Prevalence and predictors of bradyarrhythmias requiring Permanent Pacing in patients with Anderson-Fabry disease.

Introduction: Bradyarrhythmias are an established red flag for storage cardiac conditions including Anderson-Fabry disease (AFD). The prevalence of bradyarrhythmias requiring a pacemaker (PM) and their timing in AFD is unresolved. We evaluated prevalence and predictors of PM requirement in a large AFD cohort, investigating the occurrence of bradyarrhythmias as initial versus late manifestation. Methods: we retrospectively evaluated 82 consecutive AFD patients referred to our multidisciplinary referral centre from 1994 to 2020 with a median follow up of 6.9 years, identifying those requiring pacing. Univariable analysis was performed to identify cardiac features associated with PM implantantion. Results: Five of 82 (6%) AFD patients required PM implantation (5/39, i.e. 13% of those with cardiac involvement), always in the context of advanced cardiomyopathy. In none, bradyarrhythmias were the presenting feature. Indications included sick sinus syndrome in 3 patients, advanced atrio-ventricular block in 2 patients. QRS prolongation during follow up strongly correlated with the onset of bradyarrhythmias. Conclusions: Severe bradyarrhythmias are relatively frequent in patients with AFD cardiomyopathy, but do not represent a mode of presentation, occurring late in the disease course and always in the context of advanced cardiac involvement. Monitoring QRS variations over time may help to identify patients requiring pacing.

The Atrioventricular Conduction Axis and its Implications for Permanent Pacing

Extensive knowledge of the anatomy of the atrioventricular conduction axis, and its branches, is key to the success of permanent physiological pacing, either by capturing the His bundle, the left bundle branch or the adjacent septal regions. The inter-individual variability of the axis plays an important role in underscoring the technical difficulties known to exist in achieving a stable position of the stimulating leads. In this review, the key anatomical features of the location of the axis relative to the triangle of Koch, the aortic root, the inferior pyramidal space and the inferoseptal recess are summarised. In keeping with the increasing number of implants aimed at targeting the environs of the left bundle branch, an extensive review of the known variability in the pattern of ramification of the left bundle branch from the axis is included. This permits the authors to summarise in a pragmatic fashion the most relevant aspects to be taken into account when seeking to successfully deploy a permanent pacing lead.

An unusual case of pediatric inflammatory multisystem syndrome, due to severe acute respiratory syndrome coronavirus-2 infection, presenting with complete heart block needing permanent pacing

Pediatric inflammatory multisystem syndrome due to severe acute respiratory syndrome coronavirus-2 infection is not very common. It may vary from a simple febrile inflammatory response to a severe multisystem involvement including myocarditis and shock. Here, we report the case of a 10-year-old female child with fever, rash, and mucositis who presented with shock and complete heart block along with encephalopathy and convulsions. The primary investigations revealed raised inflammatory markers along with a high titer of COVID antibodies. She was given full inotrope support along with immunomodulators. She underwent temporary pacing but ultimately needed a dual-chamber pacemaker. The novel coronavirus in children can present with a myriad of manifestations affecting almost every organ. In these pandemic times, one must always keep in mind the possibility of COVID-19 infection in a child that presents with shock, carditis, and arrhythmias. These children need intense immunosuppression and must be managed very aggressively

Conduction system pacing in heart failure patients

Direct conduction system pacing delivers more physiological cardiac activation and can potentially correct intraventricular conduction disturbances and restore normal activation pathways. Permanent pacing that maintains cardiac electromechanical synchrony is essential in heart failure and reduced left ventricular ejection fraction. Conduction system pacing has recently emerged as an alternative to right ventricular pacing and biventricular resynchronization therapy. In this article, we review conduction system pacing in heart failure patients.

Temporary pacing: an overview

This article will review pacing in the context of acute care, with a focus on temporary pacing. Beginning with an overview of the heart's intrinsic electrical conduction system and its function, the discussion moves on to pacing, acute clinical presentations and indications for pacing. This includes an overview of temporary pacing, with the different approaches being percussion, transcutaneous, transvenous and epicardial. There are specific cardiac patient groups at high risk of developing bradyarrhythmias and, in the absence of other measures, potentially requiring emergency pacing. These groups include patients who have experienced a myocardial infarction following cardiac surgery and post transcatheter aortic valve implant. Nurses providing care for patients with temporary transvenous wires need an understanding of the potential complications and to recognise indications for moving on to a permanent pacing system.

Evidence-Based Analysis of the Emergency Temporary Cardiac Pacing (Electrical Stimulation from Metal Wire Electrode)

The patient’s Cr (creatinine), BUN (blood urea nitrogen), HBG (hemoglobin), VT (ventricular tachycardia), pacing frequency, puncture point, emergency to permanent pacing time, pacing current (mA), pacing threshold current (mA), and admission diagnosis data were collected. The data were subjected to frequency statistics, curve regression analysis, PLS regression analysis, adjustment analysis, chi-square test, ridge regression analysis, discriminant analysis, negative binomial regression analysis, Poisson regression analysis, and stepwise regression analysis. Some findings include the following: (1) Cr has a significant positive effect on HBG, and BUN has a significant negative effect on HBG. (2) VT has a negative correlation with age and a positive correlation with CK-MB and CK (creatinine kinase). (3) Myocarditis has a negative correlation with age and a significant positive correlation with CTnI (cardiac troponin I). (4) AST (aspartate transaminase) and ALT (alanine aminotransferase) have a significant positive impact on DDI (D-dimer), while CTnI has a significant negative impact on DDI. MYO (myoglobin) has no impact relationship to DDI. (5) ALT has a significant positive relationship with APTT (partial thromboplastin time). (6) Alb (albumin) and TBIL (total bilirubin) have a significant positive effect on PLT (platelet) count, while pro-BNP (B-type natriuretic peptide) and MYO have a significant negative effect on PLT. (7) CK has a significant positive effect on INR (international normalized ratio). (8) The relationship between sinus node dysfunction and VT significantly affect the pacing frequency (beats/minute). For third-degree atrioventricular block, different samples of sinus node dysfunction showed significant differences. (9) There is a significant positive correlation between pacing current (mA) and pacing threshold current (mA). (10) There was a significant positive correlation between perceived voltage (mV) and the time from emergency to permanent pacing. Admission diagnosis has a significant positive impact on the time from emergency to permanent pacing. The change (increase) in time from emergency to permanent pacing was 1.137-fold when an additional condition was diagnosed on admission.