49 research outputs found

    Syndromic Thoracic Aortic Disease

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    Syndromic Thoracic Aortic Disease

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    Syndromic Thoracic Aortic Disease

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    The Prognostic Value of Myocardial Deformation in Patients with Congenital Aortic Stenosis

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    Aims: To assess the prognostic value of left ventricular (LV) global longitudinal strain (GLS) and global longitudinal early diastolic strain rate (GLSre) with regard to cardiovascular events, as congenital aortic stenosis (AoS) is associated with significant mortality and morbidity but predictors for clinical outcome are scarce. Strain analysis provides a robust and reproducible method for early detection of LV dysfunction, which might be of prognostic value. Methods: This prospective study, included clinically stable patients with congenital AoS between 2011– 2013. LV GLS and GLSre was performed in the apical 4, 3 and 2-chamber views using Tomtec software. The end-point was a composite of death, heart failure, hospitalization, arrhythmia, thrombo-embolic events and re-inter-vention. Results: In total 138 patients were included (33[26–43] years, 86(62%) male), NYHA class I: 134(97%). Mean LV GLS was –15.3 ± 3.2%, GLSre 0.66 ± 0.18 s–1. Both correlated with NT-proBNP, LV volumes and ejection fraction (strongest LV GLS with LV EF: r –0.539, p &lt; 0.001, strongest LV GLSre with age: r –0.376, p &lt; 0.001). During median follow-up of 5.9[5.5–6.2] years, the endpoint occurred in 53(38%) patients: 4 patients died, 9 developed heart failure, 22 arrhythmias, 8 thrombo-embolic events and 35 re-interventions. Both LV GLS (stan-dardized HR (sHR 0.62(95%CI 0.47–0.81) and GLSre (sHR 0.62(95%CI 0.47–0.83) were associated with the end-point. Additional multivariable analysis showed that both GLS and GLSre were associated independent of left atrial volume, NT-proBNP and prior re-interventions. Conclusion: Left ventricular GLS and GLSre are reduced in adult patients with congenital AoS. Both markers are associated with adverse cardiac events and have clear clinical relevance.</p

    Qualitative grading of aortic regurgitation: a pilot study comparing CMR 4D flow and echocardiography.

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    Over the past 10 years there has been intense research in the development of volumetric visualization of intracardiac flow by cardiac magnetic resonance (CMR).This volumetric time resolved technique called CMR 4D flow imaging has several advantages over standard CMR. It offers anatomical, functional and flow information in a single free-breathing, ten-minute acquisition. However, the data obtained is large and its processing requires dedicated software. We evaluated a cloud-based application package that combines volumetric data correction and visualization of CMR 4D flow data, and assessed its accuracy for the detection and grading of aortic valve regurgitation using transthoracic echocardiography as reference. Between June 2014 and January 2015, patients planned for clinical CMR were consecutively approached to undergo the supplementary CMR 4D flow acquisition. Fifty four patients(median age 39 years, 32 males) were included. Detection and grading of the aortic valve regurgitation using CMR4D flow imaging were evaluated against transthoracic echocardiography. The agreement between 4D flow CMR and transthoracic echocardiography for grading of aortic valve regurgitation was good (j = 0.73). To identify relevant,more than mild aortic valve regurgitation, CMR 4D flow imaging had a sensitivity of 100 % and specificity of 98 %. Aortic regurgitation can be well visualized, in a similar manner as transthoracic echocardiography, when using CMR 4D flow imaging

    Transthoracic 3D echocardiographic left heart chamber quantification in patients with bicuspid aortic valve disease

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    Integration of volumetric heart chamber quantification by 3D echocardiography into clinical practice has been hampered by several factors which a new fully automated algorithm (Left Heart Model, (LHM)) may help overcome. This study therefore aims to evaluate the feasibility and accuracy of the LHM software in quantifying left atrial and left ventricular volumes and left ventricular ejection fraction in a cohort of patients with a bicuspid aortic valve. Patients with a bicuspid aortic valve were prospectively included. All patients underwent 2D and 3D transthoracic echocardiography and computed tomography. Left atrial and ventricular volumes were obtained using t

    Left ventricular global longitudinal strain in bicupsid aortic valve patients: head-to-head comparison between computed tomography, 4D flow cardiovascular magnetic resonance and speckle-tracking echocardiography

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    Left ventricular global longitudinal strain (LVGLS) analysis is a sensitive measurement of myocardial deformation most often done using speckle-tracking transthoracic echocardiography (TTE). We propose a novel approach to measure LVGLS using feature-tracking software on the magnitude dataset of 4D flow cardiovascular magnetic resonance (CMR) and compare it to dynamic computed tomography (CT) and speckle tracking TTE derived measurements. In this prospective cohort study 59 consecutive adult patients with a bicuspid aortic valve (BAV) were included. The study protocol consisted of TTE, CT, and CMR on the same day. Image analysis was done using dedicated feature-tracking (4D flow CMR and CT) and speckle-tracking (TTE) software, on apical 2-, 3-, and 4-chamber long-axis multiplanar reconstructions (4D flow CMR and CT) or standard apical 2-, 3-, and 4-chamber acquisitions (TTE). CMR and CT GLS analysis was feasible in all patients. Good correlations were observed for GLS measured by CMR (− 21 ± 3%) and CT (− 20 ± 3%) versus TTE (− 20 ± 3%, Pearson’s r: 0.67 and 0.65, p 0.61, p < 0.001). Feature-tracking GLS analysis is feasible using the magnitude images acquired with 4D flow CMR. GLS measurement by CMR correlates well with CT and speckle-tracking 2D TTE. GLS analysis on 4D flow CMR allows for an integrative approach, integrating flow and functional data in a single sequence. Not applicable, observational study

    A value-based healthcare approach: Health-related quality of life and psychosocial functioning in women with Turner syndrome

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    Objective: As part of the value-based healthcare programme in our hospital, a set of patient-reported outcome measures was developed together with patients and implemented in the dedicated Turner Syndrome (TS) outpatient clinic. This study aims to investigate different aspects of health-related quality of life (HR-QoL) and psychosocial functioning in women with TS in order to establish new possible targets for therapy. Design/Participants: A comprehensive set of questionnaires (EQ-5D, PSS-10, CIS-20, Ferti-QoL, FSFI) was developed and used to capture different aspects of HR-QoL and psychosocial functioning in a large cohort of adult women with Turner syndrome. All consecutive women, ≥18 years, who visited the outpatient clinic of our tertiary centre were eligible for inclusion. Results: Of the eligible 201 women who were invited to participate, 177 women (age 34 ± 12 years, mean ± SD) completed at least one of the validated questionnaires (88%). Women with TS reported a lower health-related quality of life (EQ-5D: 0.857 vs 0.892, P =.003), perceived more stress (PSS-10:14.7 vs 13.3; P =.012) and experienced increased fatigue (CIS-20: P <.001) compared to the general Dutch population. A relationship between noncardiac comorbidities (eg diabetes, orthopaedic complaints) and HR-QoL was found (R =.508). Conclusions: We showed that TS women suffer from impaired HR-QoL, more perceived stress and increased fatigue compared to healthy controls. A relationship between noncardiac comorbidities and HR-QoL was found. Especially perceived stress and increased fatigue can be considered targets for improvement of HR-QoL in TS women
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