Complicaciones y descargas inapropiadas tras el implante de un desfibrilador subcutáneo en pacientes pediátricos / Complications and inappropriate shocks in pediatric patients receiving a subcutaneous implantable cardioverter defibrilator

Introducción y objetivos: La experiencia con el desfibrilador automático implantable subcutáneo (DAI-SC) en pacientes pediátricos aún es reducida. El objetivo de este estudio es determinar la incidencia de complicaciones en pacientes pediátricos de nuestro centro en función del tipo de DAI y del tamaño del paciente.MétodosSe incluyó a pacientes menores de 18 años que recibieron un DAI-SC desde 2016 y pacientes contemporáneos (desde 2014) que recibieron un DAI transvenoso (DAI-TV). El evento principal fue el combinado de complicaciones y descargas inapropiadas.ResultadosSe implantó un DAI-SC a 26 pacientes (edad, 14 [intervalo, 5-17] años; índice de masa corporal [IMC], 20,2). De ellos, 23 (88%) fueron implantes intermusculares y el resto, en subserrato, 24 (92%) con 2 incisiones. Se programaron 2 zonas en todos los pacientes: condicional a 230 (220-230) lpm y de choque a 250 lpm. El grupo de DAI-TV incluyó a 19 pacientes (edad, 11 [5-16] años; IMC, 19,2; el 79% monocamerales). La supervivencia libre del evento principal a 5 años fue el 80% de los pacientes con DAI-SC y el 63% del grupo con DAI-TV (p=0,54); la de descargas inapropiadas fue similar (el 85 frente al 89%; p=0,86), mientras que la de complicaciones fue mayor en el grupo de DAI-SC (el 96 frente al 57%; cloglog p=0.016). En el grupo de DAI-SC no hubo fallo de la terapia ni mayores complicaciones con un IMC ≤ 20.ConclusionesCon las técnicas de implante y programación actuales, el DAI-SC es eficaz y seguro en pacientes pediátricos, con similares descargas inapropiadas y menos complicaciones a corto y medio plazo que el DAI-TV. (AU)

Introduction and objectives: There is limited evidence regarding the use of subcutaneous implantable cardioverter-defibrillators (S-ICD) in pediatric patients. The aim of this study was to determine the incidence of complications in these patients at our center, according to the type of ICD and patient size.MethodsWe included all patients aged<18 years who received an S-ICD since 2016 at our center. As a control group, we also included contemporary patients (since 2014) who received a transvenous ICD (TV-ICD). The primary endpoint was a composite of complications and inappropriate shocks.ResultsA total of 26 patients received an S-ICD (median age, 14 [5-17] years; body mass index [BMI], 20.2 kg/m2). Implantation was intermuscular in 23 patients (88%) and subserratus in the remainder. Two incisions were used in 24 patients (92%). In all patients, 2 zones were programmed: a conditional zone set at 230 (220-230) bpm, and a shock zone set at 250 bpm. Nineteen patients received a TV-ICD (median age, 11 [range, 5-16] years; BMI, 19.2 kg/m2, 79% single-chamber). Survival free from the primary endpoint at 5 years was 80% in the S-ICD group and 63% in the TV-ICD group (P=.54). Survival free from inappropriate shocks was similar (85% vs 89%, P=.86), while survival free from complications was higher in the S-ICD group (96% vs 57%, cloglogP=.016). There were no therapy failures in the S-ICD group, and no increased complication rates were observed in patients with BMI ≤ 20 kg/m2.ConclusionsWith contemporary implantation techniques and programming, S-ICD is a safe and effective therapy in pediatric patients. The number of inappropriate shocks is similar to TV-ICD, with fewer short- and mid-term complications. (AU)

Multiparametric Mapping via Cardiovascular Magnetic Resonance in the Risk Stratification of Ventricular Arrhythmias and Sudden Cardiac Death.

Risk stratification for malignant ventricular arrhythmias and sudden cardiac death is a daunting task for physicians in daily practice. Multiparametric mapping sequences obtained via cardiovascular magnetic resonance imaging can improve the risk stratification for malignant ventricular arrhythmias by unveiling the presence of pathophysiological pro-arrhythmogenic processes. However, their employment in clinical practice is still restricted. The present review explores the current evidence supporting the association between mapping abnormalities and the risk of ventricular arrhythmias in several cardiovascular diseases. The key message is that further clinical studies are needed to test the additional value of mapping techniques beyond conventional cardiovascular magnetic resonance imaging for selecting patients eligible for an implantable cardioverter defibrillator.

[Rhythm management in patients with heart failure].

INTRODUCTION: Arrhythmias manifest frequently in individuals with heart failure, posing a notable threat of mortality and morbidity. While the prevention of sudden cardiac death through ICD therapy remains pivotal, accurate risk stratification remains a challenging task even in 2024. Recent data underscore the early consideration of catheter ablation for ventricular tachycardias. Although antiarrhythmic drug therapy serves as an ancillary measure for symptomatic patients, it does not confer prognostic advantages. The holistic management of arrhythmias in heart failure necessitates a systematic, multidimensional approach that initiates with evidence-based medical therapy for heart failure and integrates device-based and interventional therapies. Noteworthy clinical studies have illustrated the positive prognostic impact of early rhythm control strategies, particularly catheter ablation, in individuals managing heart failure and atrial fibrillation.

[Cardiomyopathies: a practical approach to the assessment and management of patients and their families]. / Kardiomyopathien: ein praktischer Ansatz für Abklärung und Management der betroffenen Patienten und deren Familien.

INTRODUCTION: The new 2023 European Society of Cardiology (ESC) Guidelines for the management of cardiomyopathies addresses all cardiomyopathies in a single document for the first time. The focus is on a phenotype-oriented diagnostic approach, multimodal imaging and genetic testing to establish the most accurate diagnosis possible. Additionally, new recommendations for risk stratification for sudden cardiac death in various cardiomyopathy phenotypes are provided. MRI and genetic testing have significantly gained importance in this context. Recommendations for comprehensive clinical and genetic cascade screening in relatives of individuals with cardiomyopathies have been revised. This article presents the most important innovations of these guidelines in a practice-oriented approach.

Arrhythmogenic right ventricular cardiomyopathy: Unveiling clinical presentations, CMR insights and prognosis in a single-center retrospective study.

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a hereditary condition with a prevalence ranging from 1 in 2000 to 1 in 5000 individuals. ARVC is a significant contributor to sudden cardiac death, particularly in young individuals and athletes, and remains challenging to diagnose definitively. We conducted a single-center retrospective study to evaluate the presentations, electrocardiogram findings, and imaging characteristics of ARVC patients evaluated at our center between 2021 and 2023. Notably, our study is the second investigation of ARVC conducted in Pakistan. We report divergent symptom prevalence as compared to the current literature and have incorporated the Task Force Criteria. Despite limited access to cardiac magnetic resonance (CMR) facilities worldwide, our findings underscore the critical role ofCMR in ARVC diagnosis. Our cohort had a mortality rate of 17 % highlighting the importance of early detection and the need for improved diagnostic facilities for ARVC in the region.

Sex differences among subcutaneous implantable cardioverter-defibrillator recipients: a propensity-matched, multicentre, international analysis from the i-SUSI project.

AIMS: Women have been historically underrepresented in implantable cardioverter-defibrillator (ICD) trials. No data on sex differences regarding subcutaneous ICDs (S-ICD) carriers have been described. Aim of our study was to investigate sex-related differences among unselected S-ICD recipients. METHODS AND RESULTS: Consecutive patients enrolled in the multicentre, international i-SUSI registry were analysed. Comparisons between sexes were performed using a 1:1 propensity matching adjusted analysis for age, body mass index (BMI), left ventricular function, and substrate. The primary outcome was the rate of appropriate shocks during follow-up. Inappropriate shocks and other device-related complications were deemed secondary outcomes. A total of 1698 patients were extracted from the i-SUSI registry; 399 (23.5%) were females. After propensity matching, two cohorts of 374 patients presenting similar baseline characteristics were analysed. Despite similar periprocedural characteristics and a matched BMI, women resulted at lower risk of conversion failure as per PRAETORIAN score (73.4% vs. 81.3%, P = 0.049). Over a median follow-up time of 26.5 [12.7-42.5] months, appropriate shocks were more common in the male cohort (rate/year 3.4% vs. 1.7%; log-rank P = 0.049), while no significant differences in device-related complications (rate/year: 6.3% vs. 5.8%; log-rank P = 0.595) and inappropriate shocks (rate/year: 4.3% vs. 3.1%; log-rank P = 0.375) were observed. After controlling for confounders, sex remained significantly associated with the primary outcome (aHR 1.648; CI 0.999-2.655, P = 0.048), while not resulting predictor of inappropriate shocks and device-related complications. CONCLUSION: In a propensity-matched cohort of S-ICD recipients, women are less likely to experience appropriate ICD therapy, while not showing higher risk of device-related complications. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT0473876.

Long-term follow-up of patients with Brugada syndrome: Foremost risk factors associated with overall arrhythmic events.

Brugada syndrome (BS) is characterized by ST segment elevation in right precordial leads (V1-V3), ventricular tachycardia (VT), ventricular fibrillation (VF), and sudden cardiac death (SCD) in individuals without structural heart disease. The aim of this study is to contribute to the controversial issue of finding the most valuable marker that can predict poor prognosis during follow-up in patients with a diagnosis of BS. A total of 68 patients diagnosed with BS or had Brugada-type ECG change between January 1997 and July 2012 at the Department of Cardiology of Baskent University Faculty of Medicine, Ankara, Turkey, were included in this cohort study. Patients were screened every 6 months for arrhythmia-related syncope, SCD, appropriate and inappropriate defibrillation (shock), AF development and death; collectively defined as "arrhythmic events" and were the primary endpoints. Patients with and without arrhythmic events were compared. The mean age was 34.9 ±â€…12.2 years (9-71 years), and 52 (76.5%) patients were male. Mean follow-up was 49.6 ±â€…37.6 months (4-188 months). Univariate analysis showed that male sex (P = .004), type 1 electrocardiographic pattern (P = .008), SCD (P = .036), VT/VF history (P = .046), requirement for electrophysiological studies (P = .034), implantable cardioverter-defibrillator placement (P = .014) were found to demonstrate significant differences in patients with and without arrhythmic events. In multivariable analyzes, spontaneous type 1 ECG presence (HR = 8.54, 95% CI: 0.38-26.37; P = .003) and VT/VF history (HR = 9.21, 95% CI: 0.004-1.88; P = .002) were found to be independently associated with arrhythmic events. We found the presence of spontaneous type 1 ECG and a history of VT/VF to be associated with increased likelihood of overall arrhythmic events in BS. Given the higher risk of poor prognosis, we recommend additional measures in patients with BS who have these features.

Viral myocarditis in combination with genetic cardiomyopathy as a cause of sudden death. An autopsy series.

Sudden cardiac death (SCD) is a major public health issue worldwide. In the young (< 40 years of age), genetic cardiomyopathies and viral myocarditis, sometimes in combination, are the most frequent, but underestimated, causes of SCD. Molecular autopsy is essential for prevention. Several studies have shown an association between genetic cardiomyopathies and viral myocarditis, which is probably underestimated due to insufficient post-mortem investigations. We report on four autopsy cases illustrating the pathogenesis of these combined pathologies. In two cases, a genetic hypertrophic cardiomyopathy was diagnosed in combination with Herpes Virus Type 6 (HHV6) and/or Parvovirus-B19 (PVB19) in the heart. In the third case, autopsy revealed a dilated cardiomyopathy and virological analyses revealed acute myocarditis caused by three viruses: PVB19, HHV6 and Epstein-Barr virus. Genetic analyses revealed a mutation in the gene coding for desmin. The fourth case illustrated a channelopathy and a PVB19/HHV6 coinfection. Our four cases illustrate the highly probable deleterious role of cardiotropic viruses in the occurrence of SCD in subjects with genetic cardiomyopathies. We discuss the pathogenetic link between viral myocarditis and genetic cardiomyopathy. Molecular autopsy is essential in prevention of these SCD, and a close collaboration between cardiologists, pathologists, microbiologists and geneticians is mandatory.

[Recommendations for competitive sports eligibility: what's new in the 2023 COCIS protocols]. / Raccomandazioni per il giudizio di idoneità allo sport agonistico: le novità del COCIS 2023.

The benefit of physical exercise is well established, but, at the same time, it is now well known that an intense sports activity can trigger adverse cardiac events and increase sport-related death. Since 1982, Italy has a State law which obliges athletes to undergo a pre-participation evaluation, based on history, physical examination, ECG and stress test. From its introduction, a significant reduction in cardiac sport-related adverse events has been shown. During the pre-participation screening, some cardiological issues or suspects can arise and the sports medicine doctor should deal with them before releasing the certification for participation in competitive sport. In order to give precious advices to these colleagues and help athletes to securely practice sport, the Italian Society of Sports Cardiology, the Italian Federation of Sports Medicine and the other cardiological scientific societies gathered in the COCIS Committee, periodically produce and publish a booklet named "Cardiological Protocols for Competitive Sports Eligibility". The object of this review is to underline the recent 2023 version innovations when compared to previous editions.

[Treatment with cardiac electronic implantable devices]. / Therapie mit kardialen elektronischen implantierbaren Devices.

Cardiac device therapy provides not only treatment options for bradyarrhythmia but also advanced treatment for heart failure and preventive measures against sudden cardiac death. In heart failure treatment it enables synergistic reverse remodelling and reduces pharmacological side effects. Cardiac resynchronization therapy (CRT) has revolutionized the treatment of reduced left ventricular ejection fraction (LVEF) and left bundle branch block by decreasing the mortality and morbidity with improvement of the quality of life and resilience. Conduction system pacing (CSP) as an alternative method of physiological stimulation can improve heart function and reduce the risk of pacemaker-induced cardiomyopathy. Leadless pacers and subcutaneous/extravascular defibrillators offer less invasive options with lower complication rates. The prevention of infections through preoperative and postoperative strategies enhances the safety of these therapies.

Management of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is an important cause of heart failure and arrhythmias, including sudden death, with a major impact on the healthcare system. Genetic causes and different phenotypes are now increasingly being identified for this condition. In addition, specific medications, such as myosin inhibitors, have been recently shown as potentially able to modify its symptoms, hemodynamic abnormalities and clinical course. Our article aims to provide a comprehensive outline of the epidemiology, diagnosis and treatment of hypertrophic cardiomyopathy in the current era.

The association between GRACE score at admission for myocardial infarction and the incidence of sudden cardiac arrests in long-term follow-up - the MADDEC study.

Background. Sudden cardiac arrest (SCA), often also leading to sudden cardiac death (SCD), is a common complication in coronary artery disease. Despite the effort there is a lack of applicable prediction tools to identify those at high risk. We tested the association between the validated GRACE score and the incidence of SCA after myocardial infarction. Material and methods. A retrospective analysis of 1,985 patients treated for myocardial infarction (MI) between January 1st 2015 and December 31st 2018 and followed until the 31st of December of 2021. The main exposure variable was patients' GRACE score at the point of admission and main outcome variable was incident SCA after hospitalization. Their association was analyzed by subdistribution hazard (SDH) model analysis. The secondary endpoints included SCA in patients with no indication to implantable cardioverter-defibrillator (ICD) device and incident SCD. Results. A total of 1985 patients were treated for MI. Mean GRACE score at baseline was 118.7 (SD 32.0). During a median follow-up time of 5.3 years (IQR 3.8-6.1 years) 78 SCA events and 52 SCDs occurred. In unadjusted analyses one SD increase in GRACE score associated with over 50% higher risk of SCA (SDH 1.55, 95% CI 1.29-1.85, p < 0.0001) and over 40% higher risk for SCD (1.42, 1.12-1.79, p = 0.0033). The associations between SCA and GRACE remained statistically significant even with patients without indication for ICD device (1.57, 1.30-1.90, p < 0.0001) as well as when adjusting with patients LVEF and omitting the age from the GRACE score to better represent the severity of the cardiac event. The association of GRACE and SCD turned statistically insignificant when adjusting with LVEF. Conclusions. GRACE score measured at admission for MI associates with long-term risk for SCA.

What is already known about this subject?Nearly 50% of cardiac mortality is caused by sudden cardiac death, often due to sudden cardiac arrest.Despite the effort, there is a lack of applicable prediction tools to identify those at high risk.What does this study add?This study shows that GRACE score measured at the point of admission for myocardial infarction can be used to evaluate patients' risk for sudden cardiac arrest in a long-term follow-up.How might this impact on clinical practice?Based on our findings, the GRACE score at the point of admission could significantly affect the patients' need for an ICD device after hospitalization for MI and should be considered as a contributing factor when evaluating the patients' follow-up care.

Long-term reliability of the phospholamban (PLN) p.(Arg14del) risk model in predicting major ventricular arrhythmia: a landmark study.

AIMS: Recently, a genetic variant-specific prediction model for phospholamban (PLN) p.(Arg14del)-positive individuals was developed to predict individual major ventricular arrhythmia (VA) risk to support decision-making for primary prevention implantable cardioverter defibrillator (ICD) implantation. This model predicts major VA risk from baseline data, but iterative evaluation of major VA risk may be warranted considering that the risk factors for major VA are progressive. Our aim is to evaluate the diagnostic performance of the PLN p.(Arg14del) risk model at 3-year follow-up. METHODS AND RESULTS: We performed a landmark analysis 3 years after presentation and selected only patients with no prior major VA. Data were collected of 268 PLN p.(Arg14del)-positive subjects, aged 43.5 ± 16.3 years, 38.9% male. After the 3 years landmark, subjects had a mean follow-up of 4.0 years (± 3.5 years) and 28 (10%) subjects experienced major VA with an annual event rate of 2.6% [95% confidence interval (CI) 1.6-3.6], defined as sustained VA, appropriate ICD intervention, or (aborted) sudden cardiac death. The PLN p.(Arg14del) risk score yielded good discrimination in the 3 years landmark cohort with a C-statistic of 0.83 (95% CI 0.79-0.87) and calibration slope of 0.97. CONCLUSION: The PLN p.(Arg14del) risk model has sustained good model performance up to 3 years follow-up in PLN p.(Arg14del)-positive subjects with no history of major VA. It may therefore be used to support decision-making for primary prevention ICD implantation not merely at presentation but also up to at least 3 years of follow-up.

Cost effectiveness of implantable cardioverter defibrillators for 1.5 primary prevention of sudden cardiac arrest in China: an analysis from the Improve SCA study.

OBJECTIVES: Implantable cardioverter defibrillator (ICDs) for primary prevention (PP) of sudden cardiac arrest (SCA) is underutilized in developing countries. The Improve SCA study has identified a subset of 1.5 primary prevention (1.5PP) patients with a higher risk of SCA and a significant mortality benefit from ICD therapy. From the perspective of China's healthcare system, we evaluated the cost-effectiveness of ICD therapy vs. no ICD therapy among 1.5PP patients with a view to informing clinical and policy decisions. METHODS: A published Markov model was adjusted and verified to simulate the course of the disease and describe different health states of 1.5PP patients. The patient characteristics, mortality, utility and complication estimates were obtained from the Improve SCA study and other literature. Cost inputs were sourced from government tender prices, medical service prices and clinical experts' surveys in 9 Chinese public hospitals. For both ICD and no ICD therapy, the total medical costs and quality-adjusted life-years (QALYs) were modelled over a lifetime horizon and the incremental cost-effectiveness ratio (ICER) was calculated. Deterministic and probabilistic sensitivity analyses were performed to assess the uncertainty of the model parameters. We used the willingness-to-pay (WTP) threshold recommended by China Guidelines for Pharmacoeconomic Evaluations, one to three times China's GDP per capita (CNY85,698-CNY257,094) in 2022 Chinese Yuan. RESULTS: The incremental cost effectiveness ratio (ICER) of ICD therapy compared to no ICD therapy is 139,652 CNY/QALY, which is about 1-2 times China's GDP per capita. The probability that ICD therapy is cost effective was 92.1%. Results from sensitivity analysis supported the findings of the base case. CONCLUSIONS: ICD therapy compared to no ICD therapy is cost-effective for the 1.5PP patients in China.

The role for ambulatory electrocardiogram monitoring in the diagnosis and prognostication of Brugada syndrome: a sub-study of the Rare Arrhythmia Syndrome Evaluation (RASE) Brugada study.

AIMS: Brugada syndrome (BrS) diagnosis and risk stratification rely on the presence of a spontaneous type 1 (spT1) electrocardiogram (ECG) pattern; however, its spontaneous fluctuations may lead to misdiagnosis and risk underestimation. This study aims to assess the role for repeat high precordial lead (HPL) resting and ambulatory ECG monitoring in identifying a spT1, and evaluate its prognostic role. METHODS AND RESULTS: HPL resting and ambulatory monitoring ECGs of BrS subjects were reviewed retrospectively, and the presence of a spT1 associated with ventricular dysrhythmias and sudden cardiac death (SCD). Three-hundred and fifty-eight subjects (77 with spT1 pattern at presentation, Group 1, and 281 without, Group 2) were included. In total, 1651 resting HPL resting and 621 ambulatory monitoring ECGs were available for review, or adequately described. Over a median follow-up of 72 months (interquartile range - IQR - 75), 42/77 (55%) subjects in Group 1 showed a spT1 in at least one ECG. In Group 2, 36/281 subjects (13%) had a newly detected spT1 (1.9 per 100 person-year) and 23 on an HPL ambulatory recording (8%). Seven previously asymptomatic subjects, five of whom had a spT1 (four at presentation and one at follow-up), experienced arrhythmic events; survival analysis indicated that a spT1, either at presentation or during lifetime, was associated with events. Univariate models showed that a spT1 was consistently associated with increased risk [spT1 at presentation: hazard ratio (HR) 6.3, 95% confidence interval (CI) 1.4-28, P = 0.016; spT1 at follow-up: HR 3.1, 95% CI 1.3-7.2, P = 0.008]. CONCLUSION: Repeated ECG evaluation and HPL ambulatory monitoring are vital in identifying transient spT1 Brugada pattern and its associated risk.

Ethical use of artificial intelligence to prevent sudden cardiac death: an interview study of patient perspectives.

BACKGROUND: The emergence of artificial intelligence (AI) in medicine has prompted the development of numerous ethical guidelines, while the involvement of patients in the creation of these documents lags behind. As part of the European PROFID project we explore patient perspectives on the ethical implications of AI in care for patients at increased risk of sudden cardiac death (SCD). AIM: Explore perspectives of patients on the ethical use of AI, particularly in clinical decision-making regarding the implantation of an implantable cardioverter-defibrillator (ICD). METHODS: Semi-structured, future scenario-based interviews were conducted among patients who had either an ICD and/or a heart condition with increased risk of SCD in Germany (n = 9) and the Netherlands (n = 15). We used the principles of the European Commission's Ethics Guidelines for Trustworthy AI to structure the interviews. RESULTS: Six themes arose from the interviews: the ability of AI to rectify human doctors' limitations; the objectivity of data; whether AI can serve as second opinion; AI explainability and patient trust; the importance of the 'human touch'; and the personalization of care. Overall, our results reveal a strong desire among patients for more personalized and patient-centered care in the context of ICD implantation. Participants in our study express significant concerns about the further loss of the 'human touch' in healthcare when AI is introduced in clinical settings. They believe that this aspect of care is currently inadequately recognized in clinical practice. Participants attribute to doctors the responsibility of evaluating AI recommendations for clinical relevance and aligning them with patients' individual contexts and values, in consultation with the patient. CONCLUSION: The 'human touch' patients exclusively ascribe to human medical practitioners extends beyond sympathy and kindness, and has clinical relevance in medical decision-making. Because this cannot be replaced by AI, we suggest that normative research into the 'right to a human doctor' is needed. Furthermore, policies on patient-centered AI integration in clinical practice should encompass the ethics of everyday practice rather than only principle-based ethics. We suggest that an empirical ethics approach grounded in ethnographic research is exceptionally well-suited to pave the way forward.