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Laminopathies and emerinopathies are rare disorders due to mutations in A-type lamins (LMNA) and emerin (EMD) genes. Among them, disorders affecting striated muscle and fat tissues are the most frequent, with cardiac disease being a major cause of death. Clinico-genetic spectrum of these diseases have been extensively described since the 1990's. Precise phenotype/genotype relations remain elusive but several clues have been identified. Apart from symptomatic treatments, first specific treatments to prevent or slow down disease progression are undergoing therapeutic trials. Since 2013, we developed opale, an approved multicenter web-based registry dedicated to laminopathies and emerinopathies French patients. The main inclusion criterion is the presence of a proven pathogenic (or likely pathogenic = ACMG class 4) LMNA or EMD gene mutation. OPALE's objectives are to provide detailed capture of patient genetic data, neuro-cardio-endocrinological and respiratory assessments, in order to allow i) precise characterization of disease natural history, ii) estimation of the prevalence of various complications and iii) identification of prognostic factors. Currently, opale gathers 28 centers including neurological and/or cardiological and/or endocrinological departments. A total of 766 patients (749 LMNA and 17 EMD mutated) have been already included and 230 additional patients (141 LMNA , 89 EMD) are currently screened for a future inclusion. The clinical spectrum of the included patients comprises 108 asymptomatic mutation carriers (102 LMNA , 6 EMD mutated), 232 with isolated cardiac disease (228 LMNA , 4 EMD mutated), 267 with myopathy with or without cardiac disease (260 LMNA , 7 EMD mutated), 88 LMNA mutated with lipodystrophy, 7 LMNA mutated with peripheral nerve disease, 16 LMNA mutated with progeria or progeroid phenotypes and 42 LMNA mutated with diverse muscle-heart-fat-nerve-progeroid overlapping features. The remaining patients are undergoing clinical characterization. We also set up a prospective quantitative muscle evaluation study to follow muscle involvement natural history and a serum biobanking to explore blood biomarkers. OPALE registry is currently the largest registry dedicated to laminopathies and emerinopathies. It allows a better understanding of the clinico-genetic spectrum. [ABSTRACT FROM AUTHOR]

Background and Aims: Prophylactic implantable cardioverter-defibrillators (ICDs) are not recommended until left ventricular ejection fraction (LVEF) has been reassessed 40 to 90 days after an acute myocardial infarction. In the current therapeutic era, the prognosis of sustained ventricular arrhythmias (VAs) occurring during this early post-infarction phase (i.e. within 3 months of hospital discharge) has not yet been specifically evaluated in post-myocardial infarction patients with impaired LVEF. Such was the aim of this retrospective study., Methods: Data analysis was based on a nationwide registry of 1032 consecutive patients with LVEF ≤ 35% after acute myocardial infarction who were implanted with an ICD after being prescribed a wearable cardioverter-defibrillator (WCD) for a period of 3 months upon discharge from hospital after the index infarction., Results: ICDs were implanted either because a sustained VA occurred while on WCD (VA+/WCD, n = 72) or because LVEF remained ≤35% at the end of the early post-infarction phase (VA-/WCD, n = 960). The median follow-up was 30.9 months. Sustained VAs occurred within 1 year after ICD implantation in 22.2% and 3.5% of VA+/WCD and VA-/WCD patients, respectively (P < .0001). The adjusted multivariable analysis showed that sustained VAs while on WCD independently predicted recurrence of sustained VAs at 1 year (adjusted hazard ratio [HR] 6.91; 95% confidence interval [CI] 3.73-12.81; P < .0001) and at the end of follow-up (adjusted HR 3.86; 95% CI 2.37-6.30; P < .0001) as well as 1-year mortality (adjusted HR 2.86; 95% CI 1.28-6.39; P = .012)., Conclusions: In patients with LVEF ≤ 35%, sustained VA during the early post-infarction phase is predictive of recurrent sustained VAs and 1-year mortality., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

LVAD implantation in patients with a recently diagnosed cardiomyopathy has been poorly investigated. This work aims at describing the characteristics and outcomes of patients receiving a LVAD within 30 days following the diagnosis of cardiomyopathy. Patients from the ASSIST-ICD study was divided into recently and remotely diagnosed cardiomyopathy based on the time from initial diagnosis of cardiomyopathy to LVAD implantation using the cut point of 30 days. The primary end point of the study was all-cause mortality at 30-day and during follow-up. A total of 652 patients were included and followed during a median time of 9.1 (2.5 to 22.1) months. In this population, 117 (17.9%) had a recently diagnosed cardiomyopathy and had LVAD implantation after a median time of 15.0 (9.0 to 24.0) days following the diagnosis. This group of patients was significantly younger, with more ischemic cardiomyopathy, more sudden cardiac arrest (SCA) events at the time of the diagnosis and were more likely to receive temporary mechanical support before LVAD compared with the remotely diagnosed group. Postoperative in-hospital survival was similar in groups, but recently diagnosed patients had a better long-term survival after hospital discharge. SCA before LVAD and any cardiac surgery combined with LVAD implantation were identified as 2 independent predictors of postoperative mortality in recently diagnosed patients. In conclusion, rescue LVAD implantation for recently diagnosed severe cardiomyopathy is common in clinical practice. Such patients experience a relatively low postoperative mortality and have a better long-term survival compared with remotely diagnosed patients., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Cryoballoon ablation is an effective procedure to treat atrial fibrillation (AF). However, AF recurrence rate at 1-year follow-up is approximately 20% despite improvements in ablation technique., Aim: To find factors predictive of AF recurrence following a first pulmonary vein isolation procedure using a second-generation cryoballoon (PVI-2CB)., Methods: This was an observational, retrospective, single-centre study. From June 2012 to April 2017, all patients who had a PVI-2CB procedure and a scheduled follow-up at Rouen University Hospital were included. The primary endpoint was atrial arrhythmia (AA) recurrence (e.g. AF, flutter or tachycardia), considering a blanking period of 2 months following the procedure. Secondary endpoints were procedural variables for each pulmonary vein (successful isolation, time to disconnection, total cryoballoon application time, number of cryoballoon applications, level of occlusion during cryoballoon application leading to successful disconnection and lowest temperature reached during successful cryoballoon application), occurrence of redo procedures, use of antiarrhythmic drugs and adverse events., Results: The initial population consisted of 239 patients; six were excluded for lack of procedural variable data, giving an analysed population of 233 patients. The AA recurrence rate was 36.9% (mean follow-up 25±14 months). Mean time to AA recurrence was 10±12 months. No procedural variable was found to be predictive of AA recurrence. Only major left atrial enlargement (defined as diameter>50mm or left atrial area>30cm 2 or left atrial volume>50mL/m 2 ) was predictive (odds ratio 2.70, 95% confidence interval 1.54-4.72; P=0.001). Forty-one patients had redo procedures (17.6% of analysed population); in this subgroup, 75.6% had at least one pulmonary vein reconnected, mainly the right inferior pulmonary vein., Conclusions: At long-term follow-up, up to one-third of patients had AA recurrence after PVI-2CB. Important atrial remodelling was the only factor predictive of AA recurrence, whereas no procedural variable was found to be predictive., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Background: Implantable cardioverter-defibrillator (ICD) lead dysfunction has been reported after left ventricular assist device (LVAD) implantation in limited single-center studies. We aimed at describing and characterizing the incidence of ICD lead parameters dysfunction after LVAD implantation., Methods: Among the 652 patients enrolled in the ASSIST-ICD study, only patients with an ICD prior to LVAD were included (n = 401). ICD lead parameters dysfunction following LVAD implantation is defined as follows: (a) >50% decrease in sensing threshold, (b) pacing lead impedance increase/decrease by >100Ω, and (c) >50% increase in pacing threshold., Results: One hundred twenty-two patients with an ICD prior to LVAD had available ICD interrogation reports prior and after LVAD. A total of 67 (55%) patients exhibited at least one significant lead dysfunction: 17 (15%) exhibited >50% decrease in right ventricular (RV) sensing, 51 (42%) had >100 Ω increase/decrease in RV pacing impedance, and 24 (20%) experienced >50% increase in RV pacing threshold. A total of 52 patients experienced ventricular arrhythmia during follow-up and all were successfully detected and treated by the device. All lead dysfunction could be managed conservatively., Conclusion: More than 50% of LVAD-recipients may experience >1 significant change in lead parameters but none had severe clinical consequences., (© 2020 Wiley Periodicals LLC.)

Background: Left ventricular assist device (LVAD) implantation may be an attractive alternative therapeutic option for elderly patients with heart failure who are ineligible for heart transplantation., Aim: We aimed to describe the characteristics and outcomes of elderly patients (i.e. aged≥70 years) receiving an LVAD., Methods: This observational study was conducted in 19 centres between 2006 and 2016. Patients were divided into two groups-younger (aged<70 years) and elderly (aged≥70 years), based on age at time of LVAD implantation., Results: A total of 652 patients were included in the final analysis, and 74 patients (11.3%) were aged≥70 years at the time of LVAD implantation (maximal age 77.6 years). The proportion of elderly patients receiving an LVAD each year was constant, with a median of 10.6% (interquartile range 8.0-15.4%) per year, and all were implanted as destination therapy. Elderly and younger patients had similar durations of hospitalization in intensive care units and total lengths of hospital stays. Both age groups experienced similar rates of LVAD-related complications (i.e. stroke, bleeding, driveline infection and LVAD exchange), and the occurrence of LVAD complications did not impact survival in the elderly group compared with the younger group. Lastly, when compared with younger patients implanted as destination therapy, the elderly group also exhibited similar mid-term survival., Conclusion: This work strongly suggests that selected elderly adults can be scheduled for LVAD implantation., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objectives: Our goal was to provide a picture of left ventricular assist device (LVAD) activity in France between 2007 and 2016 based on the multicentric ASSIST-ICD registry., Methods: We retrospectively collected 136 variables including in-hospital data, follow-up survival rates and adverse events from 671 LVAD recipients at 20 out of 24 LVAD implant centres in France. The average follow-up time was 1.2 years (standard deviation: 1.4); the total follow-up time was 807.5 patient-years., Results: The included devices were the HeartMate II®, HeartWare LVAS® or Jarvik 2000®. The overall likelihood of being alive while on LVAD support or having a transplant (primary end point) at 1, 2, 3 and 5 years postimplantation was 65.2%, 59.7%, 55.9% and 47.7%, respectively, given a cumulative incidence of 29.2% of receiving a transplant at year 5. At implantation, 21.5% of patients were on extracorporeal life support. The overall rate of cardiogenic shock at implantation was 53%. The major complications were driveline infection (26.1%), pump pocket or cannula infection (12.6%), LVAD thrombosis (12.2%), ischaemic (12.8%) or haemorrhagic stroke (5.4%; all strokes 18.2%), non-cerebral haemorrhage (9.1%) and LVAD exchange (5.2%). The primary end point (survival) was stratified by age at surgery and by the type of device used, with inference from baseline profiles. The primary end point combined with an absence of complications (secondary end point) was also stratified by device type., Conclusions: The ASSIST-ICD registry provides a real-life picture of LVAD use in 20 of the 24 implant centres in France. Despite older average age and a higher proportion of patients chosen for destination therapy, survival rates improved compared to those in previous national registry results. This LVAD registry contrasts with other international registries because patients with implants have more severe disease, and the national policy for graft attribution is distinct. We recommend referring patients for LVAD earlier and suggest a discussion of the optimal timing of a transplant for bridged patients (more dismal results after the second year of support?)., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The introduction of catheter ablation techniques has vastly improved the treatment of cardiac arrhythmias. However, as complex ablations are technically demanding and can cause various complications, they require a high level of expertise to maximize success rates and minimize complication rates. As French recommendations regarding the required technical competence and equipment are not yet available, this position paper has been compiled by the Working Group of Pacing and Electrophysiology of the French Society of Cardiology, detailing the required features of an interventional cardiac electrophysiological centre for complex ablation procedures: (1) sufficient institutional volume; (2) physician training, qualifications and experience; (3) paramedical staff training and attendance; and (4) institutional facilities and technical equipment. The importance of being able to diagnose, monitor and manage complications associated with complex ablations is highlighted. Supplemental hospital-based resources are also discussed, such as anaesthesia, surgical back-up, intensive care, haemodynamic assistance and imaging. Further, the ideal features of an interventional cardiac electrophysiology training centre are considered. Lastly, the need for quality evaluations and national registries for complex ablations is discussed., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: The role of implantable loop recorders (ILRs) in the evaluation strategy for recurrent syncope in France is limited by lack of knowledge of the cost., Aim: To compare a conventional evaluation strategy for syncope with the early use of an ILR in low-risk patients, in terms of diagnostic yield, cost and impact on quality of life (QoL)., Methods: National prospective randomized open-label multicenter study of patients with a single syncope (if severe and recent) or at least two syncopes in the past year., Results: Seventy-eight patients (32 men) were randomized to the ILR strategy (ILR group, n=39) or the conventional evaluation strategy (CONV group, n=39): mean age 66.2±14.8 years; 4.3±6.4 previous syncopes. After 14 months of follow-up, a certain cause of syncope was established in 18 (46.2%) patients in the ILR group and two (5%) patients in the CONV group (P<0.001). Advanced cardiological tests were performed less frequently in the ILR group than in the CONV group (0.03±0.2 vs. 0.2±0.5 tests per patient; P=0.05). Patients in the ILR group were hospitalized for a non-significantly shorter period than patients in the CONV group (5.7±3.2 vs. 8.0±1.4 days). There was no difference between the two groups in terms of QoL main composite score., Conclusion: In patients with unexplained syncope, the early use of an ILR has a superior diagnostic yield compared with the conventional evaluation strategy, with lower healthcare-related costs., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Introduction: The study was designed to evaluate the feasibility and performance of right ventricular (RV) mid-septal versus apical implantable defibrillator (ICD) lead placement., Methods and Results: SEPTAL is a randomized, noninferiority trial, which randomly assigned patients to implantation of ICD leads in the RV mid-septum versus apex, with a primary objective of comparing the implant success rate of implant at each site, based on strict electrical predefined criteria. We also compared the (1) pacing lead characteristics, (2) rates of appropriate and inappropriate ICD therapies, and (3) all-cause mortality between the 2 sites at 1 year. The trial enrolled 215 patients (mean age = 59.7 ± 12.4 years, mean LVEF = 34.0 ± 14.2%, 84.2% men), of whom 148 (68.8%) presented with ischemic heart disease. The ICD indication was primary prevention in 117 patients (54.4%). The lead was successfully implanted in 96/107 patients (89.7%) assigned to the RV mid-septum, and in 99/108 (91.7%) assigned to the apex (ns). The 1-year rate of lead-related adverse events was similar in both groups. A total of 8 first inappropriate ICD therapies (7.9%) were delivered in the RV mid-septal group, versus 8 (7.8%) in the apical group (ns), while first appropriate therapies were delivered to 22 (21.4%) and 24 patients (23.8%), respectively (ns). All-cause mortality was 7.9% in the RV mid-septal versus 2.9% in the RV apical group (ns)., Conclusion: This study confirmed the technical feasibility and noninferior performance of ICD leads implanted in the RV mid-septum versus the apex., (© 2012 Wiley Periodicals, Inc.)

Introduction: Various types of complications have been reported after atrial fibrillation (AF) ablation using radiofrequency energy, but those have not been well defined when using cryoballoon ablation technique. The objective of this prospective study was to assess types, incidence, and outcome of complications after cryoballoon pulmonary vein isolation (PVI)., Methods and Results: This prospective monocentric study included 66 consecutive patients (39 males, age 57 ± 11 years) who underwent cryoballoon PVI for symptomatic paroxysmal AF. Phrenic nerve (PN) integrity was assessed by pacing from the superior vena cava during isolation of the right PVs. Before discharge, all patients were subjected to 24-hour Holter electrocardiograms, echocardiography, and esophagogastroduodenoscopy. Cardiac MRI was scheduled between 1 and 3 months postprocedure. At 3.7 ± 1.7 months after ablation, patients underwent clinical review and 24-hour Holter electrocardiograms. The mean number of cryoballoon applications was 10.0 ± 2.1 per patient and 2.5 ± 1.0 per vein. A 28 mm cryoballoon was used in 49 patients (74%) and a 23 mm cryoballoon in the remaining 17 patients (26%). Twelve complications (18%) attributing to collateral nervous damage were noticed in 9 patients: asymptomatic gastroparesis was observed in 6 patients (9%), transient PN palsy (PNP) in 5 (8%), and symptomatic inappropriate sinus tachycardia requiring beta-blocker treatment in 1 (1%). Neither cryoballoon-related esophageal ulceration nor PV stenosis was observed., Conclusion: Gastroparesis and PNP could be observed in a significant number of cases after cryoballoon ablation of AF. These complications are likely due to cryo-induced damages to nervous structures surrounding the heart., (© 2012 Wiley Periodicals, Inc.)