Identification of a SCN5A Genetic Variant Associated With Type 1 Brugada Syndrome (BrS) in a Family.

The Brugada pattern is associated with a genetic disorder characterized by ST-segment elevation in the right precordial leads on electrocardiogram (EKG) in the absence of structural heart disease. Patients with the Brugada pattern have an increased risk for ventricular tachyarrhythmia and sudden cardiac death. Loss-of-function mutations in the SCN5A gene which encodes the alpha subunit of the cardiac sodium channel have been associated with Brugada syndrome (BrS). We report a case of a patient who was found to have a spontaneous type 1 Brugada pattern on a routine EKG done prior to travel. He underwent electrophysiological testing (EPS) which provoked ventricular tachycardia and underwent implantable cardioverter defibrillator (ICD) placement. His family history revealed a history of sudden cardiac death, abnormal EKG, syncope, dilated cardiomyopathy, and BrS. Genetic testing revealed a variant of uncertain significance (VUS) in the SCN5A gene in the proband and six of his relatives. The SCN5A VUS in this clinical context and segregation with the disease in his family supports its reclassification to pathogenic.

Subcutaneous versus Transvenous Implantable Cardioverter Defibrillator in Patients with End-Stage Renal Disease Requiring Dialysis: Extended Long-Term Retrospective Multicenter Follow-Up.

BACKGROUND: Implantable cardioverter defibrillators (ICD) prevent sudden cardiac death (SCD). Patients with end-stage renal disease (ESRD) requiring dialysis are at a very high risk of infection from cardiac implantable electronic device (CIED) implantation as well as mortality. In the present study, we compared the long-term complications and outcomes between subcutaneous ICD (S-ICD) and transvenous ICD (TV-ICD) recipients. METHODS: In this retrospective analysis, we analyzed a total of 43 patients with ESRD requiring dialysis who received either a prophylactic S-ICD (26 patients) or a single right ventricular lead TV-ICD (17 patients) at seven experienced centers in Germany. Follow-up was performed bi-annually, at the end of which the data concerning comorbidities and, if applicable, reason for death were checked and confirmed with patients' general practitioner, nephrologist and cardiologist. RESULTS: The median follow up duration was 95.6 months (range 42.8-126.3 months). Baseline characteristics were without noteworthy significant differences between groups. During follow-up (FU), there were significantly more device-associated infections (HR 8.72, 95% confidence interval (CI), 1.18 to 12.85, p < 0.05) and device-associated hospitalizations (HR 10.20, 95% CI 1.22 to 84.61, p < 0.001), as well as a higher cardiovascular mortality (HR 9.17, 95% CI 1.12 to 8.33, p < 0.05), in the TV-ICD group. The number of patients requiring hospitalization for any reason was significantly higher in the TV-ICD group (HR 2.59, 95% CI 1.12 to 6.41, p < 0.05). There was no significant difference in overall mortality (HR 1.92, 95% CI 0.96 to 6.15, p = 0.274). CONCLUSIONS: Our data suggest that, in this extended follow-up in seriously compromised renal patients on dialysis, the S-ICD patients have statistically fewer device infections and hospitalizations as well as lower cardiac mortality compared with the TV-ICD cohort.

A Unique Case of Goodpasture's Syndrome-Induced Cardiorenal Syndrome.

Goodpasture's syndrome (GPS) is a rare small vessel vasculitis characterized by circulating antibodies directed against the glomerular and alveolar basement membrane leading to renal and pulmonary manifestations. Here, we discuss a unique case of a 30-year-old Caucasian male smoker initially presenting with hemoptysis and anemia who was found to have biopsy-proven GPS with elevated anti-glomerular basement membrane (anti-GBM) antibodies. Unfortunately, the patient failed four months of standard treatment for GPS leading to end-stage renal disease (ESRD), while uniquely developing cardiorenal syndrome (CRS) with non-ischemic cardiomyopathy resulting in systolic and diastolic heart failure (HF). Despite aggressive medical management and hemodialysis, the patient's cardiac function continued to decline and the decision was made to insert an automatic implantable cardioverter defibrillator (AICD). To our knowledge, this is the first reported case of an anti-GBM-positive GPS patient who developed dilated cardiomyopathy. The importance of this report is to illustrate the rarity of developing CRS with non-ischemic cardiomyopathy and congestive heart failure from GPS and highlight the difficulty of determining management changes beyond guideline-directed medical therapy (GDMT) in GPS to slow the progression of worsening cardiac function.

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.

The point on the treatment of arrhythmic storm.

Arrhythmic storm is a clinical emergency associated with high mortality, which requires multi-disciplinary management. Reprogramming of the implantable cardiac defibrillator (ICD) aimed at reducing shocks, adrenergic blockade using beta-blockers, sedation/anxiolysis, and blockade of the stellate ganglion represent the first simple and effective manoeuvres, but further suppression of arrhythmias with antiarrhythmics is often required. A low-risk patient (e.g. monomorphic ventricular tachycardia, functioning ICD, and haemodynamically stable) should be managed with a beta-blocker (possibly non-selective) plus amiodarone, in addition to sedation with a benzodiazepine or dexmedetomidine; in patients at greater risk (high burden and haemodynamic instability), autonomic modulation with blockade of the stellate ganglion and the addition of a second antiarrhythmic (lidocaine) should be considered. In patients refractory to these measures, with advanced heart failure, general anaesthesia with intubation and the establishment of a haemodynamic circulatory support should be considered. Ablation, performed early, appears to be superior in terms of mortality and reduction of future shocks compared with titration of antiarrhythmics.

Open Chest Approach Lead Extraction in a Patient with a Large Vegetation: The Importance of Multidisciplinary Approach, Advanced Imaging, and Procedural Planning.

We present a complex case of cardiac implantable electronic device infection and extraction in the setting of bacteremia, large lead vegetation, and patent foramen ovale. Following a comprehensive preprocedural workup including transesophageal echocardiogram and computed tomography lead extraction protocol, in addition to the involvement of multiple subspecialties, an open chest approach to extraction was deemed a safer option for eradication of the patient's infection. Despite percutaneous techniques having evolved as the preferred extraction method during the last few decades, this case demonstrates the importance of a thorough evaluation at an experienced center to determine the need for open chest extraction.

Cardiac Magnetic Resonance and Cardiac Implantable Electronic Devices: Are They Truly Still "Enemies"?

The application of cardiac magnetic resonance (CMR) imaging in clinical practice has grown due to technological advancements and expanded clinical indications, highlighting its superior capabilities when compared to echocardiography for the assessment of myocardial tissue. Similarly, the utilization of implantable cardiac electronic devices (CIEDs) has significantly increased in cardiac arrhythmia management, and the requirements of CMR examinations in patients with CIEDs has become more common. However, this type of exam often presents challenges due to safety concerns and image artifacts. Until a few years ago, the presence of CIED was considered an absolute contraindication to CMR. To address these challenges, various technical improvements in CIED technology, like the reduction of the ferromagnetic components, and in CMR examinations, such as the introduction of new sequences, have been developed. Moreover, a rigorous protocol involving multidisciplinary collaboration is recommended for safe CMR examinations in patients with CIEDs, emphasizing risk assessment, careful monitoring during CMR, and post-scan device evaluation. Alternative methods to CMR, such as computed tomography coronary angiography with tissue characterization techniques like dual-energy and photon-counting, offer alternative potential solutions, although their diagnostic accuracy and availability do limit their use. Despite technological advancements, close collaboration and specialized staff training remain crucial for obtaining safe diagnostic CMR images in patients with CIEDs, thus justifying the presence of specialized centers that are equipped to handle these type of exams.

Comparison of infection and complication rates associated with transvenous vs. subcutaneous defibrillators in patients with stage 4 chronic kidney disease: a multicenter long-term retrospective follow-up.

Background: Patients with progressive chronic kidney disease (CKD) are at higher risk of infections and complications from cardiac implantable electronic devices (CIED). In patients with a primary or secondary prophylactic indication, implantable cardiac defibrillators (ICD) can prevent sudden cardiac deaths (SCD). We retrospectively compared transvenous-ICD (TV-ICD) and intermuscularly implanted subcutaneous-ICD (S-ICD) associated infections and complication rates together with hospitalizations in recipients with stage 4 kidney disease. Methods: We retrospectively analyzed 70 patients from six German centers with stage 4 CKD who received either a prophylactic TV-ICD with a single right ventricular lead, 49 patients, or a S-ICD, 21 patients. Follow-Ups (FU) were performed bi-annually. Results: The TV-ICD patients were significantly older. This group had more patients with a history of atrial arrhythmias and more were prescribed anti-arrhythmic medication compared with the S-ICD group. There were no significant differences for other baseline characteristics. The median and interquartile range of FU durations were 55.2 (57.6-69.3) months. During FU, patients with a TV-ICD system experienced significantly more device associated infections (n = 8, 16.3% vs. n = 0; p < 0.05), device-associated complications (n = 13, 26.5% vs. n = 1, 4.8%; p < 0.05) and device associated hospitalizations (n = 10, 20.4% vs. n = 1, 4.8%; p < 0.05). Conclusion: In this long-term FU of patients with stage 4 CKD and an indication for a prophylactic ICD, the S-ICD was associated with significantly fewer device associated infections, complications and hospitalizations compared with TV-ICDs.

Apical Hypertrophic Cardiomyopathy Presenting With Syncope in a Hispanic Woman: A Report of a Rare Case.

Yamaguchi syndrome or apical hypertrophic cardiomyopathy is a rare subtype of non-obstructive hypertrophic cardiomyopathy that is defined as the focused hypertrophy of the left ventricular apex. It is typically seen in Asian populations. Herein, we present a rare case of Yamaguchi syndrome seen in a Hispanic female.

Impact of a pharmacist-managed remote heart failure program in patients with a multisensor-capable implanted device.

PURPOSE: Added layers of remote management in heart failure (HF) have become available for patients with an implantable cardioverter defibrillator (ICD). The aim of this study was to investigate the impact of pharmacist-managed HF monitoring in patients with a multisensor-capable ICD. METHODS: This was a retrospective, data-only, single-arm study that compared primary outcome events individually in the preactivation and postactivation periods at a single center. The primary outcomes were the total number of all-cause and HF-related hospitalizations and all-cause emergency department (ED) visits and the median length of stay for all-cause and HF-related hospitalizations. The secondary outcome quantified medication utilization. RESULTS: In total, 132 patients completed the 1-year follow-up period. Overall, there was a 49% reduction in the number of patients with an all-cause hospitalization, a 77% reduction in the number of patients with an HF-related hospitalization, and a 36% reduction in the number of patients with an ED visit. More patients were hospitalized, visited the ED (P < 0.005), and had a longer median length of stay for all-cause hospitalizations in the preactivation period (P < 0.05). Overall medication utilization increased in the postactivation period. CONCLUSION: A pharmacist-led remote monitoring program, utilizing a multisensor diagnostic, was effective at significantly reducing hospitalizations, ED visits, and length of stay.

Impact of the COVID-19 pandemic on implantation of cardiac implantable electronic devices and remote monitoring activations.

INTRODUCTION AND OBJECTIVES: Remote monitoring (RM) of cardiac implantable electronic devices (CIEDs) is considered more reliable, efficient, and safer than conventional in-person follow-up. However, the implementation of RM is still suboptimal. This study aimed to analyze the impact of the COVID-19 pandemic on the rates of CIED implants and RM activations in Spain. METHODS: The COVID-19 RM Spain Registry was used to analyze the monthly number of all CIED implantations and RM activations from January 2018 to December 2021. A descriptive analysis was performed using aggregated data from the five major CIED manufacturers. RESULTS: A total of 205 345 CIEDs were recorded. The number of implants decreased sharply (48.2%) during the pandemic lockdown (March-June 2020) but gradually increased thereafter, compensating for the previous reduction. However, pacemakers and implantable cardiac defibrillators (ICD) showed an aggregate loss of 7% and 3%, respectively, from the annual average during 2020-2021. In contrast, cardiac resynchronization therapy defibrillators (CRT-D) increased by 17%, and pacemakers (CRT-P) by 4.5% over the 2-year period. The percentage of RM activations increased from 24.5% in 2018 to 49.0% in 2021, with a sharp increase during the lockdown. The RM activation rates consistently increased during the lockdown for all devices: pacemakers (14.4% vs 37.2%; P <.001); ICD (75.6% vs 94.2%; P <.001); CRT-D/CRT-P (68.6-44.2% vs 81.6-61%; P <.001), and implantable loop recorders (50.2% vs 68.7%; P <.001). CONCLUSIONS: The significant decline in implants during the lockdown gradually recovered, except for pacemakers and ICD. However, the COVID-19 pandemic boosted RM for all CIEDs in Spain.

Safety and Efficacy of an Implantable Cardioverter Defibrillator (ICD) in the Detection and Prevention of Cardiac Arrhythmia - A Systematic Review.

Implantable cardioverter defibrillators (ICD) have been recommended as an effective therapy in treating sudden cardiac deaths. This study evaluates the safety and efficacies of ICDs in detecting arrhythmias. Different ICDs, such as the transvenous cardioverter defibrillator (TV-ICD) and the subcutaneous implantable cardioverter defibrillator (S-ICD), are used. This systematic review identified Embase, PubMed, Medical Literature Analysis and Retrieval System Online (MEDLINE), and Web of Science as the primary electronic databases for research. Supplementation of the available articles for the review was done using Google Scholar. The population, exposure, control, outcome, and studies (PECOS) criteria were used in this study. The quality of the included studies was assessed using the Critical Appraisal Skills Program (CASP) standard checklist. Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used in this systematic review. Two researchers conducted the extraction of data. A pre-designed Excel worksheet (Microsoft, Redmond, Washington) was used in the recording of extracted data. Eight studies were identified for use in this systematic review. Safety of the ICDs was observed with the minimum number of reported inappropriate shocks. Studies conducted identified that women had a lower number of incidences when a long detection setting by sex was conducted. Strategic programming of ICDs was noted as effective in lowering the levels of mortality. Studies claimed that the reduction of inappropriate shocks were important in the reduction of myocardial damage, which resulted in the mortality rate among the patients decreasing. Having high cutoff rates and long intervals for detection in ICD programming was noted to help in reducing ICD therapy intervention among patients. Differences among the male and female populations were inconsequential in the efficacy and safety of ICDs. Their effectiveness in sensitivity, pacing success, and defibrillation success were high and very significant. ICDs were safe in their use in the detection of arrhythmias.

Cardiac Sarcoidosis: A Literature Review of Current Recommendations on Diagnosis and Management.

Cardiac sarcoidosis (CS) is a rare multisystem disorder characterized by granulomatous infiltration of the myocardium, which can lead to significant morbidity and mortality. Its clinical manifestations range from asymptomatic conduction abnormalities to severe heart failure (HF) and sudden cardiac death. This comprehensive review aims to provide an overview of the diagnosis, clinical features, and current medical management strategies for CS. Additionally, the role of implantable cardioverter-defibrillators (ICDs) and the potential use of positron emission tomography in guiding management decisions are explored. A comprehensive understanding of the medical management of CS is essential for improving patient outcomes and guiding future research endeavors.

Admission triglyceride-glucose index is predictor of long-term mortality and appropriate implantable cardiac defibrillator therapy in patients with heart failure.

Background: In this study, the main aim was to evaluate the relation of the triglyceride-glucose (TyG) index to long-term mortality and proper shock therapy in patients with an implantable cardiac defibrillator (ICD) implanted for heart failure with reduced ejection fraction. Methods: This retrospective study group consisted of 773 patients treated with ICD for heart failure with reduced ejection fraction. The long-term prognostic effect of the TyG index among tertiles was evaluated regarding mortality and appropriate ICD therapy. Results: In the adjusted model, the mortality rates were 14.0% (hazard ratio: 2.24; 95% CI: 1.42-6.88) in tertile 2 and 23.3% (hazard ratio: 3.88; 95% CI: 1.84-14.38) in tertile 3. Conclusion: The TyG index was found to be an independent predictive marker for both long-term mortality and appropriate ICD therapy.

Heart Failure and Cardiac Device Therapy: A Review of Current National Institute of Health and Care Excellence and European Society of Cardiology Guidelines.

Guidelines help clinicians to deliver high-quality care with therapies based on up-to-date evidence. There has been significant progress in the management of heart failure with regards to both medication and cardiac device therapy. These advances have been incorporated into national and international guidelines with varying degrees of success. This article reviews current guidance from the National Institute of Health and Care Excellence in the UK and compares this with European Society of Cardiology guidelines, and evaluates how differences between them may impact on clinical practice.

Iatrogenic Hypoxemia and Atrial Septal Defect Due to Electrical Storm Ablation After Left Ventricular Assist Device: A Case Report.

A 59-year-old male with an implantable cardiac defibrillator, left ventricular assist device, and refractory ventricular tachycardia presented with hypoxemia due to a post-ablation iatrogenic atrial septal defect. Left ventricular assist devices generate pressure gradients that may exacerbate intracardiac shunts and can precipitate significant hypoxemia.

Effect of Home-Based Cardiac Rehabilitation Program on Self-Efficacy of Patients With Implantable Cardioverter Defibrillator.

Introduction: For more effective control and treatment of cardiac dysrhythmias caused by diseases, ischemia, or other causes, an implantable cardioverter defibrillator (ICD) is used. One of the effective ways for secondary prevention is the home-based cardiac rehabilitation (HBCR) which nurses have an effective role in its implementation. Objective: The study aimed to investigate the effect of implementing HBCR programs on the self-efficacy of patients with ICD. Methods: This is a semi-experimental study conducted on 70 patients who received ICD in Shahid Chamran Heart Center of Isfahan University of Medical Sciences (IUMS) in 2021. The patients were randomly assigned to intervention and control groups and were introduced to the practical concepts of HBCR during four training sessions. In the following, 3-month follow-up and trainings were continued by home visits, telephone follow-up, and use of social messaging networks due to the conditions of coronavirus disease 2019 (COVID-19) pandemic. The data were analyzed with SPSS/21. Results: The findings showed that performing HBCR programs was effective in improving the self-efficacy of patients with ICDs. A significant trend in the implementation of the HBCR programs in two groups was shown using chi-square test and independent t-test and variance with repeated measurements (p < .001). There was no significant difference in self-efficacy score in both groups at the beginning of the study (p < .056). Conclusion: Considering the effectiveness of HBCR programs on improving the self-efficacy of patients with ICDs, it can be used in the educational care programs of healthcare workers and in the strategic policies of health care services.

A Case of 25 Inappropriate Automatic Implantable Cardioverter Defibrillator Shocks and 22 Episodes of Antitachycardia Pacing.

An implantable cardioverter defibrillator (ICD) can save lives from fatal tachyarrhythmias. In rare cases, these devices can fail or malfunction. We present a case of a patient that suffered from 25 inappropriate shocks and 22 episodes of antitachycardia pacing (ATP), secondary to a probable non-traumatic dual lead fracture. One episode of ATP induced an R-on-T phenomenon, causing monomorphic ventricular tachycardia in the patient. The inappropriately functioning ICD also required two magnets to be placed on the patient's chest in the emergency department to convert the device to an asynchronous mode. An unexpected case of this magnitude and in such a brief timeframe has not been reported in prior ICD studies.