Dataset Information

Cardiac Evaluation using Two-Dimensional Speckle-Tracking Echocardiography and Conventional Echocardiography in Taiwanese Patients with Mucopolysaccharidoses.

ABSTRACT: BACKGROUND:Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic disorders that can damage various organs, including the heart. Cardiac abnormalities have been observed in patients with all MPS types, with the most documented abnormalities being cardiac valvular regurgitation and stenosis, valvular thickening, and hypertrophic cardiomyopathy. METHODS:Cardiac features of 53 Taiwanese patients with MPS (31 men and 22 women; age range 1.1-34.9 years; seven with MPS I, 16 with MPS II, nine with MPS III, 14 with MPS IVA, and seven with MPS VI) were evaluated using two-dimensional speckle-tracking echocardiography and conventional echocardiography. RESULTS:The mean z scores of the global longitudinal strain (GLS), left ventricular mass index (LVMI), interventricular septum diameter in diastole (IVSd), left ventricular posterior wall diameter in diastole (LVPWd), and aortic diameter of the 53 patients with MPS were 1.71, 0.35, 1.66, 1.03, and 3.15, respectively. Furthermore, z scores >2 were identified in 45%, 13%, 40%, 13%, and 70% of the GLS, LVMI, IVSd, LVPWd, and aortic diameter, respectively. The most severe GLS was observed in those with MPS VI, followed by in those with MPS II and MPS I. The GLS z score was positively correlated with the LVMI z score (p < 0.01). Moreover, diastolic dysfunction (reversed ratio between early and late (atrial) ventricular filling velocity (E/A ratio < 1)) was identified in 12 patients (23%). Ejection and shortening fractions were abnormal in four (8%) and seven (13%) patients, respectively. Mitral regurgitation (MR) (92%) was the most common valvular heart disease, followed by aortic regurgitation (AR) (57%), mitral stenosis (MS) (21%), and aortic stenosis (AS) (15%). The z scores of the GLS and LVMI and severity scores of the MS, MR, AS, and AR were all positively correlated with increasing age (p < 0.05). Twenty patients (38%) had a left ventricular remodeling pattern. CONCLUSIONS:The most significant left ventricular myocardial deformation, hypertrophy and valvular heart disease were observed in the patients with MPS VI, II, and I, followed by those with MPS IV; in contrast, patients with MPS III had the mildest manifestations. Cardiac abnormalities in patients with MPS worsened with increasing age in accordance with the progressive nature of this disease.

SUBMITTER: Lin HY

PROVIDER: S-EPMC7168914 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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Cardiac Evaluation using Two-Dimensional Speckle-Tracking Echocardiography and Conventional Echocardiography in Taiwanese Patients with Mucopolysaccharidoses.

Lin Hsiang-Yu HY Chuang Chih-Kuang CK Lee Chung-Lin CL Chen Ming-Ren MR Sung Kuo-Tzu KT Lin Shan-Miao SM Hou Charles Jia-Yin CJ Niu Dau-Ming DM Chang Tung-Ming TM Hung Chung-Lieh CL Lin Shuan-Pei SP

Diagnostics (Basel, Switzerland) 20200123 2

<h4>Background</h4>Mucopolysaccharidoses (MPSs) are a group of rare inherited metabolic disorders that can damage various organs, including the heart. Cardiac abnormalities have been observed in patients with all MPS types, with the most documented abnormalities being cardiac valvular regurgitation and stenosis, valvular thickening, and hypertrophic cardiomyopathy.<h4>Methods</h4>Cardiac features of 53 Taiwanese patients with MPS (31 men and 22 women; age range 1.1-34.9 years; seven with MPS I, ...[more]

PMID: 31979324

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Project description:ObjectivesWe aimed to investigate the association between right ventricular longitudinal strain measured by two-dimensional speckle-tracking echocardiography (2D-STE) and right heart catheterization data in pediatric patients with pulmonary hypertension (PH).MethodsTwo groups were evaluated, each consisting of 58 patients. Group 1, patients with PH; Group 2, normal matched controls. Data were collected from 58 patients with PH who underwent invasive hemodynamic evaluation. Standard transthoracic echocardiographic assessment was performed in all patients under the same circumstances. All patients underwent 2D-STE, and off-line analysis generated right ventricle longitudinal strain (RVLS) and right ventricular free wall strain (RVFW) and collected echocardiographic conventional parameters of right ventricular function, including the control group. The relationship between invasive characteristics and right ventricular function parameters was analyzed.ResultsIn all, 58 PH patients were included in our study. The mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) were strongly correlated with right ventricular free wall strain (RVFW) and right ventricular longitudinal strain (RVLS), moderately correlated with the right ventricle myocardial performance index (Tei index), weakly correlated with the transverse diameter of the right ventricle (RV) and the transverse diameter of the right atrium (RA), and moderately negatively correlated with right ventricular fractional area change (RVFAC). In terms of segments of the right ventricular free wall, the basal segment had the highest correlation coefficient with mPAP and PVR (r = 0.413, 0.523, 0.578, r = 0.421, 0.533, 0.575, p < 0.05, respectively). Tricuspid annular plane systolic excursion (TAPSE), main pulmonary artery diameter (MPA), peak systolic velocity of the right ventricle (RV-S'), and RA area parameters were not associated with mPAP and PVR (p > 0.05).ConclusionsRight ventricular longitudinal strain is a reliable indicator to evaluate right ventricular function in pediatric patients with PH. It can provide valuable reference information for the clinical judgment of the status and severity of the disease in children.

Project description:Iron-deficiency anemia (IDA) is a global health problem and a common medical condition that can be seen in everyday clinical practice. And two-dimensional speckle tracking echocardiography (2D-STE) has been reported very useful in evaluating left atrial (LA) function, as well as left ventricular (LV) function. The aim of our study is to evaluate the LA function in patients with IDA by 2D-STE.65 patients with IDA were selected. This group of patients was then divided into two groups according to the degree of hemoglobin: group B (Hb > 90 g/L) and group C (Hb60 ~ 90 g/L). Another 30 healthy people were also selected as control group A. Conventional echocardiography parameters, such as left atrial diameter (LAD), peak E and A of mitralis (E, A), E/A, end-diastolic thickness of ventricular septum (IVST d), end-diastolic thickness of LV posterior wall (PWTd) and left ventricular end-diastolic dimension (LVDd) were obtained from these three groups. Left atrial minimum volume (LAVmin), left atrial pre-atrial contraction volume (LAVp) and left atrial maximum volume (LAVmax) were measured by Simpson's rule, whereas left atrial active ejection fraction (LAAEF) and left atrial passive ejection fraction (LAPEF) were obtained from calculation. Two-dimensional images were acquired from apical four-chamber view and two-chamber view to store images for offline analysis. The global peak atrial longitudinal strain and strain rate of systolic LV (GLSs, GLSRs) as well as early and late diastolic LV strain rate (GLSRe, GLSRa) curves of LA were acquired in each LA segment from basal segment to top segment of LA by 2D-STE.Compared with group A, there were no differences between group B and group A (all P > 0.05). The LAAEF and GLSRa were significantly higher in group C compared with those of group A and group B (all P < 0.01). The LAPEF, GLSs, GLSRs and GLSRe were significantly lower in group C compared with those of group A and group B (all P < 0.01).2D-STE could evaluate the LA function in patients with IDA.

Project description:BackgroundLeft atrial (LA) size is often used as a surrogate marker of LA function in clinical practice, with larger atrial thought to represent a "dysfunctioning" atrium, since there is no accepted 'gold' standard to evaluate LA function. The exact relationship between LA size and phasic function, and whether LA dysfunction occur before LA enlargement (LAE) may be of clinical interest while have not been fully studied. Two-dimensional speckle-tracking echocardiography (2D STE) was showed a promising method in measuring LA physical deformation.Materials and methodsA community cohort of 715 subjects at cardiovascular disease high risk accepted comprehensive echocardiography. LA longitudinal phasic strain Sa (absolute peak strain during atrial contraction), Se (peak strain at early diastole) and Stot (total atrial strain = Sa + Se), representing contractile, conduit, and reservoir function respectively, were measured using off-line 2D STE software in apical 4 chamber view, and data were compared among groups at different LA size and between subgroups in normal LA size with and without hypertension (HT).ResultsWith LAE (from normal size, mild, moderate to severe LAE), the Stot (21.74 ± 5.97, 20.75 ± 4.99, 20.49 ± 5.27, 17.75 ± 4.71, respectively, ANOVA P = 0.003) and Sa (11.84 ± 3.92, 11.00 ± 3.29, 10.11 ± 2.57, 8.55 ± 2.88, respectively, ANOVA P < 0.001) reduced while Se had no change. Stot of Severe LAE group was significantly lower than that of Normal LA size group (P = 0.002). Sa of the three LAE groups were all significantly lower than that of Normal LA size group (P = 0.024, P = 0.002, P < 0.001, respectively). In normal sized LA subgroups, Stot (21.35 ± 5.91 vs. 23.01 ± 6.02, P = 0.008) and Se (9.51 ± 4.41 vs. 11.17 ± 4.89, P < 0.001) reduced in subjects with HT comparing with those without.ConclusionLA phasic function remodeling occurs before LAE and continues with LAE, with reservoir, conduit and contractile function being affected unparalleled.

Project description:BackgroundAs one of the important treatment methods for cancer patients, radiotherapy may lead to incidental irradiation of the heart, resulting in radiotherapy-induced heart disease (RIHD) arising many years after radiotherapy. While, there are few studies on early subclinical cardiac damage, which may be essential for the protection of late RIHD. To detect and predict RIHD and early subclinical cardiac damage induced by thoracic radiation therapy, based on two-dimensional speckle tracking echocardiography (2D STE) combined with multiple circulating biomarkers and accurate heart dosimetry.Methods and analysisThis is a monocentric prospective cohort study in which 104 patients treated for malignant tumors and with cardiac radiation exposure will be included. All participants will be followed for 12 months after radiotherapy. Echocardiography, 2D STE, and blood samples will be underwent at 5-time points (baseline; after completion of RT; 2, 6, and 12 months after RT). Left ventricular ejection fraction (LVEF); global longitudinal, radial, and circumferential strain; diastolic function parameters; creatine kinase (CK); creatine kinase isoenzyme (CK-MB); cardiac troponin T (cTnT); N-terminal pro-B-type natriuretic peptide (NT-proBNP) and hypersensitive C-reactive protein (hs-CRP) will be measured at baseline and every follow-up time. The incidence of major adverse cardiovascular events will be recorded.DiscussionThis study details the protocol and presents the primary limits and advantages of this single-center project. The inclusion of patients began in 2021, and the results are expected to be published in 2023. This study will be allowed to enhance knowledge on detection and prediction of early subclinical cardiac dysfunction induced by thoracic radiation therapy, based on two-dimensional speckle tracking echocardiography (2D STE) combined with circulating biomarkers and accurate heart dosimetry. Furthermore, we will evaluate risk factors of subtle cardiac damage and identify high-risk groups for early heart damage.Clinical trial registrationClinicalTrials.gov, identifier: NCT04443400.

Dataset Information

Cardiac Evaluation using Two-Dimensional Speckle-Tracking Echocardiography and Conventional Echocardiography in Taiwanese Patients with Mucopolysaccharidoses.

Publications

Cardiac Evaluation using Two-Dimensional Speckle-Tracking Echocardiography and Conventional Echocardiography in Taiwanese Patients with Mucopolysaccharidoses.

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