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Abstract Introduction Primary cardiac lymphoma is rare. Case Presentation We report the case of a 64-year-old non-immunodeficient Caucasian man, with cardiac tamponade and paroxysmal third-degree atrioventricular block. Echocardiography revealed the presence of a large pericardial effusion with signs of tamponade and a right ventricular mass was suspected. Scanner investigations clarified the sites, extension and anatomic details of myocardial and pericardial infiltration. Surgical resection was performed due to the rapid impairment of his cardiac function. Analysis of the pericardial fluid and histology confirmed the diagnosis of non-Hodgkin large B-cell lymphoma. He was treated with chemotherapy. Conclusion The prognosis remains poor for this type of tumor due to delays in diagnosis and the importance of the site of disease.

Background: Global longitudinal strain (GLS), derived from speckle-tracking echocardiography (STE), is a widely used and reproducible left ventricular deformation parameter; assessment of multilayer strain components has also become possible. However, its association with comorbidities/symptoms in low-risk populations without cardiac disease remains understudied. We report reference ranges for longitudinal deformation and their association with cardiovascular risk factors and dyspnea in a large population-based cohort., Methods: We studied 1,243 subjects without cardiac disease (47 ± 14 years, 47.4% men; 13.8% with dyspnea) enrolled at the fourth visit of the STANISLAS Cohort (Lorraine, France). Clinical evaluation included a comprehensive dyspnea questionnaire. Multilayer GLS (full-wall, subendocardial, and subepicardial) and strain rate (systolic, early, and late diastolic) were evaluated by GLS STE acquisition and measurement protocols as per recommendations by the European Association of Cardiovascular Imaging, American Society of Echocardiography, and Industry Task Force., Results: Full-wall GLS was 23.4% ± 2.7% (mean ± SD) with a subendocardial/subepicardial ratio of 1.2 ± 0.1. Age, gender, smoking status, and body mass index were significantly associated with strain variables, whereas diabetes, dyslipidemia, and hypertension/systolic blood pressure were not. Specifically, there were reductions in diastolic strain rate with aging but no differences in GLS. After propensity score matching, subjects with dyspnea had lower global endocardial strain (-23.48 ± 2.70 vs -23.02 ± 2.81; P = .043) and lower global subendocardial/subepicardial strain ratio (P = .034), whereas transmural strain and classical echocardiographic measurements were unrelated to dyspnea., Conclusions: Higher body mass index was found to be significantly associated with impaired strain variables in a low-risk population without cardiac disease. In addition, lower global endocardial strain and lower global subendocardial/subepicardial strain ratio were significantly associated with dyspnea contrary to other echocardiographic variables., (Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Diastolic dysfunction (DD) is increasingly common. However, its metabolic determinants are poorly known. This study aims to determine which metabolic and inflammatory features predict DD in initially healthy adults., Methods: We prospectively analyzed the association between metabolic features and DD in 728 initially healthy adults aged 30-60 from Eastern France enrolled in the STANISLAS population-based cohort. Clinical and biological cardiovascular features were collected at baseline (1994-1995). DD was assessed twenty years later (2011-2016) by echocardiography using current international guidelines. For replication purposes, 1463 subjects from the Malmö Preventive Project cohort were analyzed., Results: In the STANISLAS cohort, 191 subjects (26.2%) developed DD. In age-sex-adjusted logistic models, significant predictors of DD were body mass index (BMI, odds ratio for 1-standard-deviation increase (OR) 1.28, 95% CI 1.08-1.52), waist circumference (WC, OR 1.48, 95% CI 1.18-1.84), waist-hip ratio (OR 1.53, 95% CI 1.16-2.02), systolic blood pressure (OR 1.19, 95% CI 1.00-1.43) and triglycerides (TG, OR 1.18, 95% CI 1.00-1.40). Subjects with elevated WC (> 80th percentile) and TG (> 50th percentile) had a twofold higher DD risk (age-sex-adjusted odds ratio 2.00, 95% CI 1.20-3.31, P = 0.008), whereas no such interplay was observed for BMI. In the Malmö cohort, BMI was similarly associated with DD; participants with both elevated BMI and TG were at higher DD risk (age-sex-adjusted odds ratio 1.61, 95% CI 1.18-2.20, P = 0.002)., Conclusions: Subjects with elevated WC and TG may have a higher long-term DD risk. Prevention targeting visceral obesity may help reduce the incidence of DD.

Higher serum bilirubin has been associated with poorer prognosis in patients with heart failure (HF). We examined the association between serum bilirubin and clinical outcomes in patients with clinical signs of HF and/or left ventricular systolic dysfunction after acute myocardial infarction (MI). A total of 7,467 patients from the High-Risk Myocardial Infarction Database Initiative with an available baseline total bilirubin concentration were studied. The association between baseline bilirubin concentrations and the composite outcome of cardiovascular mortality (CVM), nonfatal stroke, nonfatal MI or hospitalization for HF, CVM, and all-cause mortality were assessed using Cox proportional hazards models. An interaction with time was observed with associations present only in the first 90 days after randomization. The median (percentile 25-75 ) baseline total bilirubin concentration was 11 (8 to 14) µmol/L and was above the "normal" range (>17.1 µmol/L) in 1,053 (14.1%) patients. In multivariable analysis, with adjustment for baseline characteristics (demographic, co-morbidities, Killip score, left ventricular ejection fraction, and laboratory variables), patients with a bilirubin concentration of >17.1 µmol/L had a significantly higher risk of all the studied outcomes at 90 days (e.g., CVM: adjusted hazard ratio 1.45, 95% confidence interval 1.14 to 1.86, p = 0.003). The addition of bilirubin to a validated survival model modestly improved the risk reclassification to predict 90-day events (continuous net reclassification improvement for CVM 6.4%, 95% confidence interval 0.7% to 9.6%, p = 0.04). In patients with MI complicated with HF and/or systolic dysfunction, bilirubin concentration is an independent predictor of mortality and may improve risk stratification., (Copyright © 2018. Published by Elsevier Inc.)

Introduction: There is limited evidence regarding intra-observer and inter-observer variations in echocardiographic measurements of diastolic function. This study aimed to assess this reproducibly within a population-based cohort study., Methods: Sixty subjects in sinus rhythm were randomly selected among 4th visit participants of the STANISLAS Cohort (Lorraine region, France). This 4th examination systematically included M-mode, 2-dimensional, DTI and pulsed-wave Doppler echocardiograms. Reproducibility of variables was studied by intra-class correlation coefficients (ICC) and Bland Altman plots., Results: Our population was on average middle-aged (50 ± 14 y), overweight (BMI = 26 ± 6 kg/m2) and non-smoking (87%) with a quarter of the participants having self-declared hypertension or treated with anti-hypertensive medication(s). Intra-observer ICC were > 0.90 for all analyzed parameters except for left ventricular ejection fraction (LVEF) which was 0.89 (0.81-0.93). The mean relative intra-observer differences were small and limits of agreement of relative differences were narrow for all considered parameters (<5% and <15% respectively). Inter-observer ICC were > 0.90 for all analyzed parameters except for LVEF (ICC = 0.87) and both mitral and pulmonary A wave duration (0.83 and 0.73 respectively). The mean relative inter-observer differences were <5% for all parameters except for pulmonary A wave duration (mean difference = 6.5%). Limits of agreement of relative differences were narrow (<15%), except for mitral A wave duration and velocity (both <20%) as well as left ventricular mass and pulmonary A wave duration (both <30%). Intra-observer agreements with regard to the presence and severity of diastolic dysfunction were excellent (Kappa = 0.93 (0.83-1.00) and 0.88 (0.75-0.99), respectively)., Conclusion: In this validation study within the STANISLAS cohort, diastolic function echocardiographic parameters were found to be highly reproducible. Diastolic dysfunction consequently appears as a highly effective clinical and research tool.

Postinfarction ventricular septal defect (PIVSD) is a devastating mechanical complication following acute myocardial infarction. The management of this pathology is quite challenging, especially in case of complicated cardiogenic shock. The difficulties lie in the timing and type of intervention. Debates exist with regard to immediate versus deferring repair, as well as open repair versus percutaneous closure. The anatomic characteristics and hemodynamic consequence of PIVSD are important elements determining which strategy to adopt, since large septal defect (>15 mm) cannot be appropriately treated by percutaneous occluder devices limiting by their available size, while compromised hemodynamics usually require emergent repair or mechanical support "bridging to surgery". Herein, we report our experience of successful management of a case of cardiogenic shock complicating large PIVSD (38 mm) by delayed surgical repair bridged with Extracorporeal Membrane Oxygenation (ECMO) during 7 days. We emphasize the importance of 3-dimensional transesophageal echocardiography as a decision-making tool.

Introduction: Left ventricular (LV) systolic dysfunction is the most frequent initial presentation of patient with LV noncompaction (NC). Our objectives were to evaluate myocardial contraction properties in patients with LVNC and the relationship of non-compacted segments with the degree of global and regional systolic deformation., Methods: We included 50 LVNC with an echocardiography and speckle imaging calculation of peak longitudinal strain (PLS). Each of the 16 LV myocardial segments was defined as NC (ratio NC/compacted layer > 2), borderline (NC/C 0-2) and compacted (NC/C = 0). Basal, median and apical strain values were calculated as the average of segmental strain values. For comparison a group of 50 patients with dilated cardiomyopathy (DCM) underwent the same measurements., Results: There was no statistical difference between the 2 groups for any conventional LV systolic parameters. A characteristic deformation pattern was observed in LVNC with higher strain values in the LV apical segments (- 12.8 ± 5.9 vs - 10.7 ± 5.7) and an apical-basal ratio (1.52 ± 0.73 vs 1.12 ± 0.42; p < 0.001). There was no correlation between LV function and the degree of NC. Among 726 segments, compacta thickness was thinner in NC vs C segments (6.4 ± 1.4 vs 7.7 ± 1.8 mm; p < 0.05). There was no difference in WMS but regional strain values were significantly higher in NC compared to C segments (- 13.1 ± 6.1 vs - 10.2 ± 6.3; p < 0.05)., Conclusions: Compared to DCM, LVNC presented with relatively preserved apical deformation as compared to basal segments. Lower regional deformation values in compacted segments confirm the concept that LVNC is a phenotypic marker of an underlying diffuse cardiomyopathy involving both C and NC myocardium.

Background: Several arrhythmias were reported in myotonic dystrophy (MD)., Objectives: To evaluate the prevalence of atrial fibrillation (AF) and atrial flutter (AFL) in MD and the clinical consequences., Methods: One hundred sixty-one patients, mean age 41 ± 14 years, were referred for a type 1 MD. All patients were asymptomatic except four patients and followed during 5 ± 4 years. Electrocardiogram (ECG), echocardiography assessing left ventricular ejection fraction, and Holter monitoring were obtained and repeated., Results: Twenty-seven patients (17%) presented sustained (>1 hour) AF (n = 15) or AFL (n = 12); two of them presented syncope-related 1/1 AFL. In one of them, 16 years of age, cardiac defibrillator was implanted for a diagnosis of ventricular tachycardia, but the true diagnosis was established after inappropriate shocks. AFL ablation was performed in five patients, but four developed AF. The other seven patients with AFL developed AF. During the follow-up, 22 patients died (14%) from cardiac and respiratory failure; eight patients with AF/AFL died (30%) while only 14 without AF/AFL died (10%; P < 0.01). Univariate analysis indicated that age >40 years (death: 48 ± 14 vs 40 ± 8 in alive patients), abnormal ECG, and occurrence of AF/AFL were significant factors of death. At multivariate analysis, AF at ECG (odds ratio: 3.12) and age >40 (odds ratio: 3.14) were the sole independent variables predicting death., Conclusions: AF and AFL were frequent in MD and increased mortality. AFL could present as 1/1 AFL with a poor tolerance and a risk of misdiagnosis despite frequent conduction disturbances. This arrhythmia could explain wide QRS tachycardia occurring in MD and interpreted as VT., (©2013, The Authors. Journal compilation ©2013 Wiley Periodicals, Inc.)

The arrhythmogenic right ventricular dysplasia (ARVD) is a rare heart muscle disorder, occurring typically in young adults. The diagnosis remains difficult. The aim of our study was to evaluate the contribution of Doppler echocardiography in the diagnosis and screening for ARVD. Eighteen patients, followed in our cardiology department for ARVD and 10 "witnesses" in good condition were evaluated by echocardiography. We have essentially clarified the dimensions of the RV, its kinetics segmental, Doppler tricuspid flow, the study of movement of the tricuspid annulus in M-mode and tissue Doppler. We have compared the results of echocardiography at those of MRI and RV angiography. In our series, pathological measurement of the RV was found in all patients. No one of the control group had expansion of the RV. Twelve of our patients had abnormal wall motion. The displacement of the tricuspid annulus is reduced both in septal and lateral position. In tissue Doppler, Ea/Aa was lower than in healthy subjects in 15 cases in the lateral position and 16 cases in septal position. ETT is superior to MRI and RV angiography in the diagnosis of localized forms. The family survey, conducted in 38 parents of eight consenting families allowed to screen seven subjects. All these patients had abnormal Doppler echocardiography. Doppler echocardiography is a reliable and efficient investigation for the diagnosis of ARVD. It remains on the first intention to review a suspected ARVD., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

We herein present the findings of the case of a 23-year-old man who was hospitalized for ventricular tachycardia (VT) with no previous history of cardiac disease or any family history of sudden death. Based on the clinical features as well as the echographic and MRI results, the patient was diagnosed with both acute viral myocarditis and arythmogenic right ventricular dysplasia (ARVD). The patient underwent implantation of an automatic cardioverter defibrillator. There was no recurrent VT during the 24 month follow-up. This case demonstrates the link between ARVD and myocarditis, and highlights the importance of conducting an RV assessment through a cardiac magnetic resonance (CMR) study in the context of arrhythmia and myocarditis.

Objective: The purpose of the present study is to describe our experience with patients who have a transmural myocardial infarction (MI) in the presence of a normal coronary artery. The clinical profile, demographic characteristics and outcomes of these patients are discussed., Methods: Between January 2006 and August 2011, 21 patients who presented with a Q-wave myocardial infarction were found to have normal coronary arteries. The prevalence rate of this entity was 1.5% (21 out of 1,400 Q wave MI patients). These patients were characterized by their young age (the mean age=44.95±14.86), male dominance (90.47%), and a high prevalence of smoking (85.71%). In this study, 4 patients have an evident spontaneous spasm shown on coronary angiography which disappeared after intracoronary injection of nitrates. Coagulation Disorders, such as activated protein C resistance (APC) resistance, protein C deficiency and antiphospholipid antibody syndrome were found in 4 of 12 patients who underwent systematic examination. One patient had a history of lung cancer which may be associated with a hypercoagulable state and may explain the occurrence of myocardial infarction with a normal coronary artery. The mean left ventricle ejection was 56. 5±12. The mean follow-up was 24±10 months. Six patients developed residual chest pain which was generally easily controlled by anti-spastic therapy and no patient had a major cardiovascular event., Conclusion: Patients with Q-wave MI and with normal coronary arteries seem to have a good short and long-term prognosis especially when they are treated with an exclusive medical strategy.

Intracardiac thrombosis is an exceptional complication of Behçet's disease. The management of this involvement is difficult due to the risk of recurrence. We present the case of a young man admitted to our hospital for intermittent fever. The microbiologic investigations did not show any causative germ. We discovered a right ventricle thrombus on echocardiography. We confirmed the diagnosis of pulmonary embolism on CT angiogram. The patient developed oral and genital ulcerations which were consistent with Behçet's syndrome. The thrombus had disappeared after treatment with anticoagulant, corticosteroid and immunosuppressors. Intracardiac thrombosis can reveal Behçet's disease. An exhaustive examination and close monitoring should be performed in order to reveal pathognomonic signs as soon as possible and to promptly start the appropriate treatment.

This case report represents the difficulties in diagnosing brucellosis, which is an enigma with unusual cardiovascular complications. A 32-year-old Caucasian man with acute chest pain was examined at Sfax Hedi Chaker's Hospital. He had a night fever, although his cardiac examination was normal. Further laboratory analyses showed an elevated C-reactive protein of 20.8 mg/dL and troponine I of 1.469 IU/L. A cardiac MRI using delayed enhancement was then performed. The T2-weighted short-axis showed a subepicardial delaying enhancement of infero-lateral and the basal walls of the left ventricle. Accordingly, a diagnosis of Brucella-related myocarditis was made.

Introduction: Primary cardiac lymphoma is rare., Case Presentation: We report the case of a 64-year-old non-immunodeficient Caucasian man, with cardiac tamponade and paroxysmal third-degree atrioventricular block. Echocardiography revealed the presence of a large pericardial effusion with signs of tamponade and a right ventricular mass was suspected. Scanner investigations clarified the sites, extension and anatomic details of myocardial and pericardial infiltration. Surgical resection was performed due to the rapid impairment of his cardiac function. Analysis of the pericardial fluid and histology confirmed the diagnosis of non-Hodgkin large B-cell lymphoma. He was treated with chemotherapy., Conclusion: The prognosis remains poor for this type of tumor due to delays in diagnosis and the importance of the site of disease.