardiography (ECG) signals. Methods:A multimodal explainable deep learning-based model is developed to analyze ECG signals at discrete intervals ranging from 5 to 30 min before SCD onset. The raw ECG signals, 2D scalograms generated through wavelet transform and 2D Hilbert spectrum generated through Hilbert–Huang transform (HHT) of ECG signals were applied to multiple deep learning algorithms. For raw ECG, a combination of 1D-convolutional neural networks (1D-CNN) and long short-term memory networks were employed for feature extraction and temporal pattern recognition. Besides, to extract and analyze features from scalograms and Hilbert spectra, Vision Transformer (ViT) and 2D-CNN have been used. Results:The developed model achieved high performance, with accuracy, precision, recall and F1-score of 98.81%, 98.83%, 98.81%, and 98.81% respectively to predict SCD onset 30 min in advance. Further, the proposed model can accurately classify SCD patients and normal controls with 100% accuracy. Thus, the proposed method outperforms the existing state-of-the-art methods. Conclusions:The developed model is capable of capturing diverse patterns on ECG signals recorded at multiple discrete time intervals (at 5-minute increments from 5 min to 30 min) prior to SCD onset that could discriminate for SCD. The proposed model significantly improves early SCD prediction, providing a valuable tool for continuous ECG monitoring in high-risk patients.

Amiodarone or Implantable Cardioverter-Defibrillator in Chagas Cardiomyopathy

Over 10 000 people with Chagas disease experience sudden cardiac death (SCD) annually, mostly caused by ventricular fibrillation. Amiodarone hydrochloride and the implantable cardioverter-defibrillator (ICD) have been empirically used to prevent SCD in patients with chronic Chagas cardiomyopathy. To test the hypothesis that ICD is more effective than amiodarone therapy for primary prevention of all-cause mortality in patients with chronic Chagas cardiomyopathy and moderate to high mortality risk, assessed by the Rassi score. CHAGASICS is an open-label, randomized clinical trial. The study enrolled patients from 13 centers in Brazil from May 30, 2014, to August 13, 2021, with the last follow-up November 8, 2021. Patients with serological findings positive for Chagas disease, a Rassi risk score of at least 10 points (intermediate to high risk), and at least 1 episode of nonsustained ventricular tachycardia were eligible to participate. Data were analyzed from May 3, 2022, to June 16, 2023. Patients were randomized 1:1 to receive ICD or amiodarone (with a loading dose of 600 mg after randomization). The primary outcome was all-cause mortality, and secondary outcomes included SCD, hospitalization for heart failure, and necessity of a pacemaker during the entire follow-up. The study was stopped prematurely for administrative reasons, with 323 patients randomized (166 in the amiodarone group and 157 in the ICD group), rather than the intended 1100 patients. Analysis was by intention to treat at a median follow-up of 3.6 (IQR, 1.8-4.4) years. Mean (SD) age was 57.4 (9.8) years, 185 patients (57.3%) were male, and the mean (SD) left ventricular ejection fraction was 37.0% (11.6%). There were 60 deaths (38.2%) in the ICD arm and 64 (38.6%) in the amiodarone group (hazard ratio [HR], 0.86 [95% CI, 0.60-1.22]; P = .40). The rates of SCD (6 [3.8%] vs 23 [13.9%]; HR, 0.25 [95% CI, 0.10-0.61]; P = .001), bradycardia requiring pacing (3 [1.9%] vs 27 [16.3%]; HR, 0.10 [95% CI, 0.03-0.34]; P < .001), and heart failure hospitalization (14 [8.9%] vs 28 [16.9%]; HR, 0.46 [95% CI, 0.24-0.87]; P = .01) were lower in the ICD group compared with the amiodarone arm. In patients with chronic Chagas cardiomyopathy at moderate to high risk of mortality, ICD did not reduce the risk of all-cause mortality. However, ICD significantly reduced the risk of SCD, pacing need, and heart failure hospitalization compared with amiodarone therapy. Further studies are warranted to confirm the evidence generated by this trial. ClinicalTrials.gov Identifier: NCT01722942.

Smoking and sudden cardiac death in patients with previous coronary artery disease.

Smoking is a known risk for sudden cardiac death (SCD) in the general population. However, its significance in patients with acute coronary syndrome (ACS), a condition that also elevates the risk of SCD, is disputable. A total of 9704 consecutive ACS patients with available smoking data were included in the analysis. Comprehensive patient data were obtained from the Mass Data in Detection and Prevention of Serious Adverse Events in Cardiovascular Disease research database. A composite endpoint of SCD, SCD aborted by successful resuscitation and accurate implantable cardioverter defibrillator therapy to otherwise potentially fatal ventricular fibrillation/ventricular tachycardia was used. Univariate, age- and sex-adjusted, and a multivariate fine-gray competing risk regression with adjustment to traditional risk factors was conducted. Median follow-up time was 6.8 years (IQR, 4.1-10.2), and 454 (4.7%) SCD cases were identified. At the baseline, 23.7% (N = 2444) were active smokers, and 20.8% (N = 2146) were ex-smokers. In the multivariate model, active smokers had an elevated risk of 1.79 (95% CI, 1.41-2.27; P < 0.001) for future SCD. Ex-smokers had no elevated risk for SCD in fine-gray subdistribution hazard. Also, active smokers were notably younger (mean age 58.7 years) than non- or ex-smokers (71.1 years and 68.9 years, respectively, P < 0.001 for both comparisons). Active smokers had a 79% higher risk of SCD when compared with nonsmokers. Smoking cessation should be heavily encouraged after ACS. Also, a person's smoking status should be considered in further studies developing SCD and implantable cardioverter defibrillator-benefit risk scores.

Association of antipsychotic formulations with sudden cardiac death in patients with schizophrenia: A nationwide population-based case–control study

The aim of this study was to clarify the association between various antipsychotic formulations—oral-daily antipsychotics (OAPs), long-acting injectable antipsychotics (LAIs), and their combination—and the risk of sudden cardiac death (SCD). We conducted a nationwide population-based case–control study using data from 2011 to 2020 from the National Health Insurance Research Database and multiple-cause-of-death data from Taiwan. The study included patients with a new diagnosis of schizophrenia who were followed for SCD occurrence until 2020. Cases and controls were frequency-matched at a 1:4 ratio by age, sex, and year of new schizophrenia diagnosis. Compared with the use of OAP monotherapy, the use of LAI and OAP combination (OR = 1.91) and LAI monotherapy (OR = 1.45) were associated with an increased risk of SCD. Additionally, cardiovascular comorbidities (adjusted OR = 11.15) were identified as a significant risk factor for SCD. This study revealed the following hierarchy of SCD risk associated with antipsychotic formulations (listed from lowest to highest risk): nonuse of antipsychotics, OAP monotherapy, LAI monotherapy, and their combination. These findings underscore the importance of assessing cardiovascular disease history before LAIs are prescribed to patients with schizophrenia and indicate that physicians should avoid prescribing combined antipsychotics when using LAIs.

Decision-making regarding subcutaneous implantable cardioverter defibrillator as primary prevention in patients with low ejection fraction.

Conventional transvenous implantable cardioverter-defibrillator (TV-ICD) is the standard device used for primary prevention of sudden cardiac death (SCD) in patients with reduced left ventricular ejection fraction (LVEF). Nonetheless its use is associated with lead-related complications including infection and malfunction. A subcutaneous implantable cardioverter-defibrillator (S-ICD) offers an alternative option without the need for a transvenous lead but has limitations. The decision to implant a TV-ICD or S-ICD in patients with impaired LVEF for primary prevention of SCD is controversial. Several randomised controlled trials and large observational studies have confirmed similar safety and efficacy of S-ICDs and TV-ICDs in such population. A literature review was conducted to compare the outcomes of subcutaneous (S-ICD) versus transvenous (TV-ICD) implantable cardioverter-defibrillators. Databases including PubMed, MEDLINE, and Cochrane were searched for relevant peer-reviewed articles. Studies were selected based on relevance and quality. Key outcomes like complication rates, efficacy, and patient survival were summarized in a comparative table. Different factors that influence the choice between an TV-ICD and S-ICD for primary prevention of SCD in patients with LVEF are highlighted to guide selection of the appropriate device in different patient populations. Moreover, future perspective on the combination of SICD with leadless pacemaker, and the latest development of the extravascular implantable cardioverter defibrillator are also discussed. S-ICD offers a safe and efficacious option to primary prevention in reduced ejection fraction. Future development including incorporation of leadless pacemaker will add to the arsenal of choice to protect patients from sudden cardiac death.

ARRHYTHMIAS IN HEART FAILURE: PATHOPHYSIOLOGY

Heart failure is a medical condition caused by various factors and is associated with significant morbidity and mortality. Significantly, heart failure provokes changes in the expression and regulation of ion channels, leading to electrical remodelling that can increase the risk of arrhythmias. Structural remodelling in heart failure, which involves myocardial hypertrophy, fibrosis, and scar formation, is crucial in the development of arrhythmogenic conditions. This article examines the mechanisms contributing to increased susceptibility to arrhythmias and associated ion channel expression, function, and calcium homeostasis alterations in heart failure. Understanding the mechanisms of arrhythmogenic remodelling is essential to improve arrhythmia treatment and prevent sudden cardiac death in patients with heart failure.

Brugada syndrome, early repolarization syndrome/j-wave syndromes, and a subtype of idiopathic ventricular fibrillation: microstructural cardiomyopathies

Brugada Syndrome is an inherited cardiac channelopathy with a high incidence of ventricular fibrillation and sudden cardiac death in patients with structurally normal hearts. Diagnosis is based on a characteristic electrocardiographic pattern (coved type ST-segment elevation ≥2 mm followed by a negative T-wave in ≥1 in the right precordial leads V1-V2) combined with an absence of gross structural abnormalities and several other criteria. The cornerstone of BrS diagnosis and definition, is its characteristic ECG pattern that can be present spontaneously or unmasked by drugs. This entity was described by the Brugada brothers in 1992 and belongs to a group of diseases known as inherited primary arrhythmia syndromes. The prevalence varies among regions and ethnicities, affecting mostly males. Despite several genes identified, SCN5A seems to be the most affected gene related BrS (≈ 30% of patients). The current main therapy is an implantable cardioverter-defibrillator, but radiofrequency catheter ablation has been recently reported as an effective new treatment.

Hypertrophic Cardiomyopathy Unmasked: The Hidden Danger of Left Ventricular Apical Aneurysm through Coronary Embolization

Hypertrophic Cardiomyopathy (HCM) is a genetic heart disease characterized by left ventricular (LV) hypertrophy, with outcomes ranging from asymptomatic to sudden cardiac death (SCD). Accurate diagnosis and risk stratification are essential. This case report discusses a 52-year-old female with family history of sudden deaths, presenting with acute coronary syndrome symptoms. Investigations revealed atrial fibrillation, ST-elevation, and a thrombus in the left anterior descending artery (LAD). Echocardiography and cardiac CT confirmed a left ventricular apical aneurysm, multiple apical thrombus, and hypertrophic septal wall with systolic LV dysfunction. The report highlights the risk of thromboembolic events and SCD in patients with LV apical aneurysms. Meta-analyses and guidelines emphasize the importance of integrating risk markers like LV apical aneurysms and late gadolinium enhancement into clinical assessments. Given the patient’s family history, aneurysm, and LV dysfunction, primary SCD prevention with an implantable cardioverter-defibrillator (ICD) was deemed suitable. HCM patients with LV apical aneurysms represent a high-risk phenotype requiring comprehensive management, including SCD and stroke prevention.

How do illness perceptions impact device acceptance in patients with an implantable cardioverter defibrillator?

Abstract Background The implantable cardioverter defibrillator (ICD) is the first-line treatment for the prevention of sudden cardiac death in patients at high risk of a life-threatening arrhythmia. Illness perceptions encompass the cognitive and emotional representations each ICD patient has about their health condition, thus influencing coping strategies and health behaviors essential to adjusting to life with their device and underlying heart disease. We investigate how illness perceptions are prospectively linked to device acceptance in ICD patients. Purpose To examine how illness perceptions at ICD implantation are associated with patient device acceptance one-year post-implantation. Methods Secondary analysis of the ACQUIRE-ICD, national, multi-center, randomised controlled trial. Illness perceptions were measured with the Brief Illness Perceptions Questionnaire (B-IPQ) with 350 first-time ICD or cardiac resynchronization therapy defibrillator (CRT-D) recipients filling out the questionnaire at ICD implantation. B-IPQ was only assessed at baseline. Patient device acceptance, a disease-specific quality of life measure for ICD patients, was measured at both ICD implantation and at 12 months follow-up with the Florida Patient Acceptance Survey (FPAS). Of the 350 patients, 261 patients (75%) completed FPAS at 12 months follow-up. Results The participants’ mean age was 59.7 ± 11.4 years with the majority being male (83%). Total illness perceptions scores were categorised into low experienced threat (n = 182, 52%), medium experienced threat (n = 82, 23%), and high experienced threat (n = 86, 25%). In the multivariate analysis, high experienced threat at ICD implantation was associated with significantly lower device acceptance at 12 months follow-up compared to low (B = -5.80, 95% CI [- 10.61, – -1.00], p = .018) and moderate experienced illness threat (B = -7.35, 95% CI [- 12.32, – -2.38], p = .004) while adjusting for device acceptance at baseline, age, sex, NYHA-class, indication, ischemic heart disease, education, intervention group and device type. In an exploratory analysis investigating associations of all 8 B-IPQ illness perception dimensions (single item-scores) simultaneously regressed on 12-months total FPAS-scores with baseline FPAS-scores as covariate, B-IPQ item 4 measuring treatment control showed the strongest association (B = 0.94, 95% CI [0.13, – 1.76], p = .023) with higher perceived treatment control linked with higher device acceptance. Conclusion ICD patients with high experienced illness threat at ICD implantation had substantially lower device acceptance at one-year post-implantation compared to patients with low-to-moderate experienced illness threat. Perceptions of treatment control at ICD implantation appear to be a potential mediator of this relationship.

Arrhythmic Mitral Valve Prolapse Syndrome and Ventricular Arrhythmias: A Comprehensive Review and the Role of Catheter Ablation.

Mitral valve prolapse (MVP) affects 2-3% of the general population, and despite its benign prognosis overall, it is associated with sudden death in a small subset of patients. The term "arrhythmic MVP syndrome" (AMVPS) refers to the presence of frequent or complex ventricular arrhythmias, commonly reported in female patients with a stereotypical phenotype including bileaflet myxomatous disease, ECG repolarisation abnormalities in inferior leads, mitral annular disjunction, and significant fibrosis in the inferolateral LV and papillary muscles. Modern imaging technologies have led to the identification of new risk factors that have been implemented in recent risk stratification guidelines; however, screening for patients with MVP who are at risk of sudden cardiac death (SCD) remains challenging. In addition, there is a limited amount of data on the outcomes of different treatment approaches in AMVP and no specific indication for targeted or disease-modifying therapies within current guidelines. Potential arrhythmic substrates in patients with AMVP syndrome have been the subject of interest in previous studies, with areas consisting of fibrosis at the papillary muscle level and the Purkinje system. Premature ventricular contractions (PVCs) originating from these areas have been shown to play an important role as triggers for ventricular fibrillation and SCD in patients with AMVP. Catheter ablation has emerged as a potential treatment modality in patients with MVP and ventricular arrhythmias (VAs), targeting arrhythmic substrates and triggering PVC foci. The aim of this review is to explore the role of catheter ablation in treating patients with AMVP.

Anxiety and depression in a patient with an implantable cardioverter defibrillator for early repolarization syndrome: A case report and clinical discussion

Early repolarization syndrome (ERS) was initially thought to be a good prognostic manifestation detectable by electrocardiogram. In recent years, with the gradual deepening of research and understanding, ERS is considered to possess malignant tendency and related to ventricular fibrillation (VF) and sudden cardiac death (SCD). Implantable cardioverter defibrillator (ICD) is currently the first choice of treatment used to reduce the risk of potentially life-threatening arrhythmias and SCD in patients at high risk for ERS. The stress of living with an implanted device and receiving ICD shock has been noted to exert a psychological toll on patients, especially those who have previously experienced VF and syncope. The current case report describes symptoms and signs of a patient with ERS, and discusses the changes of psychological condition after ICD implantation. Moreover, we explored a targeted treatment approach for anxiety and depression in an individual with an ICD for ERS, which combined medication, cognitive behavioral therapy, and physical exercise. We believe that the psychological experience of ICD recipients, which is a critical component in designing the biopsychosocial therapeutic approach for this growing patient population, is worthy of more attention.

Progranulin, sICAM-1, and sVCAM-1 May Predict an Increased Risk for Ventricular Arrhythmias in Patients with Systemic Sclerosis.

In systemic sclerosis (SSc), fibrosis of the myocardium along with ongoing autoimmune inflammation can alter the electric function of the cardiac myocytes, which may increase the risk for ventricular arrhythmias and sudden cardiac death. We analyzed the electrocardiographic (ECG) variables describing ventricular repolarization such as QT interval, QT dispersion (QTd), T wave peak-to-end interval (Tpe), and arrhythmogeneity index (AIX) of 26 patients with SSc and 36 healthy controls. Furthermore, echocardiographic and laboratory parameters were examined, with a focus on inflammatory proteins like C-reactive ptotein (CRP), soluble intracellular adhesion molecule-1 (sICAM-1), soluble vascular adhesion molecule-1 (sVCAM-1), and progranulin (PGRN). The CRP, sICAM-1, and sVCAM-1 levels were positively correlated with the length of the QT interval. Although the serum PGRN levels were not increased in the SSc group compared to the controls, in SSc patients, the PGRN levels were positively correlated with the QT interval and the AIX. According to our results, we conclude that there may be a potential association between autoimmune inflammation and the risk for ventricular arrhythmias in patients with SSc. We emphasize that the measurement of laboratory parameters of inflammatory activity including CRP, PGRN, sVCAM-1, and sICAM-1 could be helpful in the prediction of sudden cardiac death in patients with SSc.

Renal dysfunction is a time-varying risk predictor of sudden cardiac death in heart failure.

Sudden cardiac death (SCD) is a common mode of death in patients with congestive heart failure (CHF). Implantable cardioverter defibrillator (ICD) implantation is established treatment for SCD prevention, but current eligibility criteria based on left ventricular ejection fraction (LVEF) and New York Heart Association (NYHA) functional class may be due for reconsideration given the increasing effectiveness of pharmacological therapy. We sought to reconsider the risk stratification of SCD in patients with symptomatic CHF. In total, 1,676 consecutive patients (74±13years old; 56% male) with NYHA class II or III CHF between 2008 and 2015 were enrolled for this prospective study. The endpoint was SCD. During a median (interquartile range) follow-up period of 25 (4-70) months, 198 (11.8%) patients suffered SCD. Of those events, 23% occurred within 3months of discharge. In the adjusted analyses, estimated glomerular filtration rate (eGFR)<30ml/min/1.73m2 [hazard ratio (HR) 1.73, 95% confidence interval (CI) 1.11-2.70, P=0.01] and LVEF≤35% (HR 2.31, 95% CI 1.47-3.66, P<0.01) were independent risk predictors of SCD. Addition of eGFR to LVEF significantly improved prediction of SCD in the C-index (P=0.04), and in two metrics, net reclassification improvement (P=0.01) and integrated discrimination improvement (P=0.03). The predictive power of eGFR declined time-dependently over 2years. The addition of eGFR to current eligibility criteria may be useful for risk assessment of SCD, although its predictive power wanes over time. Roughly a quarter of the SCD occurred within 3months after discharge in patients with CHF.