暂无摘要。

UNASSIGNED: Long QT syndrome (LQTS) is an autosomal dominant inherited cardiac condition characterized by a QT interval prolongation and risk of sudden death. There are 17 subtypes of this syndrome associated with genetic variants in 11 genes. The second most common is type 2, caused by a mutation in the KCNH2 gene, which is part of the potassium channel and influences the final repolarization of the ventricular action potential. This case report presents an Ecuadorian teen with congenital Long QT Syndrome type 2 (OMIM ID: 613688), from a family without cardiac diseases or sudden cardiac death backgrounds.
UNASSIGNED: A 14-year-old girl with syncope, normal echocardiogram, and an irregular electrocardiogram was diagnosed with LQTS. Moreover, by performing Next-Generation Sequencing, a pathogenic variant in the KCNH2 gene p.(Ala614Val) (ClinVar ID: VCV000029777.14) associated with LQTS type 2, and two variants of uncertain significance in the AKAP9 p.(Arg1654GlyfsTer23) (rs779447911), and TTN p. (Arg34653Cys) (ClinVar ID: VCV001475968.4) genes were identified. Furthermore, ancestry analysis showed a mainly Native American proportion.
UNASSIGNED: Based on the genomic results, the patient was identified to have a high-risk profile, and an implantable cardioverter defibrillator was selected as the best treatment option, highlighting the importance of including both the clinical and genomics aspects for an integral diagnosis.

暂无摘要。

Device acceptance is a crucial factor in identifying implantable cardioverter defibrillator (ICD) patients at risk for psychosocial distress and unfavorable quality of life outcomes. The purpose of this study was to examine the evidence of the validity of internal structure (construct) and reliability of the Florida Patient Acceptance Survey (FPAS) in a sample of ICD patients, comparing the psychometric indicators of the complete (FPAS-18 item) and abbreviated (FPAS-12 item) versions. The sample included 151 participants (97 males, mean age of 55.7 ± 14.1 years) who completed the cross-culturally adapted version of the FPAS instrument for the Brazilian context. The psychometric properties of both versions of the FPAS instrument were evaluated by two distinct approaches:•Exploratory and confirmatory factor analysis: used to test the internal structure of the instrument•Cronbach\'s Alpha and McDonald\'s Omega: used to determine the reliability of the instrument The two versions of the FPAS-Br instrument showed consistent evidence of internal structure validity and reliability. However, the FPAS-Br 12-item showed a better psychometric adjustment, confirmed by the analysis of the quality indicators of the models.

Electrophysiological (EP) operations that have traditionally involved long hospital lengths of stay (LOS) are now being undertaken as day case procedures. The coronavirus disease-19 pandemic served as an impetus for many centres to shorten LOS for EP procedures. This survey explores LOS for elective EP procedures in the modern era.
An online survey consisting of 27 multiple-choice questions was completed by 245 respondents from 35 countries. With respect to de novo cardiac implantable electronic device (CIED) implantations, day case procedures were reported for 79.5% of implantable loop recorders, 13.3% of pacemakers (PMs), 10.4% of implantable cardioverter defibrillators (ICDs), and 10.2% of cardiac resynchronization therapy (CRT) devices. With respect to CIED generator replacements, day case procedures were reported for 61.7% of PMs, 49.2% of ICDs, and 48.2% of CRT devices. With regard to ablations, day case procedures were reported for 5.7% of atrial fibrillation (AF) ablations, 10.7% of left-sided ablations, and 17.5% of right-sided ablations. A LOS ≥ 2 days for CIED implantation was reported for 47.7% of PM, 54.5% of ICDs, and 56.9% of CRT devices and for 54.5% of AF ablations, 42.2% of right-sided ablations, and 46.1% of left-sided ablations. Reimbursement (43-56%) and bed availability (20-47%) were reported to have no consistent impact on the organization of elective procedures.
There is a wide variation in the LOS for elective EP procedures. The LOS for some procedures appears disproportionate to their complexity. Neither reimbursement nor bed availability consistently influenced LOS.

Patients with cardiomyopathies and channelopathies are usually younger and have a predominantly arrhythmia-related prognosis; they have nearly normal life expectancy thanks to the protection against sudden cardiac death provided by the implantable cardioverter defibrillator (ICD). The subcutaneous ICD (S-ICD) is an effective alternative to the transvenous ICD and has evolved over the years. This study aimed to evaluate the rate of inappropriate shocks (IS), appropriate therapies, and device-related complications in patients with cardiomyopathies and channelopathies who underwent modern S-ICD implantation.
We enrolled consecutive patients with cardiomyopathies and channelopathies who had undergone implantation of a modern S-ICD from January 2016 to December 2020 and who were followed up until December 2022. A total of 1338 S-ICD implantations were performed within the observation period. Of these patients, 628 had cardiomyopathies or channelopathies. The rate of IS at 12 months was 4.6% [95% confidence interval (CI): 2.8-6.9] in patients with cardiomyopathies and 1.1% (95% CI: 0.1-3.8) in patients with channelopathies (P = 0.032). No significant differences were noted over a median follow-up of 43 months [hazard ratio (HR): 0.76; 95% CI: 0.45-1.31; P = 0.351]. The rate of appropriate shocks at 12 months was 2.3% (95% CI: 1.1-4.1) in patients with cardiomyopathies and 2.1% (95% CI: 0.6-5.3) in patients with channelopathies (P = 1.0). The rate of device-related complications was 0.9% (95% CI: 0.3-2.3) and 3.2% (95% CI: 1.2-6.8), respectively (P = 0.074). No significant differences were noted over the entire follow-up. The need for pacing was low, occurring in 0.8% of patients.
Modern S-ICDs may be a valuable alternative to transvenous ICDs in patients with cardiomyopathies and channelopathies. Our findings suggest that modern S-ICD therapy carries a low rate of IS.
URL: http://clinicaltrials.gov/Identifier: NCT02275637.

Transcutaneous electrical nerve stimulation (TENS) is an established method for pain relief. But electrical TENS currents are also a source of electromagnetic interference (EMI). Thus, TENS is considered to be contraindicated in implantable cardioverter-defibrillator (ICD) patients. However, data might be outdated due to considerable advances in ICD and cardiac resynchronization therapy (CRT) filtering and noise protection algorithm technologies. The aim of this pilot safety study was to re-evaluate the safety of TENS in patients with modern ICDs.
One hundred and seven patients equipped with 55 different models of ICD/CRT with defibrillators from 4 manufacturers underwent a standardized test protocol including TENS at the cervical spine and the thorax, at 2 stimulation modes-high-frequency TENS (80 Hz) and burst-mode TENS (2 Hz). Potential interference monitoring included continuous documentation of ECG Lead II, intracardiac electrograms and the marker channel. Electromagnetic interference was detected in 17 of 107 patients (15.9%). Most frequent were: interpretations as a premature ventricular beats (VS/S) in 15 patients (14%), noise reversion in 5 (4.6%) which resulted in temporary asynchronous pacing in 3 (2.8%), interpretation as ventricular tachycardia/ventricular fibrillation in 2 (1.9%), and premature atrial beat in 2 (1.9%) patients. Electromagnetic interference occurrence was influenced by position (chest, P < 0.01), higher current intensity (P < 0.01), and manufacturer (P = 0.012).
Overall, only intermittent and minor EMI were detected. Prior to the use of TENS in patients with ICDs, they should undergo testing under the supervision of a cardiac device specialist.

UNASSIGNED: Cardiac device-related infective endocarditis (CDRIE) is a severe complication of cardiac device (CD) implantation and is usually treated by antibiotic therapy and percutaneous device extraction. Few studies report the management and prognosis of CDRIE in real life. In particular, the rate of device extraction in clinical practice and the management of patients with left heart infective endocarditis (LHIE) and an apparently non-infected CD (LHIE+CDRIE-) are not well described.
UNASSIGNED: We sought to study in EURO-ENDO, the characteristics, prognosis, and management of 483 patients with a CD included in the European Society of Cardiology EurObservational Research Programme EURO-ENDO registry. Three populations were compared: 280 isolated CDRIE (66.7 ± 14.3 years), 157 patients with LHIE and an apparently non-infected CD (LHIE+CDRIE-) (71.1 ± 13.6), and 46 patients with both LHIE and CDRIE (LHIE+CDRIE+) (70.2 ± 10.1). Echocardiography was not always transoesophageal echography (TOE); it was transthoracic echography (TTE) for isolated CDRIE in 88.4% (TOE = 67.6%), for LHIE+CDRIE- TTE = 93.0% (TOE = 58.6%), and for CDRIE+LHIE+ TTE = 87.0% (TOE = 63.0%). Nuclear imaging was performed in 135 patients (positive for 75.6%). In-hospital mortality was lower in isolated CDRIE 13.2% vs. 22.3% and 30.4% for LHIE+CDRIE- and LHIE+CDRIE+ (P = 0004). Device extraction was performed in 62.1% patients with isolated CDRIE, 10.2% of LHIE+CDRIE- patients, and 45.7% of CDRIE+LHIE+ patients. Device extraction was associated with a better prognosis [hazard ratio 0.59 (0.40-0.87), P = 0.0068] even in the LHIE+CDRIE- group (P = 0.047).
UNASSIGNED: Prognosis of endocarditis in patients with a CD remains poor, particularly in the presence of an associated LHIE. Although recommended by guidelines, device extraction is not always performed. Device removal was associated with better prognosis, even in the LHIE+CDRIE- group.

Subcutaneous implantable cardioverter-defibrillator (S-ICD) therapy is expanding rapidly. However, there are few data on the S-ICD extraction procedure and subsequent patient management. The aim of this analysis was to describe the procedure, management, and outcome of S-ICD extractions in clinical practice.
We enrolled consecutive patients who required complete S-ICD extraction at 66 Italian centres. From 2013 to 2022, 2718 patients undergoing de novo implantation of an S-ICD were enrolled. Of these, 71 required complete S-ICD system extraction (17 owing to infection). The S-ICD system was successfully extracted in all patients, and no complications were reported; the median procedure duration was 40 (25th-75th percentile: 20-55) min. Simple manual traction was sufficient to remove the lead in 59 (84%) patients, in whom lead-dwelling time was shorter [20 (9-32) months vs. 30 (22-41) months; P = 0.032]. Hospitalization time was short in the case of both non-infectious [2 (1-2) days] and infectious indications [3 (1-6) days]. In the case of infection, no patients required post-extraction intravenous antibiotics, the median duration of any antibiotic therapy was 10 (10-14) days, and the re-implantation was performed during the same procedure in 29% of cases. No complications arose over a median of 21 months.
The S-ICD extraction was safe and easy to perform, with no complications. Simple traction of the lead was successful in most patients, but specific tools could be needed for systems implanted for a longer time. The peri- and post-procedural management of S-ICD extraction was free from complications and not burdensome for patients and healthcare system.
URL: http://clinicaltrials.gov/Identifier: NCT02275637.

In subcutaneous implantable cardioverter defibrillator (S-ICD) recipients, the UNTOUCHED study demonstrated a very low inappropriate shock rate on programming a conditional zone between 200 and 250 bpm and a shock zone for arrhythmias >250 bpm. The extent to which this programming approach is adopted in clinical practice is still unknown, as is its impact on the rates of inappropriate and appropriate therapies.
We assessed ICD programming on implantation and during follow-up in a cohort of 1468 consecutive S-ICD recipients in 56 Italian centres. We also measured the occurrence of inappropriate and appropriate shocks during follow-up. On implantation, the median programmed conditional zone cut-off was set to 200 bpm (IQR: 200-220) and the shock zone cut-off was 230 bpm (IQR: 210-250). During follow-up, the conditional zone cut-off rate was not significantly changed, while the shock zone cut-off was changed in 622 (42%) patients and the median value increased to 250 bpm (IQR: 230-250) (P < 0.001). UNTOUCHED-like programming of detection cut-offs was adopted in 426 (29%) patients immediately after device implantation, and in 714 (49%, P < 0.001) at the last follow-up. UNTOUCHED-like programming was independently associated with fewer inappropriate shocks (hazard ratio 0.50, 95%CI 0.25-0.98, P = 0.044), and had no impact on appropriate and ineffective shocks.
In recent years, S-ICD implanting centres have increasingly programmed high arrhythmia detection cut-off rates, at the time of implantation in the case of new S-ICD recipients, and during follow-up in the case of pre-existing implants. This has contributed significantly to reducing the incidence of inappropriate shocks in clinical practice. Rordorf: Programming of the S-ICD.
URL: http://clinicaltrials.gov/Identifier: NCT02275637.