Search Results (427)

Background and Clinical Significance: Patients with a left ventricular assist device (LVAD) are at risk of ventricular arrhythmias, which are generally hemodynamically tolerated if they occur. In such cases, patients may experience painful implantable cardioverter-defibrillator (ICD) shocks. Case Presentation: A 71-year-old patient with a history of dilated cardiomyopathy caused by a phospholamban (PLN) gain-of-function mutation, with a primary prevention ICD and an LVAD, presented with multiple ICD shocks which she experienced as painful and traumatic. She was found to have ongoing ventricular fibrillation with apparent hemodynamic stability. Conversion to sinus rhythm was achieved through intravenous administration of antiarrhythmic drugs followed by external defibrillation using stacked shocks. Due to the traumatic nature of the shocks, the shock function of the ICD was turned off. Nearly two months later, the patient presented for a second time and was again found to have ventricular fibrillation which had been present for at least six weeks. Conversion to sinus rhythm was unsuccessful and the patient was discharged to her home with an advanced care plan and her LVAD was deactivated. The patient died two months later. Conclusions: Patients with an LVAD can remain hemodynamically stable for prolonged periods of time during ventricular arrhythmias. ICD shocks are therefore mostly experienced as painful and even traumatic. Therefore, the routine use of ICD shock therapy in patients with an LVAD should be reconsidered. Adjustment of ICD programming to higher rates and longer detection may be warranted. Further investigation is warranted regarding a switch to devices with an alarm function rather than therapies for tachyarrhythmias. Full article

Background: The aim of this study is to determine the safety of a locally implemented Standard of Practice (SOP) in patients with cardiac implantable electronic devices (CIEDs). With increasing use of radiotherapy in cancer treatment and the widespread adaptation of CIEDs, the British Heart Rhythm Society introduced new guidance in 2025. There remains ambiguity between various international, as well as manufacturer, guidelines on the management of these patients. Methods: This was a retrospective single-centre observational study analysing patients with CIEDs receiving radiotherapy after the implementation of our Standard of Practice in 2021. Patients were identified using the Cardiobase system. Patients were divided into non-pacemaker-dependent, pacemaker-dependent and implantable-cardioverter–defibrillator (ICD) groups. Lead sensing and impedance values were gathered pre- and post-treatment and analysed using a paired Student’s T-test. Results: A total of 320 patients were included in this study. There were no statistically significant changes in lead sensing capabilities in any of the groups pre- and post-radiotherapy with a p value of >0.05. There were no statistically significant changes in lead impedance in the ICD and non-pacemaker-dependent groups. Although statistically significant (p = 0.039), there was no clinically significant reduction in atrial lead impedance in the pacemaker-dependent cohort. Conclusions: From the obtained results, we can conclude that our locally implemented SOP is a safe alternative to BHRS guidelines. Full article

Remote monitoring of pacemakers and defibrillators increases patient safety but also increases clinical workload. Review of atrial high-rate episodes is particularly demanding as episodes can contain atrial tachycardia or atrial fibrillation (AT/AF), noise, or far-field oversensing (FFO). Automatic review of atrial high-rate episodes by an Artificial Intelligence (AI) model can decrease the workload of remote monitoring, provided it maintains high sensitivity for true atrial tachycardia. A residual network is trained using a center-level fourfold cross validation. The four resulting models achieved a precision of 97.2–99.4% for AT/AF, 93.1–97.7% for noise, and 75.4–94.4% for FFO, while maintaining high sensitivity 98.9–99.3% for AT/AF. The four models were combined through averaging prediction probabilities to create an ensemble model. Thresholding ensemble predictions with probability > 95% resulted in a robust ensemble model that made only two errors (<0.1%) after reviewing 3925 episodes (91.9%) of the total 4271 episodes. This shows how AI models can reliably assist in remote monitoring. Future research should be aimed at classification models for other episode types and clinical validation of AI models to assist remote monitoring of pacemakers and defibrillators. Full article

Implantable Cardioverter-Defibrillators (ICDs) are life-sustaining devices used in the prevention of sudden death in patients suffering from advanced cardiac diseases. Although improvements in ICD technology and monitoring capabilities have been made, existing techniques are still not effective in predicting accelerated battery drain, thereby causing unplanned generator replacement and clinically significant device-related events. In this study, machine learning techniques were employed in the assessment of the early detection of ICD battery depletion risk using the collected device interrogation reports. The dataset used consisted of 32 devices in the training set and nine in the testing set. In order to mitigate the problem of a small sample size, a GMM-based data augmentation technique was applied solely to the training data, and actual devices were used for the testing data. Five supervised models, including Logistic Regression, Random Forest, SVM, CatBoost, and a Neural Network (MLP), have been utilized using a repeated stratified cross-validation and a separate held-out test data set. All the models have been tested for their performance using classification metrics. All models demonstrated variable performance with wide confidence intervals due to limited sample size. Support vector machines showed the highest cross-validation discrimination 0.889 ± 0.203, though uncertainty remains substantial given the small datasets (n = 41). From the feature analysis, it was found that atrial sensing amplitude, RV/LV capture threshold, output settings, and implant duration were the most important features for the prediction of high battery depletion risk. These findings suggest that changes in device parameters and implant age are associated with elevated battery depletion risk, supporting the feasibility of telemetry-driven risk stratification for device management. Full article

Takotsubo cardiomyopathy (TCM) is characterized by contractile impairment of the left ventricular apex and excessive contraction of the base of the heart, resulting in transient cardiac dysfunction. Here, we report a case of an implantable cardioverter defibrillator (ICD) that was activated for ventricular fibrillation (VF) caused by TCM one day after removal of maxillomandibular exostoses. The patient was a 53-year-old female who underwent removal of maxillomandibular exostoses in the mid-palate area, bilateral molars of the maxilla, and bilateral mandibular premolars under general anesthesia. Because the patient had a history of VF, an ICD was implanted. Removal was performed without any problems, but VF occurred on the following day, and the ICD was frequently required. Ultrasound examination suggested contractile impairment of the ventricular apex and excessive contraction of the base. Examinations led to a diagnosis of TCM using the Mayo Clinic diagnostic criteria. VF was resolved by administration of amiodarone hydrochloride. The wound in the oral cavity healed favorably, and the patient was discharged from hospital on day 33 without further occurrence of VF. This case highlights important perioperative risk assessment and anesthesia considerations for oral and maxillofacial surgeons managing medically compromised patients undergoing oral surgery. Full article

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

Cardiac sarcoidosis (CS) is a critical and frequently underdiagnosed phenotype of sarcoidosis, characterized by non-caseating granulomatous infiltration of the myocardium. This review synthesizes current knowledge regarding the pathogenesis, diagnosis, and management of CS. The disease manifests with a heterogeneous clinical spectrum ranging from asymptomatic conduction abnormalities to life-threatening ventricular arrhythmias and heart failure. Diagnosis remains challenging due to the patchy distribution of granulomas, which limits the sensitivity of endomyocardial biopsy. Consequently, a multimodal diagnostic approach is essential, integrating advanced imaging modalities such as cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) and ¹⁸F-fluorodeoxyglucose positron emission tomography (FDG-PET). These tools not only facilitate detection but also enable the differentiation of active inflammation from chronic fibrosis. Histopathological assessment, supported by specific immunophenotyping and electron microscopy, remains the gold standard for confirming diagnosis and excluding mimics like giant cell myocarditis or infectious granulomatous diseases. Management requires a multidisciplinary strategy combining immunosuppressive therapy, primarily corticosteroids and steroid-sparing agents, with guideline-directed cardiac care, including implantable cardioverter-defibrillators for arrhythmia risk stratification. Emerging biomarkers and artificial intelligence-driven imaging analysis promise to further refine risk stratification and therapeutic monitoring, advancing precision medicine in this complex disorder. Full article

Background: Ultrasound (US)-guided axillary vein puncture (AVP) is an established technique for cardiac implantable electronic device (CIED) implantation. Yet real-world data concerning shifting from conventional venous access into US-guided AVP are not widely available. Methods: This is a single-center prospective registry reporting safety (complications) and efficacy (success rate: i.e., accomplishment of the vein access utilizing only the initially employed approach) of self-taught US-guided AVP integration into the standard workflow of CIED procedures. Results: A total of 539 patients (mean age 71.5 ± 12.4 years old, 78.7% males) were treated in our institution over a three-year period. Regarding CIED type and lead number, 58.3% used an implantable cardioverter defibrillator, 32% used permanent pacemakers, and two leads were involved in 65.8% of the cases and three leads in 8.9%. Before integration of US-guided AVP, the venous access success rate was 93.5%. The US-guided AVP success rate was 377/400 procedures (94.2%). After the first semester of US-guided AVP utilization, a pattern of increased success rate was observed (p = 0.002) and remained stable over the following semesters. No major complication (periprocedural or 30-day mortality, hemothorax, pneumothorax and tamponade) occurred after US AVP integration in our workflow. Conclusions: The integration of US-guided AVP in a self-taught manner is feasible among electrophysiologists with experience in US-guided vascular access. A high success rate can be reached quickly and safely. Full article

Dilated cardiomyopathy (DCM) in children is rare, but carries a high risk of progression to advanced heart failure (HF) and heart transplant (HTx). Improved short-term risk stratification is essential; however, robust pediatric prognostic tools remain limited. We aimed to evaluate the 1-year prognostic value of multichamber speckle-tracking echocardiography (STE) and biomarkers, including age-adjusted N-terminal pro-B-type natriuretic peptide (NT-proBNP) and vitamin D, in children with DCM. In this single-centre prospective cohort study, 29 children with idiopathic DCM and 27 age- and sex-matched healthy controls underwent standardised clinical, laboratory, and echocardiographic assessment. The primary endpoint was a 12-month composite of implantation of an implantable cardioverter-defibrillator (ICD), left-ventricular assist device (LVAD), HTx, or all-cause mortality. During a 1-year follow-up, 9/29 (31%) DCM patients experienced major events. Compared with event-free patients and controls, children with events had more impaired LVGLS (−5.99 ± 2.45% vs. −13.44 ± 6.88% and −19.98 ± 3.25%), lower LASr (10.97 ± 7.67% vs. 25.36 ± 10.28% and 44.0 ± 11.43%), and reduced RVFWSL (−15.32 ± 5.24% vs. −23.13 ± 8.55% and −24.78 ± 4.45%; all p < 0.01). Zlog NT-proBNP was markedly higher in the event group (5.37 [5.00–6.08] vs. 2.28 [0.71–3.68] and 0.14 [−0.02–0.88]). LVGLS, Zlog NT-proBNP, and LASr showed excellent discrimination for 1-year events (AUC 0.91, 0.91, and 0.87, respectively), with clinically applicable cut-offs (LVGLS ≥ −8%, Zlog NT-proBNP ≥ 4.6, LASr ≤ 21%). In conclusion, multichamber strain imaging combined with age-adjusted NT-proBNP provides clinically relevant, exploratory markers for short-term risk stratification in pediatric DCM, supporting earlier intensification of follow-up and timely referral for advanced heart failure therapies. These findings warrant validation in larger multicenter cohorts. Full article

Background: Cardiac sarcoidosis (CS) is associated with potentially serious complications, including heart failure and life-threatening arrhythmias. The diagnosis and management of CS is multifaceted, requiring a multi-disciplinary team (MDT)-based approach. A new regional CS clinical service was established in Sussex County (UK) in January 2025. This service is based on a core of cardiologists working with a wider MDT, including specialists in pulmonary sarcoidosis, nuclear medicine and cardiac electrophysiology. This study assessed the clinical performance of this new service. Methods: Patients with suspected CS referred to the Sussex CS Service between January and December 2025 were included, as compared to a control cohort of patients referred for CS assessment before the service was conceived. Results: Of the 51 CS service referrals, 13 patients fulfilled the Heart Rhythm Society (HRS) criteria, all of whom were correctly diagnosed with CS, whilst only two out of seven HRS-positive control patients were correctly diagnosed. In the 38 HRS-negative CS service referrals, 8 patients (21%) were still given a clinical CS diagnosis compared to none in the HRS-negative controls. Of the 21 patients diagnosed with CS, 7 (33%) had active myocardial inflammation and 8 (38%) had LV systolic dysfunction. Where indicated, immunosuppressive and heart failure therapies were initiated in all patients. Eight CS patients (38%) underwent implantable cardioverter defibrillator implantation. No deaths or heart failure hospitalisations occurred within the first 11 months. Conclusions: An MDT-based CS service model can provide multi-faceted care to patients, without major short-term adverse outcomes. The service model replicability and long-term outcomes require further assessment. Full article

Background: The use of implantable cardioverter defibrillators (ICDs) in elite athletes following sudden cardiac arrest (SCA) or the diagnosis of high-risk cardiac conditions presents a complex interplay of medical, psychological, and legal challenges. Despite evolving guidelines, data on clinical outcomes and return-to-sport (RTS) trajectories in elite athletes remain limited. Objective: To describe the clinical profiles, management strategies, and career outcomes of elite athletes who received ICDs. Methods: A retrospective multilingual media and literature search was performed up to January 2026 to identify elite athletes with ICDs. Inclusion criteria required evidence of professional or Olympic-level competition, confirmed ICD implantation, and sufficient clinical and career data. Cases were analyzed for demographics, underlying diagnosis, prevention type, post-ICD outcomes, and RTS status. Results: Thirty-seven elite athletes were identified (mean age 25.8 ± 4.3 years). The most common sport was football (n = 25). Hypertrophic cardiomyopathy, non-ischemic LV scar, and arrhythmogenic cardiomyopathy were the most frequent diagnoses, although 49% of etiologies remained unspecified. ICDs were implanted for secondary prevention in 70% of cases. Following ICD implantation, 25 athletes (68%) completed RTS, including 24 (65%) at the professional level. Among these, nine experienced shocks, and four ultimately discontinued competition. The sole fatality occurred in an athlete who had voluntarily explanted the ICD. Conclusions: A substantial proportion of elite athletes with ICDs successfully return to high-level sport, but clinical outcomes, risk tolerance, and legal frameworks remain variable. These findings support continued shifts towards personalized shared decision making and highlight the need for standardized, sport-specific RTS protocols, long-term registries, and psychosocial support in this population. Full article

Cardiac contractility modulation (CCM) is a bioelectronic therapy that delivers precisely timed electrical signals during ventricular refractoriness to modulate myocardial contractility without triggering depolarization. Unlike pacing-based therapies, CCM does not initiate a new depolarization but instead modulates intracellular signaling pathways to enhance myocardial contractility without increasing myocardial oxygen consumption. CCM therefore represents a myocardial conditioning strategy distinct from cardiac resynchronization therapy, conduction system pacing, or neuromodulation. Experimental and translational studies demonstrate that repeated CCM delivery induces sustained myocardial adaptations, including improvements in excitation–contraction coupling, molecular signaling pathways, and structural remodeling that extend beyond transient hemodynamic effects. Across clinical investigations, CCM has been associated with meaningful improvements in exercise tolerance, health-related quality of life, and functional status in carefully selected populations. Observational data further suggest a potential reduction in heart failure-related hospitalizations when therapy is applied within evidence-aligned indications. Recent technological developments—including simplified ventricular lead configurations, rechargeable compact generators, and integrated CCM–defibrillator platforms—have reduced procedural complexity and may broaden clinical applicability, particularly in patients with concomitant implantable cardioverter–defibrillator indications. This review synthesizes mechanistic insights, clinical evidence, patient selection principles, and practical considerations to define the evolving role of CCM within contemporary heart failure care pathways. Full article

Tricuspid regurgitation (TR) in patients with transvalvular cardiac implantable electronic device (CIED) leads is increasingly encountered as transcatheter tricuspid valve interventions (TTVI) expand, yet integrated guidance for managing this “two-device problem” remains limited. We performed a focused synthesis of contemporary evidence, organizing findings around mechanisms and diagnosis of TR in the setting of CIED leads, lead–device interactions across TTVI platforms, and clinical trade-offs of transvenous lead extraction (TLE) versus lead preservation or jailing. CIED-associated TR can arise from lead–leaflet impingement, leaflet injury, fibrotic adhesion, pacing-induced remodeling, or infection; true CIED-induced TR accounts for a minority of clinically significant TR, yet progression of TR after lead implantation occurs in 7–45% of patients, and moderate-to-severe TR in CIED populations is associated with 1.6- to 2.5-fold increased mortality risk. Lead conflict and lifetime consequences differ by TTVI modality: repair approaches are generally more lead-tolerant, whereas valve replacement creates obligate lead jailing with implications for lead performance, future extraction feasibility, and infection management. Management of TR with transvalvular CIED leads requires integrated Heart Team planning that anticipates downstream device needs. Standardized TR phenotyping, lead-aware TTVI selection, valve-sparing rhythm-device strategies, and structured post-procedural surveillance may improve outcomes; prospective studies are needed to define optimal extract-versus-jail pathways. Full article

Background: Sex-related differences in outcomes after implantable cardioverter-defibrillator (ICD) implantation remain incompletely understood. Although women receive ICDs less frequently, whether their long-term survival differs from that of men in real-world clinical practice is not well established. We aimed to evaluate sex-specific mortality and relative survival in a large consecutive cohort of ICD recipients from a tertiary hospital. Methods: We conducted a retrospective cohort study including all consecutive patients who underwent ICD implantation at a tertiary hospital between 2015 and 2025. Demographic features, device indication, and mortality were obtained through clinical records. Relative survival (observed vs. expected) was estimated using the Ederer II method with national life tables. A Cox proportional hazards model assessed the effect of sex on mortality. Results: A total of 1091 patients (82.1% men; mean age 63.1 ± 13.1 years) were included. During a mean follow-up of 4.33 ± 3.22 years, 230 patients died (21.1%). Women showed lower unadjusted all-cause mortality than men: 24 deaths (18.0%) vs. 206 (20.6%). Women had significantly higher left ventricular ejection fraction (41.5 ± 23.6% vs. 37.2 ± 18.1%, p = 0.0046), less ischemic cardiomyopathy, and lower prevalence of cardiovascular risk factors. Although univariable analysis suggested lower mortality in women (HR 0.58, 95% CI 0.38–0.90; p = 0.014), multivariable analysis indicated that sex was not an independent predictor of mortality (HR 0.81, 95% CI 0.53–1.26). Relative survival revealed a substantial long-term mortality burden in ICD carriers, especially in men: men: 4-year survival 82.3% (expected 93.2%); 8-year 66.7% (85.6%); 12-year 56.0% (76.8%); women: 4-year survival 89.1% (expected 96.7%); 8-year 77.1% (92.8%); 12-year 77.1% (89.2%). Conclusions: In this large real-world cohort of ICD recipients, women showed lower unadjusted mortality and a smaller excess mortality compared with the general population. However, sex was not an independent predictor of survival after multivariable adjustment. These findings may indicate that observed survival differences are largely explained by differences in clinical profile and comorbidity burden rather than by sex itself. Full article

Background: Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is a rare multisystem disorder caused by mitochondrial DNA mutations, most commonly the m.3243A>G variant in the MT-TL1 gene. Although neurological manifestations predominate, cardiac involvement, including hypertrophic cardiomyopathy (HCM), heart failure (HF), and arrhythmias, may be the initial or dominant presentation and often remains underrecognized. Case Presentation: We report a 43-year-old man with chronic kidney disease (CKD) and long-standing bilateral sensorineural hearing loss who presented with progressive dyspnea and acute decompensated HF. Transthoracic echocardiography revealed severe left ventricular (LV) systolic dysfunction with diffuse hypertrophy. Cardiac magnetic resonance showed non-ischemic cardiomyopathy with diffuse late gadolinium enhancement and increased LV wall thickness. Coronary angiography excluded obstructive disease. Initial endomyocardial biopsy performed at a referring center showed nonspecific hypertrophy and fibrosis without diagnostic features. Given the multisystem involvement, a metabolic or genetic etiology was suspected. Whole-exome sequencing identified the pathogenic m.3243A>G MT-TL1 mutation, confirming MELAS syndrome. The patient was managed with guideline-directed HF therapy, received an implantable cardioverter-defibrillator for primary prevention, and was subsequently evaluated for heart transplantation. Conclusions: This case highlights the importance of considering mitochondrial disorders in the differential diagnosis of unexplained cardiomyopathy, particularly when cardiac dysfunction coexists with renal impairment and auditory deficits. Comprehensive multimodality evaluation and genetic testing are essential to establishing a unifying diagnosis and optimizing management. Full article