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Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and sudden cardiac death represents an important mode of death in these patients. Data evaluating the implantable cardioverter defibrillator (ICD) in this patient population remain scarce. Methods: A Nationwide French Registry including all patients with tetralogy of Fallot with an ICD was initiated in 2010 by the French Institute of Health and Medical Research. The primary time to event end point was the time from ICD implantation to first appropriate ICD therapy. Secondary outcomes included ICD-related complications, heart transplantation, and death. Clinical events were centrally adjudicated by a blinded committee. Results: A total of 165 patients (mean age, 42.2±13.3 years, 70.1% males) were included from 40 centers, including 104 (63.0%) in secondary prevention. During a median (interquartile range) follow-up of 6.8 (2.5–11.4) years, 78 (47.3%) patients received at least 1 appropriate ICD therapy. The annual incidence of the primary outcome was 10.5% (7.1% and 12.5% in primary and secondary prevention, respectively; P =0.03). Overall, 71 (43.0%) patients presented with at least 1 ICD complication, including inappropriate shocks in 42 (25.5%) patients and lead dysfunction in 36 (21.8%) patients. Among 61 (37.0%) patients in primary prevention, the annual rate of appropriate ICD therapies was 4.1%, 5.3%, 9.5%, and 13.3% in patients with, respectively, 0, 1, 2, or ≥3 guidelines-recommended risk factors. QRS fragmentation was the only independent predictor of appropriate ICD therapies (hazard ratio, 3.47 [95% CI, 1.19–10.11]), and its integration in a model with current criteria increased the 5-year time-dependent area under the curve from 0.68 to 0.81 ( P =0.006). Patients with congestive heart failure or reduced left ventricular ejection fraction had a higher risk of nonarrhythmic death or heart transplantation (hazard ratio, 11.01 [95% CI, 2.96–40.95]). Conclusions: Patients with tetralogy of Fallot and an ICD experience high rates of appropriate therapies, including those implanted in primary prevention. The considerable long-term burden of ICD-related complications, however, underlines the need for careful candidate selection. A combination of easy-to-use criteria including QRS fragmentation might improve risk stratification. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT03837574.

Association between Hydroxychloroquine (HCQ) and Azithromycin (AZT) is under evaluation for patients with lower respiratory tract infection (LRTI) caused by the Severe Acute Respiratory Syndrome (SARS‐CoV‐2). Both drugs have a known torsadogenic potential, but sparse data are available concerning QT prolongation induced by this association. Our objective was to assess for COVID‐19 LRTI variations of QT interval under HCQ/AZT in patients hospitalized, and to compare manual versus automated QT measurements. Before therapy initiation, a baseline 12 lead‐ECG was electronically sent to our cardiology department for automated and manual QT analysis (Bazett and Fridericia’s correction), repeated 2 days after initiation. According to our institutional protocol (Pasteur University Hospital), HCQ/AZT was initiated only if baseline QTc ≤ 480ms and potassium level > 4.0 mmol/L. From March 24th to April 20th 2020, 73 patients were included (mean age 62 ± 14 y, male 67 %). Two patients out of 73 (2.7 %) were not eligible for drug initiation (QTc ≥ 500ms). Baseline average automated QTc was 415 ± 29 ms and lengthened to 438 ± 40 ms after 48 hours of combined therapy. The treatment had to be stopped because of significant QTc prolongation in 2 out of 71 patients (2.8 %). No drug‐induced life‐threatening arrhythmia, nor death was observed. Automated QTc measurements revealed accurate in comparison with manual QTc measurements. In this specific population of inpatients with COVID‐19 LRTI, HCQ/AZT could not be initiated or had to be interrupted in less than 6% of the cases.

Background The axillary route use for cardiac devices implantation has recently expanded either with fluoroscopy or ultrasounds guidance. Few studies included defibrillators (ICD), cardiac resynchronization therapy (CRT) and upgrade procedures for ultrasound-guided axillary vein puncture (UGVP). Puropose To assess the feasibility/safety of UGVP for complex cardiac devices implantation including CRT/ICD. Methods Consecutive patients eligible for a pacemaker or ICD implantation were included. All procedures were performed by three operators (one experienced and two fellows). Guidewires insertion time (from lidocaïne administration), and complications were systematically studied. A group of patients implanted with alternative techniques was used for comparison (cephalic, subclavian). Results In 176 consecutive patients in whom UGVP was used, a total of 68 complex procedures were analyzed (74 ± 8 y, male 61 %) with 138 leads implanted including 42 ICD, 48 CRT and 16 upgrade procedures. A majority (83 %) was under anti-thrombotic therapy. UGVP was successful in 96.8 %. Mean insertion time for 1.78 guidewires per patient was 4.4 ± 4.4 min. Guidewires insertion time reached its plateau after 10 patients. One pocket hematoma (1.4 %) was drained during a mean follow-up of 12 ± 5 months. The control group included 28 patients (12 subclavian, 16 cephalic; 15 ICD, 18 CRT, 4 upgrade procedures), with a mean insertion time of 10 ± 8 min, for 1.95 guidewires per patient (p < 0.0005). Conclusion UGVP is feasible and safe even for complex device implantations including CRT/ICD and upgrade procedures.

Aim Axillary vein access for pacemaker implantation is uncommon in many centres because of the lack of training in this technique. We assessed whether the introduction of the axillary vein technique was safe and efficient as compared with cephalic vein access, in a centre where no operators had any previous experience in axillary vein puncture. Methods and results Patients undergoing pacemaker implantation were randomized to axillary or cephalic vein access. All three operators had no experience nor training in axillary vein puncture, and self-learned the technique by reading a published review. Axillary vein puncture was fluoroscopy-guided without contrast venography. Cephalic access was performed by dissection of delto-pectoral groove. Venous access success, venous access duration (from skin incision to guidewire or lead in superior vena cava), procedure duration, X-ray exposure, and peri-procedural (1 month) complications were recorded. results We randomized 74 consecutive patients to axillary ( n = 37) or cephalic vein access ( n = 37). Axillary vein was successfully accessed in 30/37 (81.1%) patients vs. 28/37 (75.7%) of cephalic veins ( P = 0.57). Venous access time was shorter in axillary group than in cephalic group [5.7 (4.4–8.3) vs. 12.2 (10.5–14.8) min, P

Background The use of intracardiac electrograms to guide atrial fibrillation (AF) ablation has yielded conflicting results. Objectives The authors evaluated the usefulness of spatiotemporal dispersion, a visually recognizable electric footprint of AF drivers, for the ablation of all forms of AF. Methods The authors prospectively enrolled 105 patients admitted for AF ablation. AF was sequentially mapped in both atria with a 20-pole PentaRay catheter. The authors tagged and ablated only regions displaying electrogram dispersion during AF. Results were compared to a validation set in which a conventional ablation approach was used (pulmonary vein isolation/stepwise approach). To establish the mechanism underlying spatiotemporal dispersion of AF electrograms, the authors conducted realistic numerical simulations of AF drivers in a 2-dimensional model and optical mapping of ovine atrial scar-related AF. Results Ablation at dispersion areas terminated AF in 95% of the 105 patients. After ablation of 17 ± 10% of the left atrial surface and 18 months of follow-up, the atrial arrhythmia recurrence rate was 15% after 1.4 ± 0.5 procedures per patient versus 41% in the validation set after 1.5 ± 0.5 procedures per patient (arrhythmia free-survival: 85% vs. 59%; log-rank p Conclusions The clustering of intracardiac electrograms exhibiting spatiotemporal dispersion is indicative of AF drivers. Their ablation allows for a nonextensive and patient-tailored approach to AF ablation. (Substrate Ablation Guided by High Density Mapping in Atrial Fibrillation [SUBSTRATE HD]; NCT02093949)

BACKGROUND Whether cavotricuspid isthmus (CTI) is a region of conduction slowing during typical flutter has been discussed with conflicting results in the literature. We aimed to evaluate conduction velocity (CV) along the different portions of the typical flutter circuit with a recently proposed method by means of ultra-high-resolution (UHR) mapping. METHODS Consecutive patients referred for typical atrial flutter (AFL) ablation underwent UHR mapping (Rhythmia, Boston Scientific). CVs were measured in the CTI as well as laterally and septally, respectively, from its lateral and septal borders. RESULTS A total of 33 patients (mean age: 65 ± 13 years; right atrial volume: 134 ± 57 mL) were mapped either during ongoing counterclockwise (n = 25), or clockwise (n = 3) AFL (mean cycle length: 264 ± 38 ms), or during coronary sinus pacing at 400 ms (n = 1), 500 ms (n = 1), or 600 ms (n = 3). A total of 13 671 ± 7264 electrograms were acquired in 14 ± 9 min. CTI CV was significantly lower (0.56 ± 0.18 m/s) in comparison with the lateral CV (1.31 ± 0.29 m/s; P

Background Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare inherited channelopathy, responsible for potentially lethal malignant arrhythmic episodes. Case The index case was a 58-year-old woman who experienced aborted sudden cardiac arrest. The initial complete diagnostic work up (including norepinephrine challenge) was completely negative. She was implanted with an entirely subcutaneous defibrillator. During her follow-up, she received an appropriate electrical shock (ventricular fibrillation). Three sisters had sudden cardiac deaths (SCD) without etiology in the family history. There were no additional reports of pregnancy loss, neonatal death, seizures or SCD in family members. The genetic analysis revealed a Ryanodine Receptor type 2 R4608Q missense variant in this proband. Unfortunately, before the result of the proband's genetic analysis, her daughter experienced a SCD, whilst being implanted with an implantable loop recorder ( Fig. 1 ). A comprehensive genetic analysis was performed in the whole family using Next Generation Sequencing. All the carriers were treated with Nadolol 80 mg per day, and the follow-up was uneventful after six months. Conclusion A novel Ryanodine Receptor type 2 R4608Q mutation is described in a non-juvenile familial form of CPVT.

Background Active fixation leads for acute complete atrioventricular block (AVB 3) have recently been introduced in the last international guidelines for cardiac pacing. Purpose To describe the routine management of acute AVB 3 in France, with emphasis on the organisational aspects. Methods From September 2019 to November 2019, a prospective national survey including 29 questions was electronically sent to 100 physicians (Google Form). Results The answers were collected from 93 physicians (response rate 93%). A temporary active fixation lead for AVB 3 has already been implanted by 50.5% of the operators. Eighty (86%) have already observed a dislocation of the temporary pacing lead (TPL), a cardiac perforation already occurred in 57 (61.3%), a groin hematoma in 35 (37.6%), and this technique was proscribed for 4.3% of the operators. Definitive pacemaker implantation during weekends and nights (after 8PM) is possible for 48.9% of the operators ( 5 times a year), impossible for 35.9% of the operators. For AVB3 non-responsive to isoproterenol occurring during the night ( Fig. 1 ), a TPL is implanted by: the on-site medical staff on-duty (27.2%), the on-call interventional cardiologist (20.7%), the on-call electrophysiologist (18.5%), a permanent pacemaker is implanted by the electrophysiologist (12%), the strategy is not standardised (15.2%). Conclusion Our survey shows important disparities in terms of management of acute AVB3 among the different centres, and a high incidence of complications with temporary passive pacing leads. An active fixation lead with an external pacemaker was used by half of the centres.

Since more than a half century, cardiac pacing and defibrillation represent a field in constant evolution, and they have shown some great technological advances from its conception to its methods of insertion. In this review, the recent developments about the accesses for pacemakers and ICD will be described: the axillary and the femoral vein. The His bundle pacing and the advantages of the entirely subcutaneous defibrillator will also be presented.

Introduction During ablation procedures in the left atrium (LA), safe puncture of the septum requires precise localization of the fossa ovalis (FO). An intracavitary echocardiography (ICE) catheter positioned in the right atrium provides clear visualization of the interatrial septum and FO. The aim of this multicenter observational study was to assess the feasibility of trans-septal puncture under ICE guidance and its impact on procedure length. Methods This is an observational study conducted during one year in 8 centers specialized in ablation techniques requiring a trans-septal approach. The following data were collected amongst other things: time required for trans-septal catheterization, length of the FO to the LA wall before advancement of the trans-septal dilator, distance when maximum tenting of the FO by the trans-septal dilator, complications occurring during or at distance from the procedure. Results The study population included 123 patients (M 72%; mean age 60±10 years). Median times were as following: to prepare ICE catheter, 70 sec (38; 115); to insert ICE into the sheath, 40 sec (21; 88); to reach the LA, 153 sec (69; 310); time of fluoroscopy duration was 142 sec (80;256). Length of the FO was 16 ± 5 mm, distance from the FO to the LA wall before advancement of the trans-septal dilator was 29 ± 9 mm, distance when maximum tenting of the FO by the trans-septal dilator 15.9 ± 9 mm. Pericardial effusions occurred in 10 patients: 1 severe tamponade necessitating pericardial puncture (FO was not visible with ICE), 6 moderate per procedure and 3 light delayed pericardial effusions (not related to trans septal puncture). Conclusion This observational study shows that ICE does not add a long additional time to ablation procedure. It gives a direct indication of the position of the FO and the length of the LA. Although a tamponade occurred in one patient (0.8%), this technique is helpful in procedures requiring a transseptal approach. The author hereby declares no conflict of interest

This study sought to evaluate the impact of a complex fractionated atrial electrogram (CFAE)-guided ablation strategy on atrial fibrillation (AF) dynamics in patients with persistent AF.It is still unclear whether complete pulmonary vein isolation (PVI) is required or if the ablation of well-delineated pulmonary vein (PV) subregions could achieve similar outcomes in persistent AF.CFAE-guided ablations were performed in 76 patients (65.2 ± 10 years of age) with persistent AF. In 47 patients, we measured mean PVs and left atrial appendage (LAA) cycle length (CL) values (PV-CL and LAA-CL), before ablation and before AF termination. We defined "active" PVs as PV-CL ≤ LAA-CL, "rapid fires" as PV-CL ≤80% of LAA-CL, and "PV-LAA CL gradient" as a significant CL difference between the 2 regions.AF termination (sinus rhythm [SR] or atrial tachycardia [AT] conversion) occurred in 92% and SR conversion in 75%. The radiofrequency time for AF termination and total radiofrequency time were 26 ± 25 min and 61.1 ± 21.6 min, respectively. Thirty of 47 patients had active PV (with 19 PV "rapid fires"). Ablation significantly increased median CL, both at PVs and LAA from 188 ms (interquartile range [IQR]: 161 to 210 ms) to 227.5 ms (IQR: 200 to 256 ms) (p 0.0001) and from 197 ms (IQR: 168 to 220 ms) to 224 ms (IQR: 193 to 250 ms) (p 0001), respectively. After ablation, PV-LAA CL gradients were withdrawn and all PV "rapid fires" were extinguished (without PVI). After 17.2 ± 10 months of follow-up and 1.61 ± 0.75 procedures, 86.3% and 73% of the patients were free from AF and from any arrhythmia (AF/AT), respectively.CFAE-guided ablation leads to a large decrease in PV frequency of activation, preceding AF termination. A PV modulation approach, rather than complete PVI, may be preferable for persistent AF.

Introduction: Multi-electrode electrogram temporal activation and fractionation have been used to localize atrial fibrillation (AF) drivers. Yet, the feasibility of a simplified “online” analysis and its usefulness to ablate AF remain to be investigated. Hypothesis: Evaluating a simplified “online” electrogram analysis approach designed to localize and ablate AF drivers. Methods: 105 patients undergoing AF ablation (77% with persistent AF, 3 centres) were prospectively enrolled. AF was first mapped in both atria with the 20-pole contact electrode catheter PentaRay® (Biosense Webster, Inc.) in order to identify regions with a temporal activation pattern compatible with the presence of rapidly rotating rotors. Specifically, regions with consecutive activations spanning the majority of the AF cycle length (including examples of rapid and continuously fractionated electrograms) were visually selected (“hot zone”).The endpoints were AF termination acutely, and freedom from long-term AF (single procedure). Off-line, electrogram cycle length values were compared between “hot” and “non-hot” regions and a numerical simulation of PentaRay-enabled unipolar recordings of a stable rotor driving AF was conducted Results: Visually selected “hot” regions located mostly in the left atrium (6.5 ±4.2/patient; ~70% of ”hot” regions). “Hot” regions were activated at a significantly shorter cycle length than “non-hot” regions (171.8 ± 4.8 vs. 193.0 ± 6.3 ms, p=0.01, N=20). 95.6% of the “hot regions” were automatically labelled CFAEs (automated analysis, Carto). However, 4.5±3.4 distinct CFAEs regions/patient were also labelled in “non-hot” regions. Ablation at “hot” regions terminated AF in 99/104 patients (95.2%), 46 [21-72] min after the 1st radiofrequency application within 20 [10-37] min of RF. Also, numerical simulations show that a simplified PentaRay®-enabled activation analysis is sufficient to distinguish rotor and non-rotor regions. After 9+3 [6-11], months of median [Q1-Q3] follow-up, 87 % were free from AF after 1 procedure and 53 % still under antiarrhythmic drugs. Conclusions: With this simplified “online” analysis, we could successfully localize rotor regions and perform efficient AF ablation. Clinical trial.gov: NCT02093949

Enzyme replacement therapy consistently improves cardiac function in infantile and juvenile onset patients with Pompe disease and cardiomyopathy, but is apparently not effective in preventing rhythm disorders, an emerging cardiac phenotype in long term survivors. In patients with late onset Pompe disease cardiomyopathy is an exceptional finding while heart rhythm disorders seem to be more frequent. We retrospectively identified, among a cohort of 131 French late onset Pompe disease patients, four patients with severe atrio-ventricular blocks requiring pacemaker implantation. These patients had no other risk factors for cardiovascular diseases or cardiomyopathy. In one patient the atrioventricular block was discovered while still asymptomatic. Cardiac conduction defects are relatively rare in late onset Pompe disease and may occur even in absence of cardiac symptoms or EKG abnormalities. However because of the possible life-threatening complications associated with these conduction defects, cardiac follow-up in patients with late onset Pompe disease should include periodical Holter-EKG monitoring.