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Aims: Subcutaneous implantable cardioverter-defibrillators (S-ICDs) are effective in patients who require protection from sudden cardiac death while avoiding the long-term risks associated with transvenous leads. However, data on their real-world performance in paediatric and young patients remain limited. This study aims to evaluate the safety and efficacy of S-ICDs in a large, multicentre cohort of paediatric and young patients across Europe, with a focus on contemporary implantation practices and clinical outcomes.

Methods and results: This is an international, multicentre, observational, retrospective registry on S-ICD outcome in paediatric and young adult patients with congenital heart defects (CHD), cardiomyopathies, channelopathies, and idiopathic ventricular fibrillation (IVF). Data were collected on implantation techniques, acute and long-term outcomes, including defibrillation efficacy, inappropriate (IAS) and appropriate shocks, and complications. Follow-up data were analysed to assess device performance and safety. A total of 223 patients (mean age: 15 ± 3 years; 59% male, 53% cardiomyopathies, 18% channelopathies, 15% IVF, 14% CHD) were included. Most patients underwent implantation using an intermuscular (65%) and two-incision technique (88%). Acute defibrillation success was 100%, and no intraoperative complications occurred. The median follow-up was 28 (IQR: 12-55) months. Appropriate shocks were delivered in 41 (18%) patients (26% rate at 5 years). The first shock was effective in 92% of discrete episodes. The IAS and the complication rates were 20% and 5% at 5 years. Older age, intermuscular pocket, and two-incision technique were associated with fewer complications.

Conclusion: This multicentre S-ICD European registry in paediatric and young patients demonstrated favourable outcomes, low IAS, and complication rates.

In most patients with atrial fibrillation (AF), effective stroke prevention necessitates long-term (often lifelong) oral anticoagulant therapy (OAC). However, the effectiveness of OAC therapy in a clinical setting (i.e. outside the controlled environment of randomized clinical trials) is strongly influenced by patients' adherence and persistence with prescribed therapy. However, suboptimal adherence to OAC remains a substantial problem in routine practice-available evidence suggests that patients do not take their OAC one out of every four days, and approximately one in three to four patients is poorly adherent to OAC. In addition, around 15% of high-risk OAC-eligible patients with AF refuse to take OAC for a variety of patient-specific reasons. Poor adherence to OAC therapy is associated with adverse clinical outcomes [such as stroke or systemic embolism, hospitalization, mortality, bleeding (particularly with vitamin K antagonist therapy)] and increased economic costs. In this overview, we summarize important aspects of the adherence to medication concept, including the definition and measurement of adherence, the determinants and prevalence of OAC non-adherence, the clinical importance of achieving and maintaining good adherence, strategies to improve adherence to OAC, and alternative treatment options for effective thromboprophylaxis in patients with AF who are non-adherent to OAC therapy.

Aims: Evaluate the prognostic significance of arrhythmias and conduction disorders on ambulatory ECG in recently diagnosed genetic vs. non-genetic dilated cardiomyopathy (DCM).

Objective: To compare the prevalence of abnormalities on ambulatory ECG monitoring between genetic and non-genetic DCM patients and evaluate the predictive value for malignant ventricular adverse events (MVAEs).

Methods and results: Clinical and ambulatory ECG data were collected from 354 genotyped DCM probands, with a median follow-up of 8 years (IQR: 5-9 years). The malignant ventricular adverse event was defined as ventricular fibrillation, sustained ventricular tachycardia, anti-tachy pacing, appropriate device therapy, or sudden cardiac death. C-statistics assessed the predictive performance of the regression models. In total, 123 (35%) patients carried a (likely) pathogenic variant. Abnormalities on ambulatory ECG were more frequent in genetic DCM patients (80%) compared to non-genetic DCM (67%; P = 0.013). Permanent atrial fibrillation (perAF), paroxysmal supraventricular tachycardia (parox-SVT), and non-sustained ventricular tachycardia (NSVT) were more frequent in genetic DCM patients (P = 0.041, <0.001, and <0.001). Structural cardiac parameters showed minimal group differences. Using Cox proportional hazard analyses to predict MVAE, ambulatory ECG variables (perAF, AV-block, NSVT, >500 premature ventricular complexes (PVCs)/24 h) had an area under the curve (AUC) of 0.768 in genetic and 0.628 in non-genetic DCM patients (P = 0.044). The premature ventricular complex burden was only predictive for MVAE in genetic DCM. Adding clinical variables provided little incremental predictive value for genetic vs. non-genetic DCM (AUC Δ+0.004 vs. Δ+0.150, respectively).

Conclusion: Ambulatory ECG monitoring abnormalities are prevalent in genetic DCM patients. In contrast to non-genetic DCM patients, ambulatory ECG parameters have an important predictive value to determine the risk of MVAE in genetic DCM patients.

Aims: Repeat ablation strategies for persistent atrial fibrillation (PerAF) are less well studied than initial ablation strategies. The efficacy of repeat ablation remains unclear, particularly regarding the potential advantages of extra-pulmonary vein (PV) extensive ablation compared with in situ ablation.

Methods and results: Patients with recurrent PerAF were randomized (1:1) to receive extra-PV extensive ablation (EXT group, n = 66) or repeat PV isolation (PVI) and linear ablation as the first procedure (in situ group, n = 66). The primary endpoint was freedom from atrial fibrillation (AF)/atrial tachycardia (AT) episodes lasting >30 s at 12 months. At 12 months, 44 patients (66.7%) in the EXT group were free from AF/AT recurrence, in contrast to 32 patients (48.5%) in the in situ group [log-rank P = 0.037; hazard ratio (HR) 0.587 (95% confidence interval (CI), 0.348-0.992)]. The freedom from AF recurrence rate was significantly higher in the EXT group than in the in situ group [77.3% vs. 60.6%, log-rank P = 0.027; HR 0.509 (95% CI, 0.278-0.932)].The safety endpoints showed no significant difference between the two groups (4.5% vs. 6.1%, P = 0.716).

Conclusion: Among patients with PerAF undergoing repeat ablation, the EXT group demonstrated superior clinical efficacy compared with the in situ group, indicating that PV reconnection and linear lesion reconduction may not constitute the predominant mechanisms driving AF recurrence. These may still contribute significantly, but targeting additional non-PV substrates further improves outcomes.

Calmodulinopathies are very rare genetic disorders associated with a high risk for sudden cardiac death. Disease-causing variants in 1 of the 3 identical CALM genes cause severe forms of long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, or idiopathic ventricular fibrillation, and there are many unanswered questions concerning management and underlying mechanisms. What is currently known depends largely on the initial publications from the ICamR (International Calmodulinopathy Registry). However, progress is delayed because the accrual of patients in ICamR is slow. As we did long ago for long QT syndrome, this is a call for action, requesting doctors all over the world to enroll even their isolated cases in the Registry. This is the only way to obtain, for an adequate number of patients, the data necessary to define the spectrum of clinical manifestations and the genotype-phenotype correlation essential for an improved risk stratification and best therapeutic management. If you are willing to contribute, please contact us.

Aims: Traditional cardiac pacing and defibrillation devices rely on leads connected to a subcutaneous pulse generator, which can result in complications such as vascular damage, infection, or lead failure. Advances in technology have led to the development of leadless pacemakers, which combine the battery, circuitry, and electrodes into a single self-contained unit, and extravascular implantable cardioverter-defibrillators (ICDs), which position electrodes outside the vasculature. These innovations offer promising alternatives for patients requiring both defibrillation and pacing, particularly those unable or unwilling to accommodate traditional leads. However, the interactions between extravascular ICDs and leadless pacemakers remain largely unexplored and currently lack regulatory approval for combined use. This study evaluates the interactions between leadless pacemakers and extravascular ICDs to assess their simultaneous operation.

Methods and results: In-silico simulations, saline-tank experiments, and Monte Carlo simulations were conducted to evaluate device interactions, focusing on ventricular fibrillation (VF) detection during pacing conditions. Ventricular fibrillation detection was unaffected by pacing pulse widths ≤0.24 ms, with a pacing pulse-to-VF amplitude ratio of ≤2 considered safe. Wider pulse widths or higher outputs progressively increased the risk of VF undersensing. Experiments confirmed that pacing pulses ≤3 V and ≤0.24 ms minimally impacted VF detection. Proximity of device affected pacing pulse amplitude sensed by the ICD, but pacemaker orientation did not. Monte Carlo simulations indicated a 0-4% probability of undesired interactions under clinically relevant conditions.

Conclusion: Extravascular ICDs and leadless pacemakers may safely coexist, with a low observed risk of VF undersensing in our study. Further clinical studies are needed to confirm these findings.