Dataset on the use of 3D speckle tracking echocardiography in light-chain amyloidosis.
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ABSTRACT:
SUBMITTER: Vitarelli A
PROVIDER: S-EPMC5996947 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Brugada syndrome is characterized by typical electrocardiogram changes and a high risk for sudden cardiac death (Priori et al., 2013). In addition to the well known electrical substrate, morphological and functional alterations appeared to be present in a subset of the Brugada syndrome patients (Catalano et al., 2009). Echocardiographic speckle tracking enables us to detect subtle contraction alterations (Smiseth et al.,2016). We performed transthoracic echocardiography with speckle tracking analysis in 82 healthy controls and 175 Brugada syndrome patients. Main findings are presented and discussed in the article "Contraction alterations in Brugada syndrome; association with life-threatening ventricular arrhythmias" (Scheirlynck et al., 2019). This related Data article contains segmental longitudinal strain values for RV and LV, and the comparison of echocardiographic parameters between Brugada syndrome patients with spontaneous and drug-induced type 1 pattern and between patients with and without ventricular arrhythmia inducibility during electrophysiological study.
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Project description:Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of dialysis is predicted by the severity of renal involvement, defined by the baseline proteinuria and glomerular filtration rate, and by the response to therapy. The specific treatment is chemotherapy targeting the underlying plasma-cell clone. It needs to be risk-adapted, according to the severity of cardiac and/or multi-organ involvement. Autologous stem cell transplant (preceded by induction and/or followed by consolidation with bortezomib-based regimens) can be considered for low-risk patients (~20%). Bortezomib combined with alkylators is used in the majority of intermediate-risk patients, and with possible dose escalation in high-risk subjects. Novel, powerful anti-plasma cell agents were investigated in the relapsed/refractory setting, and are being moved to upfront therapy in clinical trials. In addition, the use of novel approaches based on antibodies targeting the amyloid deposits or small molecules interfering with the amyloidogenic process gave promising results in preliminary studies. Some of them are under evaluation in controlled trials. These molecules will probably add powerful complements to standard chemotherapy. The understanding of the specific molecular mechanisms of cardiac damage and the characteristics of the amyloidogenic clone are unveiling novel potential treatment approaches, moving towards a cure for this dreadful disease.
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Project description:BACKGROUND:To evaluate the possibility of left atrial (LA) remodeling using speckle tracking echocardiography (STE) in patients with interatrial block (IAB). METHODS:We performed a cross-sectional study with three groups of patients: 56 without IAB, 21 with partial IAB (pIAB), and 22 with advanced IAB (aIAB). Transthoracic echocardiographic (TTE) STE was performed and clinical and echocardiographic findings were analyzed. RESULTS:TTE showed higher LA volume/body surface area in the patients with IAB. With STE, the absolute value of strain rate during atrial booster pump function (SRa) and early reservoir period (SRs) decreased in the pIAB group and even more in the aIAB group, compared to the group without IAB. The independent variables were the echocardiographic measures of LA size and function. After adjusting for confounders, both multiple linear regression and multivariate multinomial regression showed good correlation with dependent variables: longer P-wave duration on electrocardiography and with the type of IAB, respectively. SRa (p?<?0.001), SRs (p?<?0.001), and maximal peak LA longitudinal strain in the reservoir period (p?=?0.009) were independently associated with P-wave duration. SRa was also associated with the presence of pIAB (OR?=?11.5; 95% confidence interval (CI): 2.7-49.0; p?=?0.001) and aIAB, (OR?=?98.2; 95% CI: 16-120.4; p?<?0.001) and SRs was associated with pIAB (OR: 0.03; CI: 0.003-0.29; p?=?0.003) and with aIAB (OR: 0.008; CI: 0.001-0.12; p?=?0.004). CONCLUSIONS:IAB correlates directly with structural remodeling and a decrease in the absolute value of LA SRa and SRs determined using STE.
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Project description:Two-dimensional (2D) strain analysis is constrained by geometry-dependent reference directions of deformation (i.e. radial, circumferential, and longitudinal) following the assumption of cylindrical chamber architecture. Three-dimensional (3D) principal strain analysis may overcome such limitations by referencing intrinsic (i.e. principal) directions of deformation. This study aimed to demonstrate clinical feasibility of 3D principal strain analysis from routine 2D cine MRI with validation to strain from 2D tagged cine analysis and 3D speckle tracking echocardiography. Thirty-one patients undergoing cardiac MRI were studied. 3D strain was measured from routine, multi-planar 2D cine SSFP images using custom software designed to apply 4D deformation fields to 3D cardiac models to derive principal strain. Comparisons of strain estimates versus those by 2D tagged cine, 2D non-tagged cine (feature tracking), and 3D speckle tracking echocardiography (STE) were performed. Mean age was 51?±?14 (36% female). Mean LV ejection fraction was 66?±?10% (range 37-80%). 3D principal strain analysis was feasible in all subjects and showed high inter- and intra-observer reproducibility (ICC range 0.83-0.97 and 0.83-0.98, respectively-p?<?0.001 for all directions). Strong correlations of minimum and maximum principal strain were respectively observed versus the following: 3D STE estimates of longitudinal (r?=?0.81 and r?=?-0.64), circumferential (r?=?0.76 and r?=?-0.58) and radial (r?=?-0.80 and r?=?0.63) strain (p?<?0.001 for all); 2D tagged cine estimates of longitudinal (r?=?0.81 and r?=?-0.81), circumferential (r?=?0.87 and r?=?-0.85), and radial (r?=?-0.76 and r?=?0.81) strain (p?<?0.0001 for all); and 2D cine (feature tracking) estimates of longitudinal (r?=?0.85 and -0.83), circumferential (r?=?0.88 and r?=?-0.87), and radial strain (r?=?-0.79 and r?=?0.84, p?<?0.0001 for all). 3D principal strain analysis is feasible using routine, multi-planar 2D cine MRI and shows high reproducibility with strong correlations to 2D conventional strain analysis and 3D STE-based analysis. Given its independence from geometry-related directions of deformation this technique may offer unique benefit for the detection and prognostication of myocardial disease, and warrants expanded investigation.
| S-EPMC5698377 | biostudies-literature
Project description:Light chain amyloidosis (AL) is a rare but often fatal disease due to intractable heart failure. Amyloid deposition leads to diastolic dysfunction and often preserved ejection fraction. We hypothesize that AL is associated with regional systolic dyssynchrony. The aim is to compare left ventricular (LV) regional synchrony in AL subjects versus healthy controls using 16-segment dyssynchrony index measured from 3-dimension-al (3D) echocardiography.Cardiac 3D echocardiography full volumes were acquired in 10 biopsy-proven AL subjects (60 +/- 3 years, 5 females) and 10 healthy controls (52 +/- 1 years, 5 females). The LV was subdivided into 16 segments and the time from end-diastole to the minimal systolic volume for each of the 16 segments was expressed as a percent of the cycle length. The standard deviations of these times provided a 16-segment dyssynchrony index (16-SD%). 16-SD% was compared between healthy and AL subjects.Left ventricular ejection fraction was comparable (control vs. AL: 62.4 +/- 0.6 vs. 58.6 +/- 2.8%, p = NS). 16-SD% was significantly higher in AL versus healthy subjects (5.93 +/- 4.4 vs. 1.67 +/- 0.87%, p = 0.003). 16-SD% correlated with left ventricular mass index (R 0.45, p = 0.04) but not to left ventricular ejection fraction.Light chain amyloidosis is associated with left ventricular regional systolic dyssynchrony. Regional dyssynchrony may be an unrecognized mechanism of heart failure in AL subjects.
| S-EPMC2525629 | biostudies-literature
Project description: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.
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Project description:Left ventricular (LV) contraction is characterized by shortening and thickening of longitudinal and circumferential fibres. To date, it is poorly understood how LV deformation is altered in the pathogenesis of streptozotocin (STZ)-induced type 1 diabetes mellitus-associated diabetic cardiomyopathy and how this is associated with changes in cardiac structural composition. To gain further insights in these LV alterations, eight-week-old C57BL6/j mice were intraperitoneally injected with 50 mg/kg body weight STZ during 5 consecutive days. Six, 9, and 12 weeks (w) post injections, echocardiographic analysis was performed using a Vevo 3100 device coupled to a 30-MHz linear-frequency transducer. Speckle-tracking echocardiography (STE) demonstrated impaired global longitudinal peak strain (GLS) in STZ versus control mice at all time points. 9w STZ animals displayed an impaired global circumferential peak strain (GCS) versus 6w and 12w STZ mice. They further exhibited decreased myocardial deformation behaviour of the anterior and posterior base versus controls, which was paralleled with an elevated collagen I/III protein ratio. Additionally, hypothesis-free proteome analysis by imaging mass spectrometry (IMS) identified regional- and time-dependent changes of proteins affecting sarcomere mechanics between STZ and control mice. In conclusion, STZ-induced diabetic cardiomyopathy changes global cardiac deformation associated with alterations in cardiac sarcomere proteins.
| S-EPMC7046677 | biostudies-literature
Project description:Light chain (AL) amyloidosis is the most common form of amyloidosis involving the kidney. It is characterized by albuminuria, progressing to overt nephrotic syndrome and eventually end-stage renal failure if diagnosed late or ineffectively treated, and in most cases by concomitant heart involvement. Cardiac amyloidosis is the main determinant of survival, whereas the risk of dialysis is predicted by baseline proteinuria and glomerular filtration rate, and by response to therapy. The backbone of treatment is chemotherapy targeting the underlying plasma cell clone, that needs to be risk-adapted due to the frailty of patients with AL amyloidosis who have cardiac and/or multiorgan involvement. Low-risk patients (?20%) can be considered for autologous stem cell transplantation that can be preceded by induction and/or followed by consolidation with bortezomib-based regimens. Bortezomib combined with alkylators, such as melphalan, preferred in patients harboring t(11;14), or cyclophosphamide, is used in most intermediate-risk patients, and with cautious dose escalation in high-risk subjects. Novel, powerful anti-plasma cell agents, such as pomalidomide, ixazomib, and daratumumab, prove effective in the relapsed/refractory setting, and are being moved to upfront therapy in clinical trials. Novel approaches based on small molecules interfering with the amyloidogenic process and on antibodies targeting the amyloid deposits gave promising results in preliminary uncontrolled studies, are being tested in controlled trials, and will likely prove powerful complements to chemotherapy. Finally, improvements in the understanding of the molecular mechanisms of organ damage are unveiling novel potential treatment targets, moving toward a cure for this dreadful disease.
| S-EPMC5976806 | biostudies-literature
Project description:BackgroundNewer 2D strain software has a potential to assess layer-specific strain. However, normal reference values for layer-specific strain have not been established. We aimed to establish the normal ranges of layer-specific longitudinal and circumferential strain (endocardial global longitudinal strain (GLS), transmural GLS, epicardial GLS, endocardial global circumferential strain (GCS), transmural GCS, and epicardial GCS).Methods and resultsWe retrospectively analyzed longitudinal and circumferential strain parameters in 235 healthy subjects, with use of layer-specific 2D speckle tracking software (GE). The endocardial strain/epicardial strain (Endo/Epi) ratio was also measured to assess the strain gradient across the myocardium. The endocardial, transmural, and epicardial GLS values and the Endo/Epi ratio in the normal subjects were -23.1±2.3, -20.0±2.0, -17.6±1.9, and 1.31±0.07, respectively. The corresponding values of GCS were -28.5±3.0, -20.8±2.3, -15.3±2.0, and 1.88±0.17, respectively. The layer-specific global strain parameters exhibited no age dependency but did exhibit gender dependency except for endocardial GCS. A subgroup analysis revealed that basal and middle levels of endocardial LS was decreased in the middle and elderly aged group. However, apical endocardial LS was preserved even in the elderly subjects.ConclusionsWe proposed normal reference values for layer-specific strain based on both age and gender. This detailed strain analysis provides layer-oriented information with the potential to characterize abnormal findings in various cardiovascular diseases.
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Project description:Cardiovascular mortality is high in ESRD, partly driven by sudden cardiac death and recurrent heart failure due to uremic cardiomyopathy. We investigated whether speckle-tracking echocardiography is superior to routine echocardiography in early detection of uremic cardiomyopathy in animal models and whether it predicts cardiovascular mortality in patients undergoing dialysis. Using speckle-tracking echocardiography in two rat models of uremic cardiomyopathy soon (4-6 weeks) after induction of kidney disease, we observed that global radial and circumferential strain parameters decreased significantly in both models compared with controls, whereas standard echocardiographic readouts, including fractional shortening and cardiac output, remained unchanged. Furthermore, strain parameters showed better correlations with histologic hallmarks of uremic cardiomyopathy. We then assessed echocardiographic and clinical characteristics in 171 dialysis patients. During the 2.5-year follow-up period, ejection fraction and various strain parameters were significant risk factors for cardiovascular mortality (primary end point) in a multivariate Cox model (ejection fraction hazard ratio [HR], 0.97 [95% confidence interval (95% CI), 0.95 to 0.99; P=0.012]; peak global longitudinal strain HR, 1.17 [95% CI, 1.07 to 1.28; P<0.001]; peak systolic and late diastolic longitudinal strain rates HRs, 4.7 [95% CI, 1.23 to 17.64; P=0.023] and 0.25 [95% CI, 0.08 to 0.79; P=0.02], respectively). Multivariate Cox regression analysis revealed circumferential early diastolic strain rate, among others, as an independent risk factor for all-cause mortality (secondary end point; HR, 0.43; 95% CI, 0.25 to 0.74; P=0.002). Together, these data support speckle tracking as a postprocessing echocardiographic technique to detect uremic cardiomyopathy and predict cardiovascular mortality in ESRD.
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