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Background: Familial hypercholesterolaemia (FH) is responsible for severe hypercholesterolaemia and premature cardiovascular morbidity and mortality. The first clinical event is typically an acute coronary syndrome. Unfortunately, FH is largely underdiagnosed in the general population., Aims: To assess the prevalence of clinical FH among patients with premature (aged≤50 years) acute myocardial infarction (MI) and compare it with FH prevalence in a control population., Methods: We reviewed in our database all patients with premature MI (aged≤50 years) referred to Ambroise Paré Hospital from 2014 to 2018. FH prevalence was estimated via the Dutch Lipid Clinic Network score, based on personal and family history of premature cardiovascular disease and low-density lipoprotein cholesterol concentrations. FH was "possible" with a score between 3 and 5 points, "probable" with a score between 6 and 8 and "definite" with a score above 8. FH prevalence in young patients with MI was then compared with FH prevalence in a general population of the same age from the CARVAR 92 prospective cohort., Results: Of the 457 patients with premature MI, 29 (6%) had "probable" or "definite" FH. In the CARVAR 92 cohort, 16 (0.16%) of 9900 subjects aged≤50 years had "probable" or "definite" FH. FH prevalence was 39 times greater among patients with premature MI than in the control population (P<0.0001). In multivariable analysis, FH was strongly associated with MI (adjusted odds ratio 38.4, 95% confidence interval 19.1-79.4)., Conclusions: FH is>30-fold more common in patients referred for premature MI than in the general population; this highlights the need for FH screening after a first MI to enhance lipid-lowering therapy and allow early identification of family members., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Multimodality imaging plays an important role in the initial evaluation, diagnosis and management of patients suspected of having a cardiomyopathy. Beyond functional and anatomical information, multimodality imaging provides important variables that facilitate risk stratification and prognosis evaluation. Whatever the underlying suspected cardiomyopathy, echocardiography is the most common initial imaging test used to establish the presence of cardiomyopathy, by depicting structural and functional abnormalities. However, echocardiographic findings are non-specific, and therefore have a limited role in identifying the underlying aetiology. Cardiac magnetic resonance imaging allows characterization of myocardial tissue, which can be of great help in identifying the aetiology of the cardiomyopathy. When a specific aetiology is suspected, particularly inflammation, 18 F-fluorodeoxyglucose positron emission tomography is recommended. The clinician should be capable of selecting the appropriate imaging techniques for each clinical scenario. Each technique has strengths and weaknesses, which should be known. In order to improve diagnostic performance, and as proposed by the European Association for Cardiovascular Imaging, cardiovascular imaging groups must be composed of experts from all modalities. The future of multimodality imaging in the diagnosis and management of cardiomyopathies will also involve evolution of its use in care, teaching and research. Training goals for future cardiac imaging experts must be defined; academic and industry partnerships should enable the connection to be made between imaging data and clinical data on the one hand and outcomes on the other hand, using big-data analysis and artificial intelligence., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Takotsubo cardiomyopathy preferentially affects postmenopausal women (# 90%). Takotsubo cardiomyopathy mimics an acute coronary syndrome and is defined as a transient left ventricular systolic dysfunction. Diagnosis is based on the criteria of the Mayo Clinic. Stress is found in around 80% of cases: physical stress, emotional stress, medical stress. Physical stress is less found in women. Incidence of Takotsubo cardiomyopathy is 54.9/1,000,000 inhabitants in women versus only 3.6/1,000,000 inhabitants in men. Diagnosis is based on electrocardiogram, biomarkers, echocardiography, coronary angiography and left ventricular angiography, and cardiac magnetic imaging. The complete recovery of left ventricular systolic function must be assessed, allowing to confirm the diagnosis of Takotsubo cardiomyopathy., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Hospitalization for worsening/acute heart failure is increasing in France, and limited data are available on referral/discharge modalities., Aim: To evaluate patients' journeys before and after hospitalization for this condition., Methods: On 1 day per week, between October 2014 and February 2015, this observational study enrolled 260 consecutive patients with acute/worsening heart failure in all 10 departments of cardiology and four of the departments of geriatrics in the Greater Paris University Hospitals., Results: First medical contact was an emergency unit in 45% of cases, a general practitioner in 16% of cases, an emergency medical ambulance in 13% of cases and a cardiologist in 13% of cases; 78% of patients were admitted directly after first medical contact. In-hospital stay was 13.2±11.3 days; intensive care unit stay (38% of the population) was 6.4±5 days. In-hospital mortality was 2.7%. Overall, 63% of patients were discharged home, whereas 21% were transferred to rehabilitation units. A post-discharge outpatient visit was made by only 72% of patients within 3 months (after a mean of 45±28 days). Only 53% of outpatient appointments were with a cardiologist., Conclusion: Emergency departments, ambulances and general practitioners are the main points of entry before hospitalization for acute/worsening heart failure. Home discharge occurs in two of three cases. Time to first patient post-discharge visit is delayed. Therefore, actions to improve the patient journey should target primary care physicians and emergency structures, and efforts should be made to reduce the time to the first visit after discharge., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Patients with hypertrophic cardiomyopathy (HCM) are at an increased risk of death from many causes and sudden cardiac death is one of them. The study of the sudden cardiac death of patients with HCM has allowed the identification of risk factors and among them major risk factor are: family history of sudden cardiac death, the occurrence of syncope/dizziness, the existence of non-sustained ventricular tachycardia an abnormal blood pressure response during stress test, presence of severe left ventricular hypertrophy≥30mm. Risk stratification for sudden cardiac death is essential, for symptomatic or asymptomatic HCM patients. Two approaches are possible: the classical approach or risk stratification methods with major risk factors and the new approach using the risk-calculator recommended by the ESC. Both methods are not in opposition but complementary. The risk stratification in hypertrophic cardiomyopathy should be still improved to be sure that only the most high-risk patients receive an implantable cardiac defibrillator., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Background: Takotsubo cardiomyopathy (TTC) continues to be under-diagnosed, due to its varying presentation, with potentially serious consequences if treatment is delayed., Aims: To demonstrate the consistent involvement of catecholaminergic stress in TTC, regardless of the trigger., Methods: Between 01 July 2009 and 31 August 2013, patients managed in our centre for thoracic pain syndrome, with or without troponin release, were followed up prospectively. TTC was diagnosed from the apical ballooning seen on left ventricular imaging (angiography or transthoracic echocardiography) in the absence of a significant coronary artery lesion. Triggers (emotional trauma, surgical stress and β2-mimetic intoxication) were recorded; catecholamine-secreting tumours were screened for with a urinary methoxylate-derivative assay., Results: TTC was diagnosed in 40 out of 2754 (1.5%) patients with thoracic pain syndrome, with or without troponin release. Triggers were emotional trauma (n=29, 72.5%), surgical stress (n=5, 12.5%), adrenergic intoxication (n=3, 7.5%) and catecholaminergic tumour (n=3, 7.5%). Mean left ventricular ejection fraction at admission was 38.0 ± 15.7%. Eight (20%) patients initially showed cardiogenic shock. In-hospital mortality was 7.5%, with no deaths from cardiogenic causes. Thirty-five (94.6%) of the survivors had recovered a normal left ventricular ejection fraction (> 55%) by discharge., Conclusion: Whatever the trigger, the common denominator in TTC is catecholaminergic stress. Classically suggested after emotional trauma, TTC may also be induced by surgical stress or endogenous or iatrogenic β2-mimetic intoxication. The various contexts all have a similarly excellent cardiovascular prognosis if treated early., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Tako-Tsubo cardiomyopathy usually mimics an acute coronary syndrome and is defined as a transient left ventricular systolic dysfunction. Stress is frequently present, but is not systematically found. Diagnosis is based on the criteria of the Mayo-Clinic. Tako-Tsubo cardiomyopathy preferentially affects postmenopausal women. Several explorations are performed to confirm the diagnosis: electrocardiogram, biomarkers, echocardiography, coronary angiography and left ventricular angiography, and MRI. Left ventricular dysfunction is pathognomonic, with circular akinesia regarding the different mid and apical segments of the left ventricle in its typical form. Complications may occur in the acute phase and should be systematically assessed. The complete recovery of left ventricular systolic dysfunction is the rule and must be assessed, allowing to confirm the diagnosis of Tako-Tsubo cardiomyopathy., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: An accurate assessment of left ventricular (LV) mass is important for the detection of LV hypertrophy., Aims: To assess the accuracy of four echocardiographic imaging modalities for assessing LV mass compared with cardiac magnetic resonance (CMR)., Methods: We prospectively studied 40 consecutive patients, who underwent an echocardiographic examination using four imaging modalities (M-mode fundamental imaging [FI], M-mode harmonic imaging [HI], two-dimensional [2D] FI and 2D HI) and CMR (our gold standard for LV mass measurement). All echocardiographic measurements were performed by two independent observers., Results: All echocardiographic modes significantly overestimated LV mass compared with CMR (P≤0.04), except 2D FI (P=0.25). This overestimation was significantly higher with HI (up to 15.5%) compared with FI (up to 5.7%; P≤0.04). Significant correlations were observed between the different echocardiographic methods and the two observers. The interobserver agreement over LV mass measurement was lower with FI (intraclass coefficient [ICC] range, 0.66-0.73) than with HI (ICC range, 0.72-0.82), and the best agreement was obtained with 2D HI (ICC, 0.82). Good agreement between CMR and all echocardiographic methods was observed among the smallest LV diameters (ICC range, 0.62-0.85), but not among the largest LV diameters (ICC range, 0-0.22)., Conclusions: HI overestimates LV mass compared with FI and CMR; this leads to overestimation of prevalence of LV hypertrophy in a population of hypertensive patients. HI improves interobserver reproducibility of LV mass measurement compared with FI, leading to a significant decrease in the number of patients required for clinical trials evaluating LV mass regression. Accuracy of LV mass measurement by echocardiography is affected by LV geometry., (Copyright © 2011. Published by Elsevier Masson SAS.)

Background: Tako-Tsubo cardiomyopathy is a clinical entity mimicking acute coronary syndrome. Assessment of left ventricular function may be difficult using conventional echocardiography., Aims: to characterize left ventricular systolic function using contrast echocardiography in Tako-Tsubo cardiomyopathy., Methods: We prospectively studied 63 consecutive women admitted for suspected acute coronary syndrome who underwent coronary arteriography, biplane left ventricular angiography and conventional and contrast echocardiography; 25 women had Tako-Tsubo cardiomyopathy (group 1), 25 women had proven coronary artery disease (group 2) and 13 women had no significant coronary lesion (group 3). Echocardiographic interpretation was performed by two observers: a physician trainee (observer 1) and an experienced investigator (observer 2)., Results: Left ventricular segments were assessed for wall motion abnormalities, which were present in 70 and 88% (observer 1) and in 91 and 99% (observer 2), using conventional and contrast echocardiography, respectively (P<0.0001). Accuracy for the diagnosis of Tako-Tsubo cardiomyopathy was improved significantly for both observers using contrast echocardiography: for observer 1, sensitivity was 56 and 88%, respectively, using conventional and contrast echocardiography (P=0.01), whereas for observer 2, sensitivity was 72 and 96%, respectively (P=0.04). Interobserver agreement was excellent using contrast agent (kappa=0.85 vs 0.34 using conventional echocardiography). The blinded review of left ventriculograms distinguished Tako-Tsubo cardiomyopathy from coronary artery disease correctly in 96% of cases., Conclusions: Contrast echocardiography could be used in routine practice to replace left ventricular angiography in Tako-Tsubo cardiomyopathy., (2010 Elsevier Masson SAS. All rights reserved.)

Background: Several patterns of Tako-Tsubo cardiomyopathy (TTC) have been described recently., Aims: To assess the prevalence and characteristics of an apical-sparing variant of TTC., Methods: This study included consecutive patients admitted to our catheterization laboratory for suspected acute coronary syndrome (ACS). All patients underwent coronary and left ventricular angiography systematically if no significant coronary lesions were found., Results: Among 2893 patients with a suspected ACS, 38 had confirmed TTC. Nine patients presented with the apical-sparing variant, resulting in a 24% prevalence in our TTC population. At admission, mean left ventricular ejection fraction (LVEF) was significantly higher in patients with apical-sparing TTC (45+/-4% vs 35+/-7%, p=0.01). Patients with classic TTC were significantly older (74+/-10 years vs 63+/-14 years, p=0.01) and had a significantly higher mean heart rate and New York Heart Association functional class (p=0.04 and p=0.002, respectively). Surgical or disease-related stress was found more frequently among patients with the apical-sparing variant (p=0.02). At day 7, mean LVEF was significantly higher in patients with apical-sparing TTC (55+/-6% vs 48+/-6%, p=0.04). At 1-month and 1-year follow-up, no significant difference in LVEF was observed between the two patterns of TTC (p=0.60 and p=0.46, respectively)., Conclusions: The apical-sparing variant of TTC is not rare and differs in several ways from the classic pattern of TTC. Physicians should be aware of and recognize this partial pattern of TTC.

Background: Conventional echocardiography is not a reliable method for characterizing tissue patterns of intracardiac masses., Aims: To assess the ability of contrast echocardiography to characterize intracardiac masses., Methods: Thirty-one consecutive patients with an intracardiac mass were included in this prospective study. All patients underwent conventional and contrast echocardiographic examinations. Analysis of characteristics by contrast agent allowed classification of intracardiac masses as follows: complete lack of enhancement, suggesting thrombus; partial and/or incomplete enhancement, suggesting myxoma; complete enhancement, suggesting intracardiac tumor. Tissue characteristics of intracardiac masses were also analyzed using at least one of the following techniques: cardiac magnetic resonance, pathology of intracardiac mass and/or mass resolved after anticoagulation during follow-up., Results: Using contrast echocardiography, an accurate diagnosis was made in all patients by an experienced investigator and in all patients except one (97%), by a physician trainee (p=0.31). Among patients with a history of tumor, echocardiography allowed accurate diagnosis of the nature of the mass in all patients; 50% of these patients presented with a secondary cardiac tumor and the others had a thrombus. Of the 14patients with a thrombus located in the left ventricle, 12 (86%) presented with left ventricular motion abnormalities using conventional echocardiography, whereas wall motion abnormalities were observed in all 14patients (100%) using contrast agent. In these patients, 91 and 99% of left ventricular segments were well visualized using conventional and contrast echocardiography, respectively (p<0.0001)., Conclusions: Contrast echocardiography may be useful for the tissue characterization of intracardiac masses.

Aim: To estimate the risk of venous thrombosis associated with pancreatic adenocarcinoma and its consequences on treatment and survival., Patients and Methods: We retrospectively analyzed a cohort of 90 patients (49 males, 41 females - median age: 67 years [range: 37-94]). Pancreatic adenocarcinoma was histologically proved in 72 patients (81%) and was metastatic in 49 patients (54.4%). A venous thrombosis was observed in 24 patients (26.7%). A pulmonary embolism occurred in 4 patients with 2 deaths. The risk of venous thrombosis was significantly reduced by the use of anti-thrombotic prophylaxis (HR: 0.03 [95CI: 0.003-0.27]) and increased among patients with a biological inflammatory syndrome (HR: 9.0 [95CI: 2.30-34.4]) and metastatic disease (HR: 4.4 [95CI: 1.1-17.9]). Overall survival was not different between patients with (6.6 months) or without (6.1 months) venous thrombosis., Conclusion: The risk of venous thrombosis is important and may delay the treatment in patients with advanced pancreatic carcinoma. Some patients with high risk of venous thrombosis may benefit from a prophylactic anticoagulant treatment.

Objective: Ultrasounds are a useful tool when looking for indirect evidence in favor of pulmonary embolism. The aim of this study was to determine the incidence of acute cor pulmonale and deep venous thrombosis revealed by ultrasonographic techniques in a population of patients presenting with pulmonary embolism., Methods: 96 consecutive patients with a mean (+/- SD) age of 65 +/- 15 years, admitted to our hospital for pulmonary embolism were included in this study. The diagnosis of pulmonary embolism was made either by spiral computed tomography or selective pulmonary angiography. Each patient subsequently underwent both trans-thoracic echocardiography and venous ultrasonography. The diagnostic criterion used for defining acute cor pulmonale by echocardiography was the right to left ventricular end-diastolic area ratio over (or equal to) 0.6. Diagnosis of deep venous thrombosis was supported by the visualization of thrombi or vein incompressibility and/or the absence of venous flow or loss of flow variability by venous ultrasonography., Results: Using ultrasounds, an acute cor pulmonale was found in 63% of our patients while 79% were found to have deep venous thrombosis and 92% of the patients had either acute cor pulmonale or deep venous thrombosis or both. All of the patients with proximal pulmonary embolism had acute cor pulmonale and/or deep venous thrombosis. The presence of acute cor pulmonale on echocardiography was significantly higher in patients with proximal pulmonary embolism (p < 0.0001)., Conclusion: This study emphasizes the potential value of ultrasonographic techniques in the diagnosis of acute pulmonary embolism.