0000000000147929

AUTHOR

Gilbert Habib

Phenotype/Genotype Relationship in Left Ventricular Noncompaction: Ion Channel Gene Mutations Are Associated With Preserved Left Ventricular Systolic Function and Biventricular Noncompaction

International audience; Background: Few data exist concerning genotype-phenotype relationships in left ventricular noncompaction (LVNC).Methods and results: From a multicenter French Registry, we report the genetic and clinical spectrum of 95 patients with LVNC, and their genotype-phenotype relationship. Among the 95 LVNC, 45 had at least 1 mutation, including 14 cases of mutation in ion channel genes. In a complementary analysis including 16 additional patients with ion channel gene mutations, for a total of 30 patients with ion channel gene mutation, we found that those patients had higher median LV ejection fraction (60% vs 40%; P < .001) and more biventricular noncompaction (53.1% vs 18…

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Echocardiographic reference ranges for normal left ventricular 2D strain: results from the EACVI NORRE study

International audience; Aims - To obtain the normal ranges for 2D echocardiographic (2DE) measurements of left ventricular (LV) strain from a large group of healthy volunteers accounting for age and gender. Methods and results - A total of 549 (mean age: 45.6 ± 13.3 years) healthy subjects were enrolled at 22 collaborating institutions of the Normal Reference Ranges for Echocardiography (NORRE) study. 2DE data sets have been analysed with a vendor-independent software package allowing homogeneous measurements irrespective of the echocardiographic equipment used to acquire the data sets. The lowest expected values of LV strains and twist calculated as ± 1.96 standard deviations from the mean…

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Normal Reference Ranges for Echocardiography: Rationale, study design, and methodology (NORRE Study)

International audience; BACKGROUND: Availability of normative reference values for cardiac chamber dimensions, volumes, mass, and function is a prerequisite for the accurate application of echocardiography for both clinical and research purposes. However, due to the lack of consistency in current echocardiographic 'reference values', their use for clinical decision-making remains questionable. AIMS: The aim of the 'Normal Reference Ranges for Echocardiography Study (NORRE Study)' is to obtain a set of 'normal values' for cardiac chamber geometry and function in a large cohort of healthy Caucasian individuals aged over a wide range of ages (25-75 years) using both conventional and advanced e…

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Outcomes of Patients With Asymptomatic Aortic Stenosis Followed Up in Heart Valve Clinics

International audience; Importance - The natural history and the management of patients with asymptomatic aortic stenosis (AS) have not been fully examined in the current era. Objective - To determine the clinical outcomes of patients with asymptomatic AS using data from the Heart Valve Clinic International Database. Design, setting, and participants - This registry was assembled by merging data from prospectively gathered institutional databases from 10 heart valve clinics in Europe, Canada, and the United States. Asymptomatic patients with an aortic valve area of 1.5 cm2 or less and preserved left ventricular ejection fraction (LVEF) greater than 50% at entry were considered for the prese…

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Cancer and Infective Endocarditis: Characteristics and Prognostic Impact

Background: The interplay between cancer and IE has become of increasing interest. This study sought to assess the prevalence, baseline characteristics, management, and outcomes of IE cancer patients in the ESC EORP EURO-ENDO registry.Methods: Three thousand and eighty-five patients with IE were identified based on the ESC 2015 criteria. Three hundred and fifty-nine (11.6%) IE cancer patients were compared to 2,726 (88.4%) cancer-free IE patients.Results: In cancer patients, IE was mostly community-acquired (74.8%). The most frequently identified microorganisms were S. aureus (25.4%) and Enterococci (23.8%). The most frequent complications were acute renal failure (25.9%), embolic events (2…

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Prognosis of adults with left ventricular non compaction: Results from a prospective multricentric french study

Background Left ventricular non compaction (LVNC) is a rare cause of cardiomyopathy related to abnormal in utero myocardial development, leading to prominent trabeculations. Its prognosis remains unclear with conflicting data and mortality ranging from 2 to 38%. Purpose The aim of this study was to define the prognosis of newly diagnosed LVNC as compared to recently diagnosed dilated cardiomyopathy (DCM). Methods A prospective multicentric study with a 2-year follow-up was designed. The primary endpoint combined cardiovascular death, heart transplantation and hospitalisation for cardiovascular events. Three groups of patients were defined: Group A: new cases of LVNC, Group B: new cases of D…

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3D echocardiographic reference ranges for normal left ventricular volumes and strain: results from the EACVI NORRE study

International audience; Aim: To obtain the normal ranges for 3D echocardiography (3DE) measurement of left ventricular (LV) volumes, function, and strain from a large group of healthy volunteers. Methods and results: A total of 440 (mean age: 45 ± 13 years) out of the 734 healthy subjects enrolled at 22 collaborating institutions of the Normal Reference Ranges for Echocardiography (NORRE) study had good-quality 3DE data sets that have been analysed with a vendor-independent software package allowing homogeneous measurements regardless of the echocardiographic machine used to acquire the data sets. Upper limits of LV end-diastolic and end-systolic volumes were larger in men (97 and 42 mL/m2)…

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Two-dimensional transthoracic echocardiographic normal reference ranges for proximal aorta dimensions: Results from the EACVI NORRE study

International audience; AIMS: To report normal reference ranges for echocardiographic dimensions of the proximal aorta obtained in a large group of healthy volunteers recruited using state-of-the-art cardiac ultrasound equipment, considering different measurement conventions, and taking into account gender, age, and body size of individuals. METHODS AND RESULTS: A total of 704 (mean age: 46.0 ± 13.5 years) healthy volunteers (310 men and 394 women) were prospectively recruited from the collaborating institutions of the Normal Reference Ranges for Echocardiography (NORRE) study. A comprehensive echocardiographic examination was obtained in all subjects following pre-defined protocols. Aortic…

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Aortic Function's Adaptation in Response to Exercise-Induced Stress Assessing by 1.5T MRI : A Pilot Study in Healthy Volunteers

AIM:Evaluation of the aortic "elastic reserve" might be a relevant marker to assess the risk of aortic event. Our aim was to compare regional aortic elasticity at rest and during supine bicycle exercise at 1.5 T MRI in healthy individuals. METHODS:Fifteen volunteers (8 men), with a mean age of 29 (23-41) years, completed the entire protocol. Images were acquired immediately following maximal exercise. Retrospective cine sequences were acquired to assess compliance, distensibility, maximum rates of systolic distension and diastolic recoil at four different locations: ascending aorta, proximal descending aorta, distal descending aorta and aorta above the coeliac trunk level. Segmental aortic …

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NCprocessing: a software to determine non-compacted and compacted masses from MRI

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Semiautomatic detection of myocardial contours in order to investigate normal values of the left ventricular trabeculated mass using MRI

Purpose To propose, assess, and validate a semiautomatic method allowing rapid and reproducible measurement of trabeculated and compacted left ventricular (LV) masses from cardiac magnetic resonance imaging (MRI). Materials and Methods We developed a method to automatically detect noncompacted, endocardial, and epicardial contours. Papillary muscles were segmented using semiautomatic thresholding and were included in the compacted mass. Blood was removed from trabeculae using the same threshold tool. Trabeculated, compacted masses and ratio of noncompacted to compacted (NC:C) masses were computed. Preclinical validation was performed on four transgenic mice with hypertrabeculation of the LV…

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Hypertrophic cardiomyopathy and fibrosis: correlation between late gadolinium enhancement on CMR and speckle tracking imaging using Ultrasound

Background Hypertrophic cardiomyopathy (HCM) is the most frequent genetic cardiovascular disorder and represents one of the most common cause of heart related sudden death in young adults. Myocardial fibrosis seems to be an independant predictor of adverse events including sudden death, ventricular arrhythmias and heart failure. While late gadolinium enhancement (LGE) on Cardiac Magnetic Resonance (CMR) is actually the gold-standard to detect fibrosis, new techniques are being evaluated such as 2D strain echocardiography.

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