Andersen-Tawil Syndrome type 1 (ATS1) is a rare arrhythmogenic disorder, caused by loss-of-function mutations in the KCNJ2 gene. We present here the largest cohort of patients with ATS1 with outcome ...data reported.
This study sought to define the risk of life-threatening arrhythmic events (LAE), identify predictors of such events, and define the efficacy of antiarrhythmic therapy in patients with ATS1.
Clinical and genetic data from consecutive patients with ATS1 from 23 centers were entered in a database implemented at ICS Maugeri in Pavia, Italy, and pooled for analysis.
We enrolled 118 patients with ATS1 from 57 families (age 23 ± 17 years at enrollment). Over a median follow-up of 6.2 years (interquartile range: 2.7 to 16.5 years), 17 patients experienced a first LAE, with a cumulative probability of 7.9% at 5 years. An increased risk of LAE was associated with a history of syncope (hazard ratio HR: 4.54; p = 0.02), with the documentation of sustained ventricular tachycardia (HR 9.34; p = 0.001) and with the administration of amiodarone (HR: 268; p < 0.001). The rate of LAE without therapy (1.24 per 100 person-years py) was not reduced by beta-blockers alone (1.37 per 100 py; p = 1.00), or in combination with Class Ic antiarrhythmic drugs (1.46 per 100 py, p = 1.00).
Our data demonstrate that the clinical course of patients with ATS1 is characterized by a high rate of LAE. A history of unexplained syncope or of documented sustained ventricular tachycardia is associated with a higher risk of LAE. Amiodarone is proarrhythmic and should be avoided in patients with ATS1.
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Purkinje cells (PCs) are more resistant to ischemia than myocardial cells, and are suspected to participate in ventricular arrhythmias following myocardial infarction (MI). Histological studies ...afford little evidence on the behavior and adaptation of PCs in the different stages of MI, especially in the chronic stage, and no quantitative data have been reported to date beyond subjective qualitative depictions. The present study uses a porcine model to present the first quantitative analysis of the distal cardiac conduction system and the first reported change in the spatial distribution of PCs in three representative stages of MI: an acute model both with and without reperfusion; a subacute model one week after reperfusion; and a chronic model one month after reperfusion. Purkinje cells are able to survive after 90 minutes of ischemia and subsequent reperfusion to a greater extent than cardiomyocytes. A decrease is observed in the number of PCs, which suffer reversible subcellular alterations such as cytoplasm vacuolization, together with redistribution from the mesocardium-the main localization of PCs in the heart of ungulate species-towards the endocardium and perivascular epicardial areas. However, these changes mainly occur during the first week after ischemia and reperfusion, and are maintained in the chronic stages. This anatomical substrate can explain the effectiveness of endo-epicardial catheter ablation of monomorphic ventricular tachycardias in the chronic scar after infarction, and sets a basis for further electrophysiological and molecular studies, and future therapeutic strategies.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background:
Arrhythmia recurrence after atrial fibrillation (AF) ablation is often associated with pulmonary vein reconnection (PVR). We prospectively examined anatomical sites of both acute and ...chronic PVR.
Methods:
One hundred and fifty AF patients underwent PV wide encirclement and sites where immediate electrical isolation (EI) occurred were tagged using electroanatomic mapping/CT integration (Cartomerge™, Biosense Webster, Diamond Bar, CA, USA). After 30 minutes PVs were checked and acute PVR sites marked at reisolation. Chronic PVR sites were marked at the time of repeat procedures.
Results:
On the left, immediate EI sites were predominantly on the intervenous ridge (IVR) and PV‐left atrial appendage (PV‐LAA) ridge. On the right they were at the roof, IVR, and floor of the PVs. Ninety‐eight of one hundred and fifty patients had PVs checked after >30 minutes. Thirty‐two of ninety‐eight had acute PVR. This was mostly on the IVR and PV‐LAA ridge on the left (88%), and on the roof and IVR on the right (78%). At repeat procedure, 38/39 patients had chronic PVR, predominantly on the IVR (61%) and PV‐LAA ridge (21%) on the left, and on the roof, IVR, and floor of the right PVs (79%). There was minimal acute or chronic PVR posteriorly. Acutely PVR occurred close to the immediate EI site 60% of the time, but only 30% of the time chronically.
Conclusion:
Acute and chronic PVR sites have a preferential distribution. This may be determined by anatomical and technical factors. Knowledge of immediate EI sites may be beneficial acutely, but with chronic PVR a careful survey is required. These findings may help target ablation, improving safety and success.
Cardiac resynchronization therapy (CRT) via biventricular pacing (BiVP-CRT) is considered a mainstay treatment for symptomatic heart failure patients with reduced ejection fraction and wide QRS. ...However, up to one-third of patients receiving BiVP-CRT are considered non-responders to the therapy. Multiple strategies have been proposed to maximize the percentage of CRT responders including two new physiological pacing modalities that have emerged in recent years: His bundle pacing (HBP) and left bundle branch area pacing (LBBAP). Both pacing techniques aim at restoring the normal electrical activation of the ventricles through the native conduction system in opposition to the cell-to-cell activation of conventional right ventricular myocardial pacing. Conduction system pacing (CSP), including both HBP and LBBAP, appears to be a promising pacing modality for delivering CRT and has proven to be safe and feasible in this particular setting. This article will review the current state of the art of CSP-based CRT, its limitations, and future directions.
Abstract Objectives The aim of this study was to determine if noninvasive measurement of scar by contrast-enhanced magnetic resonance imaging (MRI)–based signal intensity (SI) mapping predicts ...ventricular tachycardia (VT) recurrence after endocardial ablation. Background Scar extension on voltage mapping predicts VT recurrence after ablation procedures. Methods A total of 46 consecutive patients with previous myocardial infarction (87% men, mean age 68 ± 9 years, mean left ventricular ejection fraction 36 ± 10%) who underwent VT substrate ablation before the implantation of a cardioverter-defibrillator were included. Before ablation, contrast-enhanced MRI was performed, and areas of endocardial and epicardial scarring and heterogeneous tissue were measured; averaged subendocardial and subepicardial signal intensities were projected onto 3-dimensional endocardial and epicardial shells in which dense scar, heterogeneous tissue, and normal tissue were differentiated. Results During a mean follow-up period of 32 ± 24 months 17 patients (37%) had VT recurrence. Patients with recurrence had larger scar and heterogeneous tissue areas on SI maps in both endocardium (81 ± 27 cm2 vs. 48 ± 21 cm2 p = 0.001 and 53 ± 21 cm2 vs. 30 ± 15 cm2 p = 0.001, respectively) and epicardium (76 ± 28 cm2 vs. 51 ± 29 cm2 p = 0.032 and 59 ± 25 cm2 vs. 37 ± 19 cm2 p = 0.008). In the multivariate analysis, MRI endocardial scar extension was the only independent predictor of VT recurrence (hazard ratio: 1.310 per 10 cm2 ; 95% confidence interval: 1.051 to 1.632; p = 0.034). Freedom from VT recurrence was higher in patients with small endocardial scars by MRI (<65 cm2 ) than in those with larger scars (≥65 cm2 ) (85% vs. 20%, log-rank p = 0.018). Conclusions Pre-procedure endocardial scar extension assessment by contrast-enhanced MRI predicts VT recurrence after endocardial substrate ablation. This information may be useful to select patients for ablation procedures.
Introduction
Implant procedure features and clinical implications of left bundle branch pacing (LBBP) and left ventricular septal pacing (LVSP) have not been yet fully described. We sought to compare ...two different left bundle branch area pacing (LBBAP) implant strategies: the first one accepting LVSP as a procedural endpoint and the second one aiming at achieving LBBP in every patient in spite of evidence of previous LVSP criteria.
Methods
LVSP was accepted as a procedural endpoint in 162 consecutive patients (LVSP strategy group). In a second phase, LBBP was attempted in every patient in spite of achieving previous LVSP criteria (n = 161, LBBP strategy group). Baseline patient characteristics, implant procedure, and follow‐up data were compared.
Results
The final capture pattern was LBBP in 71.4% and LVSP in 24.2% in the LBBP strategy group compared to 42.7% and 50%, respectively, in the LVSP strategy group. One hundred and eighty‐four patients (57%) had proven LBB capture criteria with a significantly shorter paced QRS duration than the 120 patients (37%) with LVSP criteria (115 ± 9 vs. 121 ± 13 ms, p < .001). Implant parameters were comparable between the two strategies but the LBBP strategy resulted in a higher rate of acute septal perforation (11.8% vs. 4.9%, p = .026) without any clinical sequelae. Patients with CRT indications significantly improved left ventricular ejection fraction (LVEF) during follow‐up irrespective of the capture pattern (from 35 ± 11% to 45 ± 14% in proven LBBP, p = .024; and from 39 ± 13% to 47 ± 12% for LVSP, p = .003). The presence of structural heart disease and baseline LBBB independently predicted unsuccessful LBB capture.
Conclusion
The LBBP strategy was associated with comparable implant parameters than the LVSP strategy but resulted in higher rates of septal perforation. Proven LBB capture and LVSP showed comparable effects on LVEF during follow‐up.
During LBBAP, a strategy aiming to obtain LBB capture in every patient compared to a strategy accepting LVSP as a primary endpoint resulted in comparable implant parameters but higher rates of septal perforation. Patients with proven LBB capture showed a significantly narrow paced QRS but comparable LVEF changes to LVSP.
Introduction
Conduction system pacing (CSP) has emerged as an ideal physiologic pacing strategy for patients with permanent pacing indications. We sought to evaluate the safety and feasibility of CSP ...in a consecutive series of unselected patients with congenital heart disease (CHD).
Methods
Consecutive patients with CHD in which CSP was attempted were included. Safety and feasibility, implant tools and electrical parameters at implant and at follow‐up were evaluated.
Results
A total of 20 patients were included (10 with a previous device). A total of 10 patients had complex forms of CHD, 9 moderate defects and 1 a simple defect. CSP was achieved in 75% of cases (10 His bundle pacing, 5 left bundle branch pacing) with left ventricular septal pacing in the remaining 5 patients. Procedure times and fluoroscopy times were prolongued (126 ± 82 min and 27 ± 30 min, respectively). Ventricular lead implant times widely varied ranging from 4 to 115 min, (mean 31 ± 28 min) and the use of multiple delivery sheaths was frequent (50%). The QRS width was reduced from 145 ± 36 ms at baseline to 116 ± 18 ms with CSP. Implant electrical parameters included: CSP pacing threshold 0.95 ± 0.65 V; R wave amplitude 9.2 ± 8.8 mV and pacing impedance 632 ± 183 Ohms, and remained stable at a median follow‐up of 478 days (interquartile range: 225–567). Systemic ventricle systolic function and NYHA class (1.50 ± 0.51 vs. 1.10 ± 0.31; p = .008) significantly improved at follow‐up. Lead revision was required in one patient at Day 4.
Conclusions
Permanent CSP is safe and feasible in patients with CHD although implant technique is complex.
BACKGROUND—Epicardial ablation has shown improvement in clinical outcomes of patients with ischemic heart disease (IHD) after ventricular tachycardia (VT) ablation. However, usually epicardial access ...is only performed when endocardial ablation has failed. Our aim was to compare the efficacy of endocardial+epicardial ablation versus only endocardial ablation in the first procedure in patients with IHD.
METHODS AND RESULTS—Fifty-three patients with IHD, referred for a first VT ablation to our institution, from 2012 to 2014, were included. They were divided in 2 groups according to enrollment timefrom May 2013, we started to systematically perform endo-epicardial access (Epi-Group) as first-line approach in consecutive patients with IHD (n=15). Patients who underwent only an endocardial VT ablation in their first procedure (Endo-Group) included patients with previous cardiac surgery and the historical (before May 2013; n=35). All late-potentials in the scar zone were eliminated, and if VT was tolerated, critical isthmuses were also approached. The end point was the noninducibility of any VT. During a median follow-up of 15±10 months, the combined end point (hospital or emergency admission because of a ventricular tachycardia or reablation) occurred in 14 patients of the Endo-group and in one patient in the Epi-group (event-free survival curves by Grey-test, P=0.03). Ventricular arrhythmia recurrences occurred in 16 and in 3 patients in the Endo and Epi-Group, respectively (Grey-test, P=0.2).
CONCLUSIONS—A combined endocardial–epicardial ablation approach for initial VT ablation was associated with fewer readmissions for VT and repeat ablations. Further studies are warranted.
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