Search

Anderson–Fabry disease (AFD) is a rare X-linked lysosomal storage disorder characterized by the accumulation of globotriaosylceramide, leading to multi-organ involvement and significant morbidity. Cardiovascular manifestations, particularly arrhythmias, are common and pose a considerable risk to affected individuals. This overview examines current approaches to arrhythmic risk stratification in AFD, focusing on the identification, assessment, and management of cardiac arrhythmias associated with the disease. We explore advancements in diagnostic techniques, including echocardiography, cardiac MRI, and ambulatory ECG monitoring, to enhance the detection of arrhythmogenic substrate. Furthermore, we discuss the role of genetic and biochemical markers in predicting arrhythmic risk and the implications for personalized treatment strategies. Current therapeutic interventions, including enzyme replacement therapy and antiarrhythmic medications, are reviewed in the context of their efficacy and limitations. Finally, we highlight ongoing research and future directions with the aim of improving arrhythmic risk assessment and management in AFD. This overview underscores the need for a multidisciplinary approach to optimize care and outcomes for patients with AFD. [ABSTRACT FROM AUTHOR] more...

Cardiac amyloidosis is the most frequent infiltrative disease caused by the deposition of misfolded proteins in the cardiac tissue, leading to heart failure, brady- and tachyarrhythmia and death. Conduction disorders, atrial fibrillation (AF) and ventricular arrhythmia (VA) significantly impact patient outcomes and demand recognition. However, several issues remain unresolved regarding early diagnosis and optimal management. Extreme bradycardia is the most common cause of arrhythmic death, while fast and sustained VAs can be found even in the early phases of the disease. Risk stratification and the prevention of sudden cardiac death are therefore to be considered in these patients, although the time for defibrillator implantation is still a subject of debate. Moreover, atrial impairment due to amyloid fibrils is associated with an increased risk of AF resistant to antiarrhythmic therapy, as well as recurrent thromboembolic events despite adequate anticoagulation. In the last few years, the aging of the population and progressive improvements in imaging methods have led to increases in the diagnosis of cardiac amyloidosis. Novel therapies have been developed to improve patients’ functional status, quality of life and mortality, without data regarding their effect on arrhythmia prevention. In this review, we consider the latest evidence regarding the arrhythmic risk stratification of cardiac amyloidosis, as well as the available therapeutic strategies. more...

A 40-year-old woman with symptomatic advanced atrioventricular (AV) block underwent cardioneuroablation (CNA) for the treatment of functional bradyarrhythmia, following the exclusion of reversible causes and intrinsic disease of the conduction system. Selective ablation of ganglia related to the AV node, performed exclusively in the left atrium, restored AV conduction, which has remained unchanged with 2 years of follow-up. CNA is a viable and effective option with low risk for the treatment of functional bradyarrhythmias, regardless of their severity., (© 2025 Wiley Periodicals LLC.) more...

Right Ventricular Pacing (RVP) may have detrimental effects in ventricular function. Left Bundle Branch Area Pacing (LBBAP) is a new pacing strategy that appears to have better results. The aim of this systematic review and meta-analysis is to compare the safety and efficacy of LBBAP vs RVP in patients with bradyarrhythmia and conduction system disorders. MEDLINE, EMBASE and Pubmed databases were searched for studies comparing LBBAP with RVP. Outcomes were all-cause mortality, atrial fibrillation (AF) occurrence, heart failure hospitalizations (HFH) and complications. QRS duration, mechanical synchrony and LVEF changes were also assessed. Pairwise meta-analysis was conducted using random and fixed effects models. Twenty-five trials with 4250 patients (2127 LBBAP) were included in the analysis. LBBAP was associated with lower risk for HFH (RR:0.33, CI 95%:0.21 to 0.50; p < 0.001), all-cause mortality (RR:0.52 CI 95%:0.34 to 0.80; p = 0.003), and AF occurrence (RR:0.43 CI 95%:0.27 to 0.68; p < 0.001) than RVP. Lead related complications were not different between the two groups (p = 0.780). QRSd was shorter in the LBBAP group at follow-up (WMD: −32.20 msec, CI 95%: −40.70 to −23.71; p < 0.001) and LBBAP achieved better intraventricular mechanical synchrony than RVP (SMD: -1.77, CI 95%: −2.45 to −1.09; p < 0.001). LBBAP had similar pacing thresholds (p = 0.860) and higher R wave amplitudes (p = 0.009) than RVP. LBBAP has better clinical outcomes, preserves ventricular electrical and mechanical synchrony and has excellent pacing parameters, with no difference in complications compared to RVP. • Systematic review and meta-analysis of twenty-five trials with 4250 patients. • LBBAP has lower risk of Heart Failure Hospitalizations compared to RVP. • LBBAP shows better results in all-cause mortality than RVP. • AF occurrence rates are lower with LBBAP. [ABSTRACT FROM AUTHOR] more...

Cardiac electrophysiologic derangements often coexist with disorders of the circulatory system. Capturing and diagnosing arrhythmias and conduction system disease may lead to a change in diagnosis, clinical management and patient outcomes. Standard 12-lead electrocardiogram (ECG), Holter monitors and event recorders have served as useful diagnostic tools over the last few decades. However, their shortcomings are only recently being addressed by emerging technologies. With advances in device miniaturization and wireless technologies, and changing consumer expectations, wearable ‘on-body’ ECG patch devices have evolved to meet contemporary needs. These devices are unobtrusive and easy to use, leading to increased device wear time and diagnostic yield. While becoming the standard for detecting arrhythmias and conduction system disorders in the outpatient setting where continuous ECG monitoring in the short to medium term (days to weeks) is indicated, these cardiac devices and related digital mobile health technologies are reshaping the clinician-patient interface with important implications for future healthcare delivery. more...