Predictive Value of Right Ventricular Sensing During Cardiac Device Implantation to Diagnose Cardiac Amyloidosis.

Background: Cardiac amyloidosis (CA) is an underdiagnosed disease, often presenting with electrical conduction abnormalities or ventricular arrhythmias, prompting implantation of cardiac implantable electrical devices (CIEDs).

Objectives: This study sought to evaluate the performance of right ventricular sensing (RVSens) during implantation of CIEDs in patients presenting with increased left ventricular (LV) wall thicknesses (ILVWT) and preserved LV function to predict the diagnosis of CA.

Methods: All consecutive patients with ILVWT (maximal LV wall thickness ≥12 mm), preserved LV function and no significant aortic stenosis who underwent implantation of CIEDs at 2 French referral centers for rare and inheritable cardiac diseases were retrospectively evaluated.

Clinical course of arrhythmogenic right ventricular cardiomyopathy with end-stage heart failure and outcome after heart transplantation.

Background: Few data exist on the characteristics and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy and advanced heart failure who undergo heart transplantation.

Aim: To explore the pretransplant course and outcomes of patients with arrhythmogenic right ventricular cardiomyopathy after heart transplantation.

Methods: This observational retrospective monocentric study included all consecutive patients with arrhythmogenic right ventricular cardiomyopathy who underwent heart transplantation during a 13-year period (2006-2019) at Pitié-Salpêtrière University Hospital (Paris).

Evaluation of an Ambulatory ECG Analysis Platform Using Deep Neural Networks in Routine Clinical Practice.

Background Holter analysis requires significant clinical resources to achieve a high-quality diagnosis. This study sought to assess whether an artificial intelligence (AI)-based Holter analysis platform using deep neural networks is noninferior to a conventional one used in clinical routine in detecting a major rhythm abnormality. Methods and Results A total of 1000 Holter (24-hour) recordings were collected from 3 tertiary hospitals.

Safety and efficacy of flecainide associated with beta-blockers in arrhythmogenic right ventricular cardiomyopathy.

Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy associated with a high risk of ventricular arrhythmia (VA). Current guidelines recommend beta-blockers as first-line medical therapy and if ineffective, sotalol or amiodarone. We describe our experience, as a tertiary centre for ARVC, with the effectiveness and tolerance of flecainide in addition to beta-blockers to prevent VA in ARVC.

External Validation of a Risk Prediction Model for Ventricular Arrhythmias in Arrhythmogenic Right Ventricular Cardiomyopathy.

The new 5-year ventricular arrhythmia (VA) occurrence risk model is a major breakthrough for arrhythmia risk stratification in the challenging population of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). In the original study, the model resulted in a 20.6% reduction in implantable cardioverter-defibrillator (ICD) placement compared with the 2015 consensus, for the same protection level.

Evolution of high-grade atrioventricular conduction disorders after transcatheter aortic valve implantation in patients who underwent implantation of a pacemaker with specific mode-that minimizes ventricular pacing-activated.

Introduction: The evolution of atrioventricular conduction disorders after transcatheter aortic valve implantation (TAVI) remains poorly understood. We sought to identify factors associated with late (occurring ≥7 days after the procedure) high-grade atrioventricular blocks after TAVI, based on specific pacemaker memory data.

Methods And Results: STIM-TAVI (NCT03338582) was a prospective, multicentre, observational study that enrolled all patients (from November 2015 to January 2017) implanted with a specific dual chamber pacemaker after TAVI, with the SafeR algorithm activated, allowing continuous monitoring of atrioventricular conduction.

Significance of NT-proBNP and High-sensitivity Troponin in Friedreich Ataxia.

Background: Friedreich's ataxia (FA) is a rare autosomal recessive mitochondrial disease resulting of a triplet repeat expansion guanine-adenine-adenine (GAA) in the frataxin (FXN) gene, exhibiting progressive cerebellar ataxia, diabetes and cardiomyopathy. We aimed to determine the relationship between cardiac biomarkers, serum N-terminal pro-brain natriuretic peptide (NT-proBNP), and serum cardiac high-sensitivity troponin (hsTnT) concentrations, and the extent of genetic abnormality and cardiac parameters.

Methods: Between 2013 and 2015, 85 consecutive genetically confirmed FA adult patients were prospectively evaluated by measuring plasma hsTnT and NT-proBNP concentrations, electrocardiogram, and echocardiography.

Psychosocial Impact of Predictive Genetic Testing in Hereditary Heart Diseases: The PREDICT Study.

Predictive genetic testing (PGT) is offered to asymptomatic relatives at risk of hereditary heart disease, but the impact of result disclosure has been little studied. We evaluated the psychosocial impacts of PGT in hereditary heart disease, using self-report questionnaires (including the State-Trait Anxiety Inventory) in 517 adults, administered three times to the prospective cohort (PCo: = 264) and once to the retrospective cohort (RCo: = 253). The main motivations for undergoing PGT were "to remove doubt" and "for their children".

Catheter ablation of electrical storm in patients with arrhythmogenic right ventricular cardiomyopathy.

Background: Therapeutic strategies for electrical storm (ES) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well defined.

Objective: The purpose of this study was to report the acute and long-term results of ventricular tachycardia (VT) radiofrequency catheter ablation (RFCA) as a treatment of ES in patients with ARVC.

Methods: This multicenter study retrospectively enrolled 23 consecutive patients with ARVC (mean age 43.

Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Without an Implantable Cardioverter-Defibrillator.

Objectives: The purpose of this study was to identify clinical factors associated with arrhythmic events and sudden cardiac death (SCD), and to evaluate the prognostic value of electrophysiological study (EPS) in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients without implantable cardioverter-defibrillators (ICDs).

Background: ARVC/D is an inherited cardiomyopathy characterized by a risk of SCD. Few studies have evaluated predictive factors of ventricular arrhythmias (VAs) in patients without ICDs.

Pregnancy and newborn outcomes in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

Introduction: The prognosis of pregnancy in patients with Arrhythmogenic Right Ventricular Cardiomyopathy/dysplasia (ARVC/D) is poorly documented. The aim of this study is to assess the cardiac risks during pregnancy and the impact of ARVC/D on fetuses/neonates/children.

Methods: We included all ARVC/D women with a history of pregnancy from the ARVC/D Pitié-Salpêtrière registry.

Should We Perform an Immediate Coronary Angiogram in All Patients After Cardiac Arrest?: Insights From a Large French Registry.

Objectives: This study sought to assess the relationship between an immediate invasive strategy and survival after an out-of-hospital cardiac arrest (OHCA) of presumed cardiac cause, according to prognosis evaluated on hospital arrival.

Background: An immediate coronary angiogram (CAG) may be associated with better outcome after OHCA in neurologically preserved patients but could be futile in other cases.

Methods: From May 2011 to May 2015, we collected data for all patients admitted in hospital after OHCA in Paris and its suburbs (France).

The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology: baseline data and contemporary management of adult patients with cardiomyopathies.

Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry.

Methods And Results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)].

Catheter ablation of organized atrial arrhythmias in orthotopic heart transplantation.

Background: Organized atrial arrhythmias (OAAs) are common after orthotopic heart transplantation (OHT). Some controversies remain about their clinical presentation, relationship with atrial anastomosis and electrophysiologic features. The objectives of this retrospective study were to determine the mechanisms of OAAs after OHT and describe the outcomes of radiofrequency catheter ablation (RFCA).

Shortening of the Short Refractory Periods in Short QT Syndrome.

Background: Diagnosis of short QT syndrome (SQTS) remains difficult in case of borderline QT values as often found in normal populations. Whether some shortening of refractory periods (RP) may help in differentiating SQTS from normal subjects is unknown.

Methods And Results: Atrial and right ventricular RP at the apex and right ventricular outflow tract as determined during standard electrophysiological study were compared between 16 SQTS patients (QTc 324±24 ms) and 15 controls with similar clinical characteristics (QTc 417±32 ms).

Respective role of surface electrocardiogram and His bundle recordings to assess the risk of atrioventricular block after transcatheter aortic valve replacement.

Background: Atrioventricular block (AVB) is common after transcatheter aortic valve replacement (TAVR) and permanent pacemaker (PPM) implantation is needed in up to 30% of patients. Main predictors of long term AVB are electrocardiographic. The purpose of this study is to assess the prognostic value of serial HV intervals measured before and after TAVR to shorten the timing of PPM implantation.

Thrombus composition in sudden cardiac death from acute myocardial infarction.

Background And Aim: It was hypothesized that the pattern of coronary occlusion (thrombus composition) might contribute to the onset of ventricular arrhythmia and sudden cardiac death (SCD) in myocardial infarction (MI).

Methods: The TIDE (Thrombus and Inflammation in sudden DEath) study included patients with angiographically-proven acute coronary occlusion as the cause of a ST elevation MI (STEMI) complicated by Sudden Cardiac Death (SCD group) or not (STEMI group). Thrombi were obtained by thrombo-aspiration before primary percutaneous coronary stenting and analyzed with a quantitative method using scanning electron microscopy.

Post-cardiac arrest shock treated with veno-arterial extracorporeal membrane oxygenation: An observational study and propensity-score analysis.

Purpose: Cardiogenic shock due to post-resuscitation myocardial dysfunction is a major cause of mortality among patients hospitalized after cardiac arrest (CA). Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) has been proposed in the most severe cases but the level of evidence is very low. We assessed characteristics, outcome and prognostic factors of patients treated with VA-ECMO for post-CA shock.

Implantable cardioverter-defibrillators in end-stage heart failure patients listed for heart transplantation: Results from a large retrospective registry.

Background: Implantable cardioverter-defibrillators (ICDs) are recommended in patients with low ejection fraction. However, the survival benefit of ICDs in patients with end-stage heart failure listed for heart transplantation is unclear.

Aim: To evaluate the ICD benefit on mortality in this population.

The CAHP (Cardiac Arrest Hospital Prognosis) score: a tool for risk stratification after out-of-hospital cardiac arrest.

Aims: Survival after out-of-hospital cardiac arrest (OHCA) remains disappointingly low. Among patients admitted alive, early prognostication remains challenging. This study aims to establish a stratification score for patients admitted in intensive care unit (ICU) after OHCA, according to their neurological outcome.

Synovial sarcoma relapses in children and adolescents: prognostic factors, treatment, and outcome.

Introduction: Twenty-five to 32% of patients with synovial sarcoma (SS) relapse after appropriate treatment, and experience a poor outcome. Patients who can be salvaged by second-line therapy need to be more clearly identified.

Patients And Methods: Data of patients treated in SFCE (Société Française des Cancers de l'Enfant) centers with an initial diagnosis of localized SS before the age of 18 years and treated from 1/1988 to 12/2008, and who experienced at least one relapse, were retrieved.