Risk Of Sudden Cardiac Death Research Articles

Management of Ventricular Arrhythmias in Heart Failure: Can Less Be More?

Ventricular arrhythmias (VAs) affect many patients with heart failure and underlying structural heart disease and are associated with significant morbidity and mortality. Antiarrhythmic drugs are often the initial treatment, but medication alone often fails to sufficiently suppress VAs. While catheter ablation (CA) remains the gold standard for treatment of VAs, CA is an invasive procedure and can be associated with periprocedural complications including acute clinical decompensation. Thus, there is an important need for alternative therapies. Recent advances in risk stratification and the development of new ablation technologies may reduce some of the periprocedural complications and limitations of CA. In addition, less invasive therapies for VAs may provide an alternative treatment strategy for patients in both the acute and chronic setting. For patients acutely admitted with ventricular tachycardia electrical storm (VT-ES) or recurrent VT and cardiogenic shock, risk stratification tools have been developed to identify patients at high risk of acute hemodynamic decompensation during CA. These patients require a multidisciplinary approach and might need mechanical circulatory support (MCS) if CA is selected as the treatment strategy. Alternatively, less invasive therapies targeting the autonomic nervous system may be reasonable. In the chronic setting, developments in medical therapy have reduced the risk of sudden cardiac death in heart failure patients and stereotactic whole-body radiation (SBRT) has evolved as a potential, non-invasive therapy. Further research is needed to personalize VA therapy for individual patients.

Cardiac magnetic resonance imaging in the evaluation and management of mitral valve prolapse - a comprehensive review.

Mitral valve prolapse is a common valve disorder that usually has a benign prognosis unless there is significant regurgitation or LV impairment. However, a subset of patients are at an increased risk of ventricular arrhythmias and sudden cardiac death, which has led to the recognition of "arrhythmic mitral valve prolapse" as a clinical entity. Emerging risk factors include mitral annular disjunction and myocardial fibrosis. While echocardiography remains the primary method of evaluation, cardiac magnetic resonance has become crucial in managing this condition. Cine magnetic resonance sequences provide accurate characterization of prolapse and annular disjunction, assessment of ventricular volumes and function, identification of early dysfunction and remodeling, and quantitative assessment of mitral regurgitation when integrated with flow imaging. However, the unique strength of magnetic resonance lies in its ability to identify tissue changes. T1 mapping sequences identify diffuse fibrosis, in turn related to early ventricular dysfunction and remodeling. Late gadolinium enhancement sequences detect replacement fibrosis, an independent risk factor for ventricular arrhythmias and sudden cardiac death. There are consensus documents and reviews on the use of cardiac magnetic resonance specifically in arrhythmic mitral valve prolapse. However, in this article, we propose an algorithm for the broader use of cardiac magnetic resonance in managing this condition in various scenarios. Future advancements may involve implementing techniques for tissue characterization and flow analysis, such as 4D flow imaging, to identify patients with ventricular dysfunction and remodeling, increased arrhythmic risk, and more accurate grading of mitral regurgitation, ultimately benefiting patient selection for surgical therapy.

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.

Excellent Outcomes After First-Line Ablation in Post-MI Patients With Tolerated VT and LVEF >35.

Post-myocardial infarction (MI) patients with ventricular tachycardia (VT) are considered at risk for VT recurrence and sudden cardiac death (SCD). Recent guidelines indicate that in selected patients catheter ablation should be considered instead of an implantable cardioverter-defibrillator (ICD). This study aimed to analyze outcomes of patients referred for VT ablation according to left ventricular ejection fraction (LVEF), tolerance of VT, and acute ablation outcome. Post-MI patients without prior ICD undergoing VT ablation at a single center between 2009 and 2022 were included. Patients who presented with tolerated VT and who had an LVEF >35% were offered catheter ablation as first-line therapy. ICD implantation was offered to all patients but was subject to shared decision according to clinical presentation, LVEF, and ablation outcome. Eighty-six patients (mean age 69 ± 9 years, 84% male, mean LVEF 41 ± 9%) underwent VT ablation. In 66 patients, LVEF was >35%, of whom 51 had tolerated VT. Of these 51 patients, 37 (73%) were rendered noninducible. In 5 of 37 noninducible and in 11 of 14 inducible patients, an ICD was implanted. During a median follow-up of 40months (Q1-Q3: 24-70months), 10 of 86 patients had VT recurrence. The overall mortality was 27%, and 1 patient with ICD died suddenly. Among the 37 patients (none on antiarrhythmic drugs) with LVEF >35%, tolerated VT, and noninducibility, no SCD or VT recurrence occurred. Among the 14 patients with LVEF >35%, tolerated VT, and inducibility after ablation, no SCD occurred, but VT recurred in 29%. Post-MI patients with LVEF >35%, tolerated VT, and noninducibility after ablation have an excellent prognosis. Deferring ICD implantation seems to be safe in these patients.

The domperidone dilemma in Pakistan: Balancing access and safety concerns.

Madam, Domperidone is used in the treatment of nausea and vomiting as an antiemetic and prokinetic drug. Its pro-kinetic effects mainly manifest in the upper gastrointestinal tract by acting as a dopamine receptor antagonist [1]. However, the drug has been banned by the FDA in the USA due to its association with cardiac arrhythmias and cardiac arrest. A recent meta-analysis has shown a statistically significant association between sudden cardiac death [2] and the use of domperidone which raises the question of drug usage in Pakistan as an over-the-counter medication. The meta-analysis considers six different case-control studies, between 2005 and 2016, that have shown an increase in the risk of sudden cardiac death and ventricular arrhythmias associated with domperidone. The observational studies were conducted in North America, Asia, and Europe, proving that it is a global phenomenon, stemming from the use of the drug. The forest plot of the studies has been mentioned later in the abstract showing heterogeneity at 0%, OR ratio 1.69 with 95% CI (1.46-1.95). To further highlight the risks associated with domperidone, another study has reported that the safety index of the drug is 5.25, which is remarkably lower than the international safety ratio of 30[3]. The safety index, or therapeutic index, is the ratio of the toxic dose in 50% of the population (TD50) and the effective dose in 50% of the population (ED50) with the same therapeutic effect. A wider safety index indicates more room for error in the drug's therapeutic effect; essentially a larger barrier before the drug is toxic. With a safety index of 5.25, the action potential duration is markedly increased, resulting in arrhythmias. The author also comments on restricting the drug only to patients where the potential benefit is proven to justify the risks. In Pakistan, Domperidone is readily available as an over-the-counter drug. The casual availability raises the concern that the associated safety issues must be critically evaluated to determine whether the uses of the drug outweigh the risk of sudden cardiac death and ventricular arrhythmias [4] mentioned in these studies.

The influence of vigorous physical exertion on cardiac demand under conditions of daily living among firefighters with elevated blood pressure

BackgroundFirefighters have a high prevalence of cardiovascular disease. The poor heart health of firefighters is implicated in their increased risk of sudden cardiac death (SCD). Exercise may be protective against SCD partially due to the immediate blood pressure (BP) reductions of 5–8 mmHg following exercise, termed postexercise hypotension (PEH) ObjectivesTo examine PEH under ambulatory conditions after a maximal cardiopulmonary exercise test (CPET) among career firefighters MethodsFirefighters (n = 19) completed a maximal CPET and non-exercise control (CONTROL) in random order on separate non-workdays and left the laboratory instrumented to an ambulatory BP (ABP) monitor. Ambulatory systolic BP (ASBP), diastolic BP (ADBP), and heart rate (AHR) were recorded at hourly intervals over 19hr. The ambulatory rate pressure product (ARPP) was calculated as ASBPxAHRx10–3 at each hourly interval. Repeated measures ANCOVA tested if the ABP, AHR, and ARPP responses differed after CPET vs CONTROL over 19hr ResultsFirefighters were middle-aged (39.5 ± 8.9 yr), overweight (29.2 ± 4.0 kg/m2) men with elevated BP (123.1 ± 9.6/79.8 ± 10.4 mmHg), while resting HR (67.7 ± 11.3 bpm) and RPP (8.4 ± 1.7mmHg*bpm*10–3) were in normal ranges. ASBP (16.6 ± 5.7 mmHg) and ADBP (3.1 ± 4.6 mmHg) increased after the CPET vs CONTROL over 19hr (ps<0.01), as did AHR (9.4 ± 7.9 bpm, p = 0.02) and ARPP (2.5 ± 1.1mmHg*bpm*10–3, p < 0.01). ConclusionsUnexpectedly, the firefighters exhibited postexercise hypertension rather than PEH. The increases in ABP and AHR we observed indicated a sustained increase in cardiac demand. Further investigation is needed to confirm our findings and determine whether the adverse hemodynamic responses we observed contribute to the high prevalence of SCD that firefighters experience on the job.

An unusual cause of inappropriate shocks delivered by an implantable cardioverter defibrillator

IntroductionCardiac implantable electronic device (CIED) complications present significant challenges in clinical practice, especially in elderly patients with multiple comorbidities. Common adverse events include infection, lead malfunction, and device migration. Twiddler’s Syndrome, a rare but serious CIED complication characterised by patient manipulation causing lead displacement and device malfunction, is often underreported. The literature consists mainly of case reports and small series, providing limited guidance on prevention and management. As CIEDs are critical for managing cardiac arrhythmias and heart failure, understanding and addressing Twiddler’s Syndrome is essential. This case report aims to contribute to the literature by detailing a case of Twiddler’s Syndrome, emphasising the importance of a multidisciplinary approach for optimal management.Case PresentationA 59-year-old male presented with discomfort around his implantable cardioverter defibrillator (ICD) site and the sternal area over the past two days. He denied pain, dyspnoea, or dizziness. Clinical examination revealed a normal heart rhythm and no peripheral pulse deficit. Ultrasound revealed a reduced left ventricular ejection fraction. The atrial lead was not visible, and the shock coil was misplaced. ICD interrogation showed inappropriate shocks due to sensing artifacts and exit block in both leads, with no arrhythmias detected. An X-ray confirmed lead dislodgement and significant entanglement in the pocket. The patient was diagnosed with Twiddler’s Syndrome and scheduled for surgical revision.Discussion/ConclusionsDilated cardiomyopathy (DCM), characterised by left ventricular dilatation and dysfunction, accounts for a significant proportion of systolic heart failure cases. Despite advancements in heart failure management, DCM patients remain at high risk for sudden cardiac death (SCD), making ICD implantation crucial. However, CIED placement carries risks of complications, including Twiddler’s Syndrome. This condition can lead to lead dislodgement and device malfunction, resulting in inappropriate shocks and potential patient harm. In this case, a single-session extraction and re-implantation were successfully performed using a multidisciplinary approach, emphasising the importance of comprehensive management strategies to address such complications effectively. Regular follow-up showed no adverse events, highlighting the procedure’s success and the potential benefits of using advanced antimicrobial adjuncts to prevent infections. This case underscores the need for awareness and standardised protocols for managing Twiddler’s Syndrome to improve patient outcomes in the growing population of CIED recipients.

Cardiomyopathy and Sudden Cardiac Death: Bridging Clinical Practice with Cutting-Edge Research.

Sudden cardiac death (SCD) prevention in cardiomyopathies such as hypertrophic (HCM), dilated (DCM), non-dilated left ventricular (NDLCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC) remains a crucial but complex clinical challenge, especially among younger populations. Accurate risk stratification is hampered by the variability in phenotypic expression and genetic heterogeneity inherent in these conditions. This article explores the multifaceted strategies for preventing SCD across a spectrum of cardiomyopathies and emphasizes the integration of clinical evaluations, genetic insights, and advanced imaging techniques such as cardiac magnetic resonance (CMR) in assessing SCD risks. Advanced imaging, particularly CMR, not only enhances our understanding of myocardial architecture but also serves as a cornerstone for identifying at-risk patients. The integration of new research findings with current practices is essential for advancing patient care and improving survival rates among those at the highest risk of SCD. This review calls for ongoing research to refine risk stratification models and enhance the predictive accuracy of both clinical and imaging techniques in the management of cardiomyopathies.

The place and cause of death of Japanese peritoneal dialysis patients

BackgroundAlthough 70% of Japanese individuals wish to die at home, the in-hospital death rate in Japan is 71%. The in-hospital death rate is more than 80% in dialysis patients, most of whom are hemodialysis patients. Few reports exist on the relationship between place and cause of death in peritoneal dialysis (PD) patients.MethodsWe conducted a retrospective study of PD patients who died between January 2008 and June 2022 at three facilities.ResultsNinety-four patients died, 71% in hospital, 25% at home, and 4% in elderly care facilities. The in-hospital death rates were 67%, 74%, and 71% (P = 0.90) in the three facilities. Of the 67 in-hospital deaths, infection was the most common cause of death (30%), followed by sudden cardiac death (SCD) (15%), senility/cachexia (15%), heart failure (12%), and malignancy (9%). In the out-of-hospital deaths, there were no cases of infection, and SCD accounted for the majority (59%), followed by senility/cachexia (11%), heart failure (7%), stroke (4%), and malignancy (4%). Limiting to SCD, patients who died outside the hospital were significantly younger and had a higher prevalence of ischemic heart disease than those who died in hospital.ConclusionsThe in-hospital death rate for PD patients was lower than that previously reported for dialysis patients and similar to that of the Japanese population. Based on the place and cause of death in PD patients, to further increase the death rate at home, which many PD patients would have preferred, it may be necessary to prevent infectious diseases, strengthen interventions for patients at high risk of SCD, and shift to home care for patients with senility/cachexia and malignancies.

Cardiomyopathies matter: a multistakeholder european initiative to improve patient outcomes through effective health policies

Abstract Background/Introduction Cardiomyopathies are common and affect up to 1:250 of the population. Despite conferring a risk for sudden cardiac death and substantial disease burdens that could be reduced through better awareness, detection and care, cardiomyopathy care is rarely incorporated in European and national health policies. New European Society of Cardiology cardiomyopathy guidelines (1) could substantially benefit care but need to be supported by effective policies. Cardiomyopathies Matter is a Europe-wide, multistakeholder initiative aiming to raise awareness about cardiomyopathies and to ensure they receive the policy attention they warrant from EU and national decision-makers. Methods Cardiomyopathies Matters participants include experts from the fields of cardiology, cardiac nursing, economics, and patient advocacy, coordinated by a Secretariat, representing 36 countries and 6 patient advocacy groups (PAGs). A Policy Roadmap and other publications were co-created based on i) reviews of published and grey literature, and ii) input from topic experts and PAGs through 2 group workshops, individual consultation meetings, and a modified nominal group technique reviewing successive drafts toward consensus approval. Results The Roadmap (2) identifies unmet needs and challenges throughout the patient care pathway and offers holistic EU and national policy recommendations to address these and improve patient outcomes (Figure 1). These include policy measures to support early and accurate diagnosis, access to expert multidisciplinary care, guidelines-informed disease investigation and management, holistic support and empowerment of patients and carers, and research and innovation. Key aspects for nurses and allied health professionals include the need for 1)improved knowledge and awareness to promote early diagnosis; 2)rapid referral systems to multidisciplinary care and access to specialist cardiomyopathy or heart failure nurses care coordination, 3)genetic counselling (index cases and cascade/family screening), and 4)support of patients and families. Building on existing examples of good practice at national expert centre networks (e.g. France, Spain, UK),Cardiomyopathies Matter published updated policy recommendations for the European Union mandate (3), which are now being adopted more widely. National initiatives are underway or in development in Belgium, Hungary, Italy, Netherlands, Poland, and Spain. Local actions include multistakeholder roundtables and events, national Roadmap publications, events, educational activities, and media outreach. Conclusions EU and national Cardiovascular Health Action Plans are needed, with specific attention to cardiomyopathies and other inherited CVDs. Cardiomyopathies Matter fosters collaboration between healthcare professionals, PAGs and policymakers to promote policies that ensure all patients and families have access to guidelines-informed care.Figure 1Cardiomyopathy Priorities

The challenges in diagnosing hypertrophic cardiomyopathy in the presence of arterial hypertension: a clinical case

Aim. To describe a clinical case and analyze the challenges in diagnosing hypertrophic cardiomyopathy (HCM) in a patient with concomitant arterial hypertension (AH). Materials and methods. The article presents the clinical case of HCM in the patient with concomitant AH that was observed in an ambulatory setting in the Municipal Enterprise “Dnipropetrovsk Regional Clinical Center for Diagnostics and Treatment” of Dnipropetrovsk Regional Council. Results. A 66-year-old woman N., diagnosed by her family physician with coronary artery disease (CAD): stable angina FC II, grade 2 AH, chronic heart failure FC II NYHA, was referred to a cardiologist because of experiencing exertional dyspnea, chest pain, and uncontrolled blood pressure despite treatment compliance. Echocardiography identified concentric left ventricular hypertrophy with the left ventricular outflow tract (LVOT) obstruction (a mean gradient of 35 mmHg as per catheterization). Cardiac MRI confirmed the diagnosis and coronary angiography ruled out CAD. Adjustments to the treatment regimen, taking into account HCM with LVOT obstruction, effectively alleviated the patient’s symptoms and stabilized her blood pressure. Conclusions. It is especially important to follow the guidelines of AH management and perform echocardiography in all patients with high blood pressure, so as not to miss the signs of concomitant HCM, particularly with LVOT obstruction. In addition, in the case of HCM, it is necessary to timely detect, provide prevention and manage patients at risk for sudden cardiac death. Since HCM encompasses various diagnoses with different pathogenesis and distinct management, cardiac MRI, enzymatic or genetic testing may be needed according to guidelines. As HCM is a relatively common inherited cardiac disease, general practitioners could often encounter such patients in everyday clinical practice. Hence, they should have a certain suspicion of this diagnosis in persons with AH whose left ventricular mass meets the criteria for HCM.

Quality of life in patients with heart failure and implantable cardioverter defibrillator. Results from 42 heart failure clinics in norway

Abstract Background Patients with heart failure (HF) have an increased risk of sudden cardiac death, and treatment with an implantable cardioverter defibrillator (ICD) has become standard treatment for selected patient groups. HF is associated with impaired QoL, but there is no consensus regarding the implication of having an ICD. Knowledge regarding QoL in patients with HF and ICD attending follow-up in outpatient HF-clinics is sparse. Purpose The aim of this study was to determine QoL in patients with HF and ICD. Further, to compare QoL in patients with HF with and without ICD, using data from the first and follow-up visits in outpatient HF clinics. Methods Longitudinal data from the National Norwegian Heart Failure Registry (NNHFR), including 9 274 patients, of which 1 206 with an ICD, were studied. QoL was assessed using the Minnesota Living with Heart Failure Questionnaire (MLHFQ). Data was collected at the first and follow-up visits in outpatient HF clinics approximately six months after optimal treatment was achieved. Results Patients with an ICD were younger, more often men, and had higher frequency of coronary artery disease (all p&amp;lt;0.001). At the first visit, there was no significant difference in QoL between patients with and without ICD, with both groups reporting moderate QoL. Both groups had a significant improvement in QoL from the first visit to the follow-up visit (p&amp;lt;0.001). However, patients with ICD reported significantly less improvement in QoL (p&amp;lt;0.001) at follow-up. This disparity was evident regarding both the physical (p&amp;lt;0.001) and the emotional (p&amp;lt;0.001) dimension. Conclusion QoL in patients with HF and ICD improves after follow-up in outpatient HF clinics, although not to the same extent as in patients without ICD. Thus, tailored interventions in outpatient HF clinics may be necessary to improve outcomes in this subgroup of patients with both HF and ICD.

Racial Disparities in Sports Cardiology

ImportanceRacial disparities in cardiovascular health, including sudden cardiac death (SCD), exist among both the general and athlete populations. Among competitive athletes, disparities in health outcomes potentially influenced by social determinants of health (SDOH) and structural racism remain inadequately understood. This narrative review centers on race in sports cardiology, addressing racial disparities in SCD risk, false-positive cardiac screening rates among athletes, and the prevalence of left ventricular hypertrophy, and encourages a reexamination of race-based practices in sports cardiology, such as the interpretation of screening 12-lead electrocardiogram findings.ObservationsDrawing from an array of sources, including epidemiological data and broader medical literature, this narrative review discusses racial disparities in sports cardiology and calls for a paradigm shift in approach that encompasses 3 key principles: race-conscious awareness, clinical inclusivity, and research-driven refinement of clinical practice. These proposed principles call for a shift away from race-based assumptions towards individualized, health-focused care in sports cardiology. This shift would include fostering awareness of sociopolitical constructs, diversifying the medical team workforce, and conducting diverse, evidence-based research to better understand disparities and address inequities in sports cardiology care.Conclusions and RelevanceIn sports cardiology, inadequate consideration of the impact of structural racism and SDOH on racial disparities in health outcomes among athletes has resulted in potential biases in current normative standards and in the clinical approach to the cardiovascular care of athletes. An evidence-based approach to successfully address disparities requires pivoting from outdated race-based practices to a race-conscious framework to better understand and improve health care outcomes for diverse athletic populations.

Sudden cardiac death risk stratification in heritable cardiomyopathies: phenotype-based to genotype-based risk scores.

Sudden cardiac death risk stratification in heritable cardiomyopathies: phenotype-based to genotype-based risk scores.

Ventricular arrhythmias in the context of chronic kidney disease and electrolyte imbalance.

Patients with chronic kidney disease face ahigh risk of sudden cardiac death, particularly in more advanced stages of renal dysfunction. Ventricular arrhythmias are prevalent and contribute to the heightened cardiovascular mortality. This review aims to explore the intricate interplay of disease-specific risk factors, arrhythmic triggers, and electrolyte disorders that amplify susceptibility to ventricular arrhythmias and sudden cardiac death in this population and influence the efficacy of available treatments.

Enhancing patient acceptance of ICD implantation through structured shared decision making: conversation is key.

Implantable cardioverter-defibrillators (ICDs) and cardiac resynchronization therapy defibrillators (CRT-D) are lifesaving treatments for patients at risk for sudden cardiac death. Effective physician-patient communication during the shared decision-making process is essential. Electrophysiologist-patient conversations were targeted to obtain objective data on the interaction, understand the conversation framework, and uncover opportunities for improved communication. Individuals previously identified as requiring an ICD/CRT-D but declined implantation were recruited for this four-stage interview and survey-based study. Quantitative analysis of surveys and AI analysis of conversation videos was conducted to evaluate patient participant expectations, analyze feedback about the conversations with study physicians, and gauge willingness for device implantation. The study included 27 patients (mean age 51years, 51.9% female) and 9 study physicians. Patients were significantly more willing to undergo ICD/CRT-D implantation after conversing with study physicians compared to their own physicians and pre-conversation surveys (mean scores: 5.0, 3.1, and 4.4 out of 7, respectively; p < 0.001). Patient participants had higher satisfaction with the study conversation, rating study physicians higher in effectiveness of explanations, responsiveness to questions, and overall quality of the conversation compared to their own physicians (all p < 0.001). In a cohort of patients who previously declined ICD/CRT-D implantation, patient satisfaction and willingness to undergo implantation of a guideline-directed device therapy increased significantly following a structured conversation with study physicians. Identified key elements could be integrated into user-friendly tools and educational materials to facilitate these conversations, improving patient engagement with the decision-making process and enhancing informed acceptance of indicated device therapies.

Predictors of ventricular tachyarrhythmia in patients with a wearable cardioverter defibrillator: an international multicenter registry.

Wearable cardioverter defibrillator (WCD) can protect patients from sudden cardiac death due to ventricular tachyarrhythmias and serve as a bridge to decision of definite defibrillator implantation. The aim of this analysis from an international, multicenter WCD registry was to identify predictors of sustained ventricular tachycardia (VT) and/or ventricular fibrillation (VF) in this population. One thousand six hundred seventy-five patients with WCD were included in a multicenter registry from 9 European centers, with a median follow-up of 440days (IQR 120-893). The primary study end point was the occurrence of sustained VT/VF. Sustained VT was detected by WCD in 5.4% and VF in 0.9% of all patients. Of the 30.3% of patients receiving ICD implantation during follow-up, sustained VT was recorded in 9.3% and VF in 2.6%. Non-ischemic cardiomyopathy (HR 0.5, p < 0.001), and medication with angiotensin-converting enzyme inhibitors (HR 0.7, p = 0.027) and aldosterone antagonists (HR 0.7, p = 0.005) were associated with a significantly lower risk of VT/VF. Patients who received WCD due to a transient increased risk of sudden cardiac death have a comparatively lower risk of VT/VF in the presence of non-ischemic cardiomyopathy. Of note, optimal medical treatment for heart failure not only results in an improvement in left ventricular ejection fraction but also in a reduction in the risk for VT/VF.

Ventriculo-arterial (VA) coupling and fQRS as new selection criteria for primary prevention ICD placement

For decades, left ventricular ejection fraction (LVEF < 35%) has been a mainstay for identifying heart failure (HF) patients most likely to benefit from an implantable cardioverter defibrillator (ICD). However, LVEF is a poor predictor of sudden cardiac death (SCD) and ignores 50% of HF patients with mildly reduced and preserved LVEF. The current international guidelines for primary prophylaxis ICD therapy are inadequate. Instead of LVEF, which is not a good measure of LV contractility or hemodynamic characterization, we hypothesize ventriculo-arterial (VA) coupling combined with fragmented QRS (fQRS) will improve risk stratification and patient suitability for an ICD. Quantifying cardiac and aortic mechanics, and predicting active arrhythmogenic substrate, from varying fQRS morphologies, may help to stratify ischemic and non-ischemic patients with different functional capacities and predisposition for lethal arrhythmias. We propose HF patients with a low physiological reserve may not benefit from ICD therapy, whereas those patients with higher reserves and extensive arrhythmogenic substrate may benefit. Our hypothesis combining VA coupling with fQRS changes has the potential to widen HF patient participation (low and high LVEF) and advance personalized medicine for HF patients at high risk of SCD.

Genetic therapies for cardiomyopathy: survey of attitudes of the patient community for the CureHeart project

Cardiomyopathies are a group of inherited heart muscle disorders. Expressivity is variable and while sometimes mild, complications can result in sudden cardiac death (SCD) at any age, heart failure and stroke. In around a third of patients a monogenic cause is identifiable, and development of genetic therapies that aim to correct the underlying genetic defect is underway. Here we describe results of a survey designed to understand preliminary views of the patient community about genetic therapies in the context of disease burden. The internet survey was publicized with a bespoke information video via patient support groups in the UK and USA; 634 people responded of whom 96% had a personal and/or family history of cardiomyopathy. Findings show that concern about cardiomyopathy-related issues with a future dimension, such as disease progression, is significantly greater than concern about current issues. A total of 93.6% thought that genetic therapies should be developed for cardiomyopathy. A majority would consider participation in a genetic therapy trial in six scenarios varying by age and clinical situation significantly more in the scenario of an adult with symptomatic disease and evident progression than an asymptomatic adult with SCD risk, or a child. In all scenarios, a majority said that the chance genetic therapy would stop or slow progression, and risk of serious adverse and unintended effects, were important considerations. Qualitative analysis of free-text responses found that concern was often informed by family experience. Patient consideration of genetic therapy is likely to require individualized assessment of the benefits and risks.

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.