Background LMNA gene-related dilated cardiomyopathy (DCM), or laminopathy, leads to severe heart failure, neuromuscular involvement and ventricular arrhythmia and has been described as the most frequent DCM genetic cause requiring heart transplantation (HTx). However, no study has specifically evaluated outcomes of HTx in patients with laminopathy. We aimed to describe and evaluate the clinical outcomes of this specific population. Methods Data from all consecutive patients with laminopathy who underwent HTx (LMNA-HTx) between 2003 and 2024 in three French centres were retrospectively extracted. Patients in the LMNA-HTx group were compared with patients with matched non-ischaemic DCM who underwent HTx (DCM-HTx) using propensity score matching. The primary endpoints were 1 year and long-term post-HTx survival. Secondary objectives were to describe mortality risk factors and assess the progression and impact of neuromuscular symptoms after HTx. Results 40 patients with laminopathy underwent HTx (median age 45.4 years, 57.5% males). HTx followed electrical storm (ES) in 33% of cases. Post-HTx survival at 3 months, 1 year and 5 years was 90%, 74% and 70%, respectively, comparable to 160 matched patients in the DCM-HTx group (Kaplan-Meier survival analysis: HR: 1.01; 95% CI 0.52 to 1.95; p=0.98). The occurrence of ES in the days preceding HTx emerged as the only factor associated with mortality (Kaplan-Meier survival analysis: HR: 8.46; 95% CI 1.66 to 43.17; p=0.002). Neuromuscular and extracardiac involvement was not associated with mortality and did not worsen after HTx. Conclusions In this first systematic evaluation of HTx in patients with laminopathy, we show that post-transplant survival was comparable between LMNA-HTx and matched DCM-HTx patients and was not associated with neuromuscular involvement. In contrast, ES occurred in one-third of patients prior to HTx and was associated with mortality. These findings support considering HTx early in the disease course, particularly in the setting of arrhythmogenic instability, while mild neuromuscular involvement should not be viewed as a contraindication.

Background: Cardiovascular diseases became the first cause of maternal death in France, but only scarce epidemiological data have been published about heart failure (HF) in pregnancy/postpartum. Objective: The aim of the present study was to describe the epidemiology of HF in pregnancy or until 6 weeks postpartum in France. Study Design: This is an observational cohort study. Using the French National Health Insurance Information System database (Système National des Données de Santé), all women who gave birth in France after 22 weeks of gestation between January 1, 2010, and December 31, 2018, were selected. HF hospitalizations occurring during the pregnancies or 6 weeks postpartum were identified. Results: On 6293,367 deliveries analyzed, 2241 (35.6/100,000) had at least one hospitalization for HF. This rate remained quite stable between 2010 and 2018. A total of 22.6% of these cases had heart disease history, including HF before pregnancy, and 49.0% had preceding or concomitant heart disease. Compared to women without HF, those with HF were older (32.0 [6.1] vs 29.9 [5.3] years), had more frequently multiple pregnancy (14.6% vs 1.7%), history of medically assisted reproduction (7.9% vs 3.2%), cardiovascular risk factors (arterial hypertension [17.0% vs 1.7%], gestational diabetes [15.5% vs 9.6%], obesity [14.9% vs 4.7%], tobacco smoking [12.4% vs 9.4%]), and had a lower socio-economic level with 23.4% living in the French most deprived area against 18.1% living in the least deprived area. The most frequent cardiac condition found were cardiomyopathy (29.5%), arrhythmias (14.0%), and valvulopathy (8.5%). The rate of pre-eclampsia reached 29.4% in pregnancies with HF against 2.0% in pregnancies without HF. Fetal complications were much higher in pregnancies with HF than without HF, with 11.5% vs 0.9% very/extremely preterm birth, and 5.3% vs 0.8% fetus/child death. One year after delivery, maternal death was observed in 2.2% of women with HF against <0.01% in those without HF. Conclusion: Despite occurring in only 1 in 2808 pregnancies, HF during pregnancy or the postpartum period has a significant impact on maternal and fetal outcomes.

BACKGROUND:Heart failure (HF) prevalence may increase because of population ageing and has become a major public health issue in European countries. AIM:To update the epidemiology of HF in France in 2022. METHODS:Adults hospitalized for HF in 2022 were identified in the National Health Data System (SNDS) and followed up for 1year. The first stay of the year was taken as the index hospitalization. The prevalence of HF was estimated by combining hospitalization data and patients with 100% coverage for a long-term disease associated with HF. Patients and their hospital stays were described on the basis of the sociodemographic and medical information in the SNDS. RESULTS:In 2022, 181,178 adults were hospitalized for HF in France, which equates to a crude rate of 339.3 per 100,000 inhabitants, and 1,376,692 prevalent cases of HF were recorded, which is an estimated prevalence of 2.6% in the adult population. For people living in the most socioeconomically deprived municipalities, the rate of hospitalization was 1.6 times higher than for those living in the least deprived municipalities. The departments of Haut-de-France and Réunion Island, and some departments in Normandy and the Grand-Est had much higher rates than others. The fatality rate was 10.2% in hospital, and 34.0% at 1year. Only 20.1% of patients were admitted to a rehabilitation unit within 6months, and 47.9% of patients alive at 1year were being treated with a combination of angiotensin-converting enzyme inhibitors/angiotensin II receptor blockers and beta-blockers. CONCLUSIONS:The large number of people hospitalized for HF, and the fact that rates vary across the different French departments, means that more ambitious general cardiovascular prevention measures are needed, and that healthcare provision needs significant adaptation. Short-term patient outcomes could be improved by following recommendations more closely and taking into account patients' social circumstances.

Abstract Aims Inherited cardiomyopathies are relatively rare but carry a high risk of cardiac maternal morbidity and mortality during pregnancy and postpartum. However, data for risk stratification are scarce. The new CARPREG II score improves prediction of prognosis in pregnancies associated with heart disease, though its role in inherited cardiomyopathies is unclear. We aim to describe characteristics and cardiac maternal outcomes in patients with inherited cardiomyopathy during pregnancy, and to evaluate the interest of the CARPREG II risk score in this population. Methods and results In this retrospective single‐centre study, 90 consecutive pregnancies in 74 patients were included (mean age 32 ± 5 years), including 28 cases of dilated cardiomyopathy (DCM), 46 of hypertrophic cardiomyopathy, 11 of arrhythmogenic right ventricular cardiomyopathy and 5 of left ventricular noncompaction, excluding peripartum cardiomyopathy. The discriminatory power of several risk scores was assessed by the area under the receiver‐operating characteristic curve (AUC). Median CARPREG II score was 2 [0;3] and was higher in the DCM subgroup. A severe cardiac maternal complication was observed in 18 (20%) pregnancies, mainly driven by arrhythmia and heart failure (each event in 10 pregnancies), with 3 cardiovascular deaths. Forty‐three pregnancies (48%) presented foetal/neonatal complications (18 premature delivery, 3 foetal/neonatal death). CARPREG II was significantly associated with cardiac maternal complications (P < 0.05 for all) and showed a higher AUC (0.782) than CARPREG (0.755), mWHO (0.697) and ZAHARA (0.604). Conclusions Pregnancy in women with inherited cardiomyopathy carries a high risk of maternal cardiovascular complications. CARPREG II is the most efficient predictor of cardiovascular complications in this population.

AbstractAimsWe aim to describe the incidence of HF hospitalization in France in the post‐pandemic era, the prevalence of HF cases and patients' characteristics, management and outcomes while focusing on sex, age and socio‐economic differences and to analyse time‐trends between 2012 and 2022.Methods and resultsBased on the French health care database providing medical information for almost the whole French population, patients hospitalized for acute decompensated HF without history of HF in the 5 years were identified by the International Classification of Diseases – 10th revision (ICD‐10) codes. In 2022, the estimated prevalence of HF was 1.7% in France and has increased until the COVID‐19 pandemic and decreased thereafter. The incidence of acute HF decompensation reached 201.4 per 100 000 inhabitants and has decreased since 2012 (−1% per year). A significant increase of the HF incidence was found in men aged <45 years. Women aged <65 years were less likely to be admitted in a cardiac rehabilitation (CR) unit and had higher probability of one‐year mortality compared with men of the same age. One‐year mortality was significantly increased in patients from the most deprived area among extreme age group only (under 65 and ≥85 years). One‐year rehospitalization rates have decreased significantly, particularly in men aged <75 years. A decrease in ACE/ARBs deliveries was observed in both men and women.ConclusionsDespite the decrease in acute HF decompensation incidence and improvements in the management, the prevalence of HF remains stable in France and prognosis remains poor.

L’insufficienza cardiaca è una malattia la cui frequenza è in aumento a causa dell’invecchiamento della popolazione e del miglioramento nella gestione delle cardiopatie, la maggior parte delle quali può portare all’insufficienza cardiaca. La diagnosi della malattia è clinica ed è confermata da semplici esami non invasivi: ecocardiografia e peptidi natriuretici. L’insufficienza cardiaca con funzione sistolica conservata rappresenta quasi la metà delle insufficienze cardiache congestizie, ma il suo trattamento rimane scarsamente codificato, a differenza dell’insufficienza cardiaca con funzione sistolica compromessa, per la quale le raccomandazioni terapeutiche sono robuste. La prognosi della malattia resta grave e i ricoveri ospedalieri sono frequenti. Il medico curante svolge un ruolo importante nella gestione e nel monitoraggio dei pazienti con insufficienza cardiaca cronica.

Abstract Backgrounds Pulmonary congestion assessed by B-lines using lung ultrasound (LUS) is a hallmark of heart failure (HF). Latent HF at rest can be unmasked during exercise but the diagnosis remains challenging. Exercise-induced B-lines could help diagnose HF with preserved left ventricular ejection fraction (LVEF) at an early stage. The patients in whom coronary artery disease is suspected are also at risk of HF. Objectives To assess exercise-induced pulmonary congestion in patients referred to rule out myocardial ischemia. Methods Data of stress echocardiography combined with LUS at rest and immediately after exercise in patients with LVEF≥50% referred for investigation of inducible myocardial ischemia in a tertiary center of cardiology, during a 3-years period of time, were retrospectively analyzed. Patients with induced myocardial ischemia, hypertrophic cardiomyopathy or moderate to severe valvular disease were excluded. B-lines were assessed by scanning 2 chest sites. Exercise-induced pulmonary congestion was defined as an increase of B-lines ≥ 2 between baseline and exercise. Results A total of 1114 patients were included. Mean age was 63±11 years, 54% of patients had hypertension and 27% diabetes. Exercise-induced pulmonary congestion was identified in 131 (12%) patients. Age, left atrial volume index (LAVi), resting and 20W septal E/e’>15 and peak tricuspid regurgitation velocity (TRV) were associated with exercise-induced B-lines. In a multivariate logistic regression analysis, LAVi (Odds ratio (OR) = 1.03; 95%confidence interval (CI): 1.01–1.06; p = 0.003) and peak TRV (OR = 3.8; 95%CI: 1.4–10.1; p = 0.009) were independent predictors of exercise-induced pulmonary congestion. Conclusion Exercise-induced pulmonary congestion could be diagnosed in patients with preserved LVEF referred to rule out inducible myocardial ischemia. Exercise B-lines are linked with left ventricular diastolic dysfunction indices.

Lung ultrasound (LUS) can detect pulmonary congestion assessed by B-lines in heart failure patients undergoing stress test. Exercise B-lines could help diagnose heart failure with preserved left ventricular ejection fraction (LVEF) at an early stage. This study aimed to assess exercise-induced pulmonary congestion in patients with preserved LVEF referred for exercise echocardiography to rule out myocardial ischemia. Data of stress echocardiography combined with LUS at rest and immediately after exercise in patients with LVEF ≥ 50% referred for investigation of inducible myocardial ischemia in a tertiary center of cardiology were retrospectively analyzed. B-lines were assessed by scanning 2 chest sites. Exercise-induced pulmonary congestion was defined as an increase of B-lines ≥ 2 between baseline and exercise. A total of 1114 patients were included. Mean age was 63 years, 54% of patients had hypertension and 27% had diabetes. Exercise-induced pulmonary congestion was identified in 131 (12%) patients. Age, left atrial volume index (LAVi), resting and 20 W septal E/e' > 15 and peak tricuspid regurgitation velocity (TRV) were associated with increased exercise-induced B-lines. At multivariable analysis, LAVi (OR = 1.03; 95% confidence interval (CI): 1.01–1.06; P = 0.003) and peak TRV (OR = 3.8; 95%CI: 1.4–10.1; P = 0.009) were independent predictors of exercise-induced pulmonary congestion. Exercise-induced pulmonary congestion could be diagnosed in patients with preserved LVEF referred to rule out inducible myocardial ischemia. Exercise B-lines are linked with left ventricular diastolic dysfunction indices.

Background. - Friedreich's ataxia is an autosomal recessive mitochondrial disease caused by a triplet repeat expansion in the frataxin gene (FXN), exhibiting cerebellar sensory ataxia, diabetes and cardiomyopathy. Cardiac complications are the major cause of early death. Aims. - To characterize the cardiac phenotype associated with Friedreich's ataxia, and to assess the evolution of the associated cardiopathy over 1 year. Methods. - This observational single-centre open label study consisted of two groups: 20 subjects with Friedreich's ataxia and 20 healthy controls studied over two visits over 1 year. All subjects had transthoracic echocardiography, cardiac magnetic resonance imaging, cardiopulmonary exercise testing, quantification of serum cardiac biomarkers and neurological assessment. Results. - Patients with Friedreich's ataxia had left ventricular hypertrophy, with significantly smaller left ventricular diastolic diameters and volumes and increased wall thicknesses. Cardiac magnetic resonance imaging demonstrated significant concentric left ventricular remodelling, according to the mass/volume ratio, and focal myocardial fibrosis in 50% of patients with Friedreich's ataxia. Cardiopulmonary exercise testing showed alteration of left ventricular diastolic filling in patients with Friedreich's ataxia, with an elevated VE/VCO2 slope (ventilatory flow/exhaled volume of carbon dioxide). High-sensitivity troponin T plasma concentrations were higher in subjects with Friedreich's ataxia. None of the previous variables changed at 1 year. Neurological assessments remained stable for both groups, except for the nine-hole pegboard test, which was altered over 1 year. Conclusions. - The multivariable characterization of the cardiac phenotype of patients with Friedreich's ataxia was significantly different from controls at baseline. Over 1 year there were no clinically significant changes in patients with Friedreich's ataxia compared with healthy controls, whereas the neurological severity score increased modestly. (C) 2021 Elsevier Masson SAS. All rights reserved.

Background and Objectives:Friedreich ataxia (FRDA) is an autosomal recessive ataxia with no approved treatments. Leriglitazone is a selective peroxisome proliferator-activated receptor γ agonist that crosses the blood-brain barrier and, in preclinical models, improved mitochondrial function and energy production. We assessed effects of leriglitazone in patients with FRDA in a proof-of-concept study. Methods:In this double-blind, randomized controlled trial, eligible participants (age 12-60 years) had genetically confirmed FRDA, a Scale for the Assessment and Rating of Ataxia (SARA) total score <25, and a SARA item 1 score of 2-6, inclusive. Key exclusion criteria were age at FRDA onset ≥25 years and history of cardiac dysfunction. Participants were randomly assigned (2:1) to receive a daily, oral, individualized dose of leriglitazone or placebo for 48 weeks. The primary endpoint was the change from baseline to week 48 in spinal cord area (C2-C3) (measured by MRI). Secondary endpoints included the change from baseline to week 48 in iron accumulation in the dentate nucleus (quantitative susceptibility mapping) and total N-acetylaspartate to myo-inositol (tNAA/mIns) ratio. Results:Overall, 39 patients were enrolled (mean age 24 years; 43.6% women; mean time since symptom onset 10.5 years): 26 patients received leriglitazone (20 completed) and 13 received placebo (12 completed). There was no difference between groups in spinal cord area from baseline to week 48 (least-squares [LS] mean change [standard error (SE)]: leriglitazone, -0.39 [0.55] mm2; placebo, 0.08 [0.72] mm2; p = 0.61). Iron accumulation in the dentate nucleus was greater with placebo (LS mean change [SE]: leriglitazone, 0.10 [1.33] ppb; placebo, 4.86 [1.84] ppb; p = 0.05), and a numerical difference was seen in tNAA/mIns ratio (LS mean change [SE]: leriglitazone, 0.03 [0.02]; placebo, -0.02 [0.03]; p = 0.25). The most frequent adverse event was peripheral edema (leriglitazone 73.1%, placebo 0%). Discussion:The primary endpoint of change in spinal cord area was not met. Secondary endpoints provide evidence supporting proof of concept for leriglitazone mode of action and, with acceptable safety data, support larger studies in patients with FRDA. Trial Registration Information:ClinicalTrials.gov: NCT03917225; EudraCT: 2018-004405-64; submitted April 17, 2019; first patient enrolled April 2, 2019. clinicaltrials.gov/ct2/show/NCT03917225?term=NCT03917225&draw=2&rank=1. Classification of Evidence:This study provides Class I evidence that individualized dosing of leriglitazone, compared with placebo, is not associated with changes in spinal cord area in patients with FRDA.

(1) Background: There is much debate about the use of salt-restricted diet for managing heart failure (HF). Dietary guidelines are inconsistent and lack evidence. (2) Method: The OFICSel observatory collected data about adults hospitalised for HF. The data, collected using study-specific surveys, were used to describe HF management, including diets, from the cardiologists' and patients' perspectives. Cardiologists provided the patients' clinical, biological, echocardiography, and treatment data, while the patients provided dietary, medical history, sociodemographic, morphometric, quality of life, and burden data (burden scale in restricted diets (BIRD) questionnaire). The differences between the diet recommended by the cardiologist, understood by the patient, and the estimated salt intake (by the patient) and diet burden were assessed. (3) Results: Between March and June 2017, 300 cardiologists enrolled 2822 patients. Most patients (90%) were recommended diets with <6 g of salt/day. Mean daily salt consumption was 4.7 g (standard deviation (SD): 2.4). Only 33% of patients complied with their recommended diet, 34% over-complied, and 19% under-complied (14% unknown). Dietary restrictions in HF patients were associated with increased burden (mean BIRD score of 8.1/48 [SD: 8.8]). (4) Conclusion: Healthcare professionals do not always follow dietary recommendations, and their patients do not always understand and comply with diets recommended. Restrictive diets in HF patients are associated with increased burden. An evidence-based approach to developing and recommending HF-specific diets is required.

Background and Objectives Friedreich ataxia (FRDA) is an autosomal recessive ataxia with no approved treatments. Leriglitazone is a selective peroxisome proliferator-activated receptor. agonist that crosses the blood-brain barrier and, in preclinical models, improved mitochondrial function and energy production. We assessed effects of leriglitazone in patients with FRDA in a proof-of-concept study. Methods In this double-blind, randomized controlled trial, eligible participants (age 12-60 years) had genetically confirmed FRDA, a Scale for the Assessment and Rating of Ataxia (SARA) total score <25, and a SARA item 1 score of 2-6, inclusive. Key exclusion criteria were age at FRDA onset >= 25 years and history of cardiac dysfunction. Participants were randomly assigned (2:1) to receive a daily, oral, individualized dose of leriglitazone or placebo for 48 weeks. The primary endpoint was the change from baseline to week 48 in spinal cord area (C2-C3) (measured by MRI). Secondary endpoints included the change from baseline to week 48 in iron accumulation in the dentate nucleus (quantitative susceptibility mapping) and total N-acetylaspartate to myoinositol (tNAA/mIns) ratio. Results Overall, 39 patients were enrolled (mean age 24 years; 43.6% women; mean time since symptom onset 10.5 years): 26 patients received leriglitazone (20 completed) and 13 received placebo (12 completed). There was no difference between groups in spinal cord area from baseline to week 48 (least-squares [LS] mean change [standard error (SE)]: leriglitazone, -0.39 [0.55] mm(2); placebo, 0.08 [0.72] mm(2); p = 0.61). Iron accumulation in the dentate nucleus was greater with placebo (LS mean change [SE]: leriglitazone, 0.10 [1.33] ppb; placebo, 4.86 [1.84] ppb; p = 0.05), and a numerical difference was seen in tNAA/mIns ratio (LS mean change [SE]: leriglitazone, 0.03 [0.02]; placebo, -0.02 [0.03]; p = 0.25). The most frequent adverse event was peripheral edema (leriglitazone 73.1%, placebo 0%). Discussion The primary endpoint of change in spinal cord area was not met. Secondary endpoints provide evidence supporting proof of concept for leriglitazone mode of action and, with acceptable safety data, support larger studies in patients with FRDA. Trial Registration Information ClinicalTrials.gov: NCT03917225; EudraCT: 2018-004405-64; submitted April 17, 2019; first patient enrolled April 2, 2019. clinicaltrials.gov/ct2/show/NCT03917225?term=NCT03917225&draw=2&rank=1. Classification of Evidence This study provides Class I evidence that individualized dosing of leriglitazone, compared with placebo, is not associated with changes in spinal cord area in patients with FRDA.

Fréquentes dans la population générale, l’insuffisance cardiaque (IC) et le diabète sont deux maladies chroniques dont la prévalence augmente et qui représentent un lourd fardeau pour les systèmes de soins. Leur association est fréquente. De fait, le diabète est un facteur de risque important d’IC, qu’il s’agisse d’IC à fraction d’éjection réduite ou préservée. De nombreux mécanismes expliquent ces interactions, au premier rang desquels l’ischémie myocardique, l’hypertension artérielle, mais aussi l’âge, le sexe, l’obésité. Une IC est présente chez 10 à 15 % des diabétiques, et son incidence annuelle chez les patients à plus haut risque vasculaire ou en prévention secondaire est de l’ordre de 1 à 2 % par an. À l’inverse, le diabète concerne 30 à 40 % des patients insuffisants cardiaques, quelle que soit la fraction d’éjection, et est un facteur de mauvais pronostic dans une pathologie déjà très sévère. L’arrivée dans l’arsenal thérapeutique de l’IC de la classe des gliflozines, initialement développées comme antidiabétiques et dont les mécanismes d’action sur le cœur restent encore énigmatiques, va certainement contribuer à mieux comprendre les interactions physiopathologiques entre diabète et IC.

Le 13/03/20, Mayotte déclare son premier cas de Covid-19. Le contexte socio-économique précaire rend inconcevable l'application des recommandations de prévention. Bien que la moitié de la population ait moins de 18 ans ; chez les adultes, la prévalence des facteurs de risque de formes graves fait redouter une saturation de l'unique hôpital de l'île : 38 % d'HTA, 12 % de diabète tandis que l'IMC moyen est de 26,9 kg/m2. Afin de comprendre la dynamique et la sévérité du Covid-19 à Mayotte, plusieurs dispositifs de surveillance ont été mis en place. Ce travail décrit l'évolution épidémique du 9/03/20 au 14/03/21 en distinguant deux vagues différentes du fait de l'émergence de variants: 2020 (9/03/20-31/12/20) versus 2021 (01/01/21-14/03/21). L'épidémie a débuté en mars 2020 avec un pic en mai (taux d'incidence (TI) de 133/100000 hbts). L'âge médian des cas était de 36 ans et la plupart d'entre eux était sans activité professionnelle. Le TI a diminué pour passer début juillet, sous le seuil d'alerte, suggérant une accalmie durant l'hiver austral. Cette première vague s'est avérée relativement peu sévère avec un maximum de cas hospitalisés en S21 : 51 patients hospitalisés dont 11 en réanimation. La circulation virale a continué sans impact sanitaire majeur jusqu'en décembre 2020 où l'Afrique du Sud a signalé l'émergence du variant 501Y.V2. Quelques semaines après les Comores, Mayotte enregistrait une dégradation rapide et intense de la situation: doublement du TI, triplement du taux de positivité Sars-Cov-2 (TP) entre mi-décembre et mi-janvier, y compris chez les 0-14 ans. En S6-2021, le TI a atteint un pic (894/100000 hbts) tout comme le TP culminant à 28 %. Deux enquêtes flash en février ont montré la part majoritaire d'un variant avec la mutation 501Y, représentant 87 % (150/172) des échantillons analysés par criblage. Les indicateurs hospitaliers témoignaient de la sévérité de cette nouvelle vague avec un pic atteint en S18-2021 : 148 patients hospitalisés dont 27 en réanimation, majoritairement des formes oxygéno-requérentes. En 2021, 149 cas ont été admis en réanimation contre 104 en 2020 (sex ratio H/F = 1,9). Parmi les patients présentant une forme pulmonaire, le profil des cas admis en réanimation en 2021 a changé par rapport à 2020 : ils étaient significativement plus jeunes (57 vs 62 ans) et présentaient un profil de comorbidités différent. Au total, en un an, 18899 cas ont été confirmés biologiquement et 253 patients admis en réanimation: ils correspondaient à 203 cas de formes pulmonaires, 46 cas de portage asymptomatique et 14 cas de syndrome inflammatoire multi-systématique associé à une infection par le Sars-Cov-2. En 2021, 88 patients sont décédés contre 55 en 2020; âges médians respectifs de 73 et 64 ans. Le confinement de février 2021 s'est suivi d'une inversion de la tendance épidémique. Fin mars, l'épidémie se poursuit avec un TI de 63/100000 hbts. Alors que moins de 5000 personnes ont été vaccinées à deux doses, la vigilance est de mise pour ce territoire vulnérable.

As opposed to ventricular arrhythmias (VA), clinical implications of atrial tachyarrhythmias (AT) in arrhythmogenic right ventricular cardiomyopathy (ARVC) remain scarcely explored. This study sought to evaluate the prevalence and prognostic significance of AT in ARVC. In total, 171 patients diagnosed with ARVC between 1985 and 2018 in a single tertiary center were retrospectively included. Were defined as follows: AT: sustained atrial fibrillation, atrial flutter and focal atrial tachycardia; major adverse cardiovascular events (MAE) as a composite criterion including heart failure, cardiac assistance, transplantation, and death. After a median follow-up of 6 years (IQR 3 to 11), prevalence of AT, MAE and VA were respectively of 16%, 8% and 60%. AT occurred later in the disease course: mean age 54 ± 14.4, while 50% of patients had VA at diagnosis. Age at diagnosis (hazard-ratio [HR]: 1.05, 95% CI [1.02–1.08]; P < 0.001), RVEF (HR: 0.96, 95% CI [0.92–0.99]; P = 0.03) and LVEF (HR: 0.96; 95% CI: 0.92–0.99; P = 0.04) predicted AT occurrence in univariable analysis. Intensive sport activity was significantly associated with AT in survival and multivariable Cox analysis. AT was predictive of MAE occurrence (HR: 2.6, 95% CI [1.1–6.3]; P = 0.03). AT are common in ARVC and AT is associated with intensive sport activity and MAE. Our results mandate careful monitoring of ARVC patients with new-onset AT.

BACKGROUND:Friedreich's ataxia (FA) is a rare autosomal recessive mitochondrial disease most commonly due to a triplet repeat expansion guanine-adenine-adenine (GAA) in the FXN gene. Cardiac disease is the major cause of death, patients with reduced left ventricular ejection fraction (LVEF) having the worse prognosis. Longitudinal strain (LS) appeared to be a better predictor of outcome than LVEF in different diseases. We compared the prognostic value of LS measured from the 4 chambers view to LVEF. METHODS:From 2003 to 2017 consecutive patients with FA were included and LS analysis was retrospectively performed. RESULTS:We studied 140 patients, with a median age of 34 (26-41) years (Q1-Q3) with age at onset of 14 (11-19) years and GAA repeats on the shorter allele of 600 (467-783) pb. Mean LS was 19.9 ± 5.0% and LVEF 64 ± 8%. After a mean follow-up of 7.4 ± 3.9 years, 14 patients died. In univariate Cox analysis, all-cause mortality was associated with: LS (HR 0.83; 95%CI, 0.75-0.91, p = 0.0002), LVEF (HR 0.30; 95%CI, 0.19-0.49, p < 0.0001), GAA repeats on the shorter allele (HR 1.29; 95%CI, 1.10-1.51, p = 0.002), age at onset (HR 0.87; 95%CI, 0.77-0.98, p = 0.018), LVSystolic Diameter (HR 1.17; 95%CI, 1.09-1.26, p < 0.0001), LVMass index (HR 1.02; 95%CI, 1.00-1.04, p = 0.027), and LVDiastolic Diameter (HR1.12; 95%CI, 1.01-1.23, p = 0.028). In multivariate analysis, LVEF was the only independent predictor of mortality (HR 0.41; 95%CI, 0.23-0.74, p = 0.0029). CONCLUSION:In FA, LS was not an independent predictor of mortality, LVEF remained the only independent predictor in the present study.

Friedreich’s ataxia (FRDA) is a cerebellar ataxia due to GAA repeat expansions in the FXN gene, and in affected patients, lower left ventricular ejection fraction (LVEF) leads to poorer prognosis. We aimed to identify patients likely to develop worsening LVEF at an early stage. We included 115 FRDA patients aged 30 ± 10 years with 620 ± 238 GAA repeats on the shorter allele and disease onset of 15 ± 7 years. At baseline, left ventricular (LV) hypertrophy was present in 53%, with LVEF 65 ± 7%, LV end diastolic diameter (LVEDD) 43 ± 5 mm, septal wall thickness (SWT) 11.8 ± 2.7 mm, and posterior wall thickness 11.1 ± 2.5 mm. After a mean follow-up of 13 ± 6 years, LVEF ≤ 50% was observed in 12 patients. The main determinants of LVEF ≤ 50% were GAA repeat number on the shorter allele (odds ratio [OR] 1.007, 95% confidence interval [CI] 1.003–1.012, p = 0.002), LVEDD (OR 1.217, 95% CI 1.058–1.399, p = 0.006), and SWT (OR 1.352, 95% CI 1.016–1.799, p = 0.04). High-risk patients were predicted 5 years before LVEF ≤ 50% occurred: area under the curve of 0.91, 95% CI 0.85–0.97. Patients with GAA repeats > 800 were categorized as high risk, patients with 500 < GAA < 800 were high risk if LVEDD was ≥ 52.6 mm and SWT was ≥ 13.3 mm, and patients with GAA < 500 were low risk if LVEDD was < 52.6 mm and SWT was < 13.3 mm. Echocardiographic follow-up combined with size assessment of GAA repeat expansions is a powerful tool to identify patients at high risk of developing LV systolic dysfunction up to 5 years before clinical symptoms. Further studies are mandatory to investigate if these patients would benefit from cardiac interventions.

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. Results: The 85 FA patients (49% women) with a mean age of 39 +/- 12 years, a mean disease onset of 17 +/- 11 years had a mean SARA (Scale for the Assessment and Rating of Ataxia) score of 26 +/- 10. The median hsTnT concentration was 10 ng/L (3 to 85 ng/L) and 34% had a significant elevated hsTnT >= 14 ng/L. Increased septal wall thickness was associated with increased hsTnT plasma levels (p< 0.001). The median NT-proBNP concentration was 31 ng/L (5 to 775 ng/L) and 14% had significant elevated NT-proBNP >= 125 ng/L. Markers of increased left ventricular filling pressure (trans mitral E/A and lateral E/E' ratio) were associated with increased NT-proBNP plasma levels (p= 0.01 andp= 0.01). Length of GAA or the SARA score were not associated with hsTnT or NT-proBNP plasma levels. Conclusion: hsTnT was increased in 1/3 of the adult FA and associated with increased septal wall thickness. Increased NT-proBNP remained a marker of increased left ventricular filling pressure. This could be used to identify patients that should undergo a closer cardiac surveillance.

Left Ventricular Thrombus (LVT) is associated with a high risk of thromboembolic complications such as stroke. Contemporary data are lacking on the management, prognosis and treatment of LVT, particularly with the emergence of the non-vitamin K antagonist anticoagulants (NOACs). To study the time and predictive factors associated with thrombus regression on treatment and its association with survival, embolic and bleeding complications. From January 2011 to January 2018, a computerized case sensitive search of LVT was performed on 90 065 consecutive echocardiogram reports. All patients with a confirmed LVT were included in this analysis after imaging review by two independent experts. Repeated echocardiographic data, treatment management and clinical outcomes were collected during follow-up. Major adverse cardiac events (MACE), defined as the composite of death, ischemic stroke or transient ischemic attack (TIA), myocardial infarction (MI) or embolic peripheral artery occlusion were analyzed as well as major bleeding events (BARC ≥3) and the predictive factors and impact of LVT regression. We identified 174 patients with a suspected LVT of whom 159 had confirmed LVT on two different cardiac imaging exams. Ischemic cardiomyopathy was the main cause of LVT (n=125, 78.6%) including 56 (35.2%) patients with an acute ST segment elevation MI. The mean left ventricular ejection fraction was 31.9±12.5% with predominant (98.1%) apical location of the LVT. Anticoagulation therapy was achieved with vitamin K antagonists, NOACs and parenteral heparins in 48.7%, 22.8% and 27.8% of patients, respectively. Concomitant antiplatelet therapy was prescribed in 67.9% of patients. Total LVT regression was reached in two third of patients (62.3%, n=99) within a median time of 103 [32–392] days. Independent predictors of total LVT regression were an ischemic cardiomyopathy (HR: 0.36 [0.19–0.70], p=0.002), a larger baseline thrombus area (HR=0.66 [0.45–0.96], p<0.031) and a prolonged anticoagulation therapy over 3 months (HR=0.11 [0.05–0.22], p<0.0001). During a median follow-up of 632 [187–1126] days, MACE occurred in 59 (37.1%) patients with a 18.9% rate of mortality and 13.2% of major bleeding. Patients with a total LVT regression had a non-significant lower rate of MACE as compared with patients without total LVT regression (35.4% vs. 40.0%; HR=0.71 [0.42–1.21]; p=0.20), and a significant lower rate of mortality (15.2% vs. 25.0%; HR=0.48 [0.23–0.98]; p=0.039). Occurence of mortality (A) and MACE (B) The prognosis of LVT remains severe with a high risk of major cardiovascular event and mortality. Total LVT regression, mostly reached in 3 months, can be obtained with both vitamin K antagonists and NOACs and is associated with a better prognosis.