History of syncope, clinical examination and electrocardiographic (ECG) findings are fundamental to assess the risk of major cardiovascular events (MACE) in patients attending the emergency department (ED) for syncope. However, in the absence of abnormal clinical examination findings or an abnormal ECG in the ED, transient rhythm or conduction disorders may not be safely excluded, hence predicting MACE remains challenging. High-sensitivity cardiac troponin T (hs-cTnT) may be a useful tool in this context. The primary objective was to evaluate the performance of hs-cTnT in the diagnosis of MACE at 30days in patients attending the ED for syncope with a normal initial ECG. This was a prospective observational cohort study that took place in the ED of a French university hospital between June 2018 and June 2019. Patients≥18years admitted to the ED for syncope with a normal ECG were eligible. After receiving verbal consent from patients, the ED physician collected clinical and ECG data and all patients had a blood sample taken that included hs-cTnT measurement. The primary outcome was MACE within 30days after the ED visit. MACE were evaluated by consulting the patient's medical records and telephoning patients or their general practitioners. Sensitivity, specificity, positive and negative predictive values were calculated with their 95% confidence intervals (CI) for different hs-cTnT thresholds. Data from 246 patients were analysed, including 21 (9%) with MACE. Hs-cTnT had an area under the curve of 0.917 (CI: 0.872-0.962). Hs-cTnT with a threshold of 19ng/L had a sensitivity of 86% (CI: 64-97) and a specificity of 86% (CI: 81-90) for predicting MACE. Hs-cTnT may be a relevant tool for assessing MACE risk in patients with syncope and normal ECG results.

Although, valve sparing is commonly performed in patients with Marfan syndrome, feasibility and results of cusp repair for aortic insufficiency have not been studied. To report on the outcomes and durability of aortic cusp repair in valve sparing in patients with Marfan syndrome. All consecutive adult patients with Marfan syndrome who underwent remodelling and annuloplasty with aortic valve repair for aortic insufficiency between May 2005 and December 2020 were included. Patients with Marfan syndrome treated for aortic aneurysm, but without aortic insufficiency, were excluded. Data were collected prospectively and reviewed retrospectively from the Aorticvalve repair International Registry (AVIATOR). During the study period, 71 patients with Marfan syndrome were referred to surgery. Fifty-five patients with connective tissue disease and aortic insufficiency with aorta aneurysm were treated: 46 underwent aortic valve repair and nine underwent aortic valve replacement (five mechanical aortic valve replacements and four biological aortic valve replacements). The mean age was 42.9±15.4years, and the mean EuroScore II was 2.5±2.2. No patient died, and no patient had significant aortic insufficiency (grade≥II) at discharge. The 5-year survival rate estimate was 94.4%, which seems statistically similar to that of the age- and sex-matched general population. At 5years, freedom from reoperation was 94.6%, and the incidence of infective endocarditis was 2.6%. No valve thrombosis, aortic dissection, major bleeding events, thromboembolic events (stroke) or myocardial infarctions were noted during follow-up. Remodelling and aortic valve repair showed excellent durability at 5years, even in connective tissue disorders.

Acute myocarditis is an inflammation of the myocardium that can cause life-threatening events. However, anti-inflammatory strategies did not reduce the risk of clinical outcomes in randomized trials. Recently, experimental studies have suggested that specific blockade of the interleukin-1β immune innate pathway could be effective in acute myocarditis. To test the hypothesis that inhibition of the interleukin-1β immune innate pathway can reduce the risk of clinical events in acute myocarditis. The "Anakinra versus placebo double blind Randomized controlled trial for the treatment of Acute MyocarditIS" (ARAMIS) trial (ClinicalTrials.gov identifier: NCT03018834) is a national multicentre randomized parallel-group double blind study among symptomatic patients with elevated cardiac troponin and cardiac magnetic resonance-proven acute myocarditis. Patients (n=120) are randomized within 72hours of hospital admission to receive a daily subcutaneous dose of anakinra 100mg or placebo during the hospitalization, in addition to standard of care, including an angiotensin-converting enzyme inhibitor and a beta-blocker. The primary endpoint is the number of days alive free from any myocarditis complication, including ventricular arrhythmias, heart failure, recurrent chest pain requiring medication and ventricular dysfunction (defined as left ventricular ejection fraction<50%), from randomization to 28 days after hospital discharge. At 28 days after discharge, patients with normal left ventricular ejection fraction are then randomized to angiotensin-converting enzyme inhibitor continuation or discontinuation and all patients are followed for 1 year, with regular left ventricular function evaluation. ARAMIS is the first trial evaluating inhibition of the interleukin-1β immune innate pathway in the setting of acute myocarditis. Although of small size, it will be the largest randomized trial in acute myocarditis, a serious and poorly studied cardiac condition.

Early cardiac amyloidosis (CA) diagnosis enables patients to access effective treatments for better long-term outcomes, yet it remains under-recognised, misdiagnosed and inadequately managed. To reduce diagnostic delays, we aimed to describe the epidemiological and clinical characteristics and changes over an 11-year period. This was a retrospective, observational cohort study of all patients referred to the Henri-Mondor Hospital for suspected CA. Overall, 3194 patients were identified and 3022 were included and analysed. Our patients came from varied ethnic backgrounds, and more than half (55.2%) had confirmed CA. Over 11 years, referrals increased 4.4-fold, mostly from cardiologists. Notably, wild-type transthyretin amyloidosis (ATTRwt) became the predominant diagnosis, with referrals increasing 15-fold from 20 in 2010-2012 to 308 in 2019-2020. The number of amyloid light chain (AL) diagnoses increased, whilst variant transthyretin amyloidosis (ATTRv) numbers remained relatively stable. Concerning disease severity, AL patients presented more frequently with severe cardiac involvement whereas an increasing number of ATTRwt patients presented with National Amyloid Centre stage I (22.0% in 2013-2014 to 45.9% in 2019-2020). Lastly, among patients diagnosed with ATTRv in 2019-2020, 83.9% had ATTR Val122Ile cardiac phenotype. This study shows that increasing cardiologist awareness and referrals have increased CA diagnoses. With improved awareness and non-invasive diagnostic techniques, more patients with ATTRwt with milder disease and more ATTRv Val122Ile mutations are being referred and diagnosed. Although more AL cases are being recognised, patients are diagnosed with severe cardiac involvement.

Aortic valve stenosis is associated with age, rheumatic fever and bicuspid aortic valve, but its association with other co-morbidities, such as inflammatory disease and race/ethnicity, is less known. To investigate any association between aortic stenosis and many co-morbidities. We used the large Nationwide Inpatient Sample database to evaluate any association between aortic stenosis and risk factors. We performed univariate and multivariable analyses, adjusting for co-morbid conditions. Data were extracted from the first available database that used the International Classification of Diseases, Tenth Revision codes specifically coding for aortic stenosis alone, spanning from 2016 to 2020 (n=112,982,565). A total of 2,322,649 patients had aortic stenosis; the remaining 110,659,916 served as controls. We found a strong and independent significant association between aortic stenosis and coronary artery disease (odds ratio [OR]: 2.11, 95% confidence interval [CI]: 2.09-2.13), smoking (OR: 1.08, 95% CI: 1.07-1.08), diabetes mellitus (OR: 1.15, 95% CI: 1.14-1.16), hypertension (OR: 1.41, 95% CI: 1.4-1.42), hyperlipidaemia (OR: 1.31, 95% CI: 1.3-1.32), renal disease (OR: 1.3, 95% CI: 1.29-1.31), chronic obstructive pulmonary disease (OR: 1.05, 95% CI: 1.04-1.05), obesity (OR: 1.3, 95% CI: 1.29-1.32), white race/ethnicity (OR: 1.47, 95% CI: 1.42-1.52), rheumatoid arthritis (OR: 1.13, 95% CI: 1.11-1.15), scleroderma (OR: 1.93, 95% CI: 1.79-2.09), systemic connective tissue disease (OR: 1.24, 95% CI: 1.2-1.27), polyarteritis nodosa (OR: 1.5, CI: 1.24-1.81) and Raynaud's syndrome (OR: 1.16, 95% CI: 1.09-1.24) (all P<0.001), in addition to known factors, such as age, male sex and bicuspid aortic valve. Using a very large database, we found many new associations with aortic valve stenosis, including race/ethnicity, renal disease, several inflammatory diseases, chronic obstructive pulmonary disease and obesity, in addition to many other known cardiovascular risk factors.