scholarly journals P1532 Segmental assessment of left ventricle in patients with Fabry"s disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
V Losi ◽  
M S Rodolico ◽  
D C Faro ◽  
S Licciardi ◽  
C Tamburino ◽  
...  

Abstract Anderson-Fabry disease (AFD) is a rare X-linked metabolic disorder due to deficiency in lysosomal enzyme activity of a-galactosidase A, resulting in pathological accumulation of glycosphingolipids in several tissues and a progressive multi-organ dysfunction. The Global Longitudinal Strain (GLS) of the left ventricle (LV) by Speckle-tracking echocardiography (STE) has been show to detect subclinical cardiac involvement in many cardiomyopathies. The Mechanical Dispersion (MD), derived from STE is considered able to reflect a heterogeneous myocardial contraction, evaluated in many cardiopathies. A reduction in GLS was associated with myocardial fibrosis in the subclinical stages of AFD cardiomyopathy, but there is no MD data in AFD. The aim of the study was to evaluate the distribution of GLS and MD of LV in patients with AFD. 47 consecutive AFD adult patients treated (37F, 12 M, age 45 + 17 years) were examined by a complete echocardiographic examination from a Vivid E95 ultrasound scanner (Horten, Norway). The STE post-processing was performed by Echopac 2.02, in apical long-axis, 4-chambers, and 2-chambers views to determine GLS and MD. Data were expressed as mean± standard deviation. The comparison between the AFD group and 20 normal subjects (N) was performed with unpaired T-test. Compared to N, the AFD group showed higher values of LVMi, LAVi, E/E’, and MD, but a lower value of GLS (Table). GLS was significantly lower in the basal (sept p<.002; post p<.0001) and mid segments (ant-sept p<.008; post p<.0001; ant p<.001). MD was significantly higher in the inferior (basal p<.003; mid p<.01; apical p<.005) and lateral segments (mid p<.004; apical p<.001). In patients with AFD, MD added to GLS seems to be a promising tool for the early diagnosis of segmental dysfunction . Table1: Results AFD N p< m SD m SD LVMi g/sqm 77.1 29.8 62.3 14.1 0.008 LVEF % 65.7 5 63.6 3.5 0.04 LAVi ml/sqm 25.5 11 18.2 4.5 0.000 E/E" 8.3 3 5.4 1.1 0.000 TV vmax m/s 2.2 0.4 2.1 0.3 - LV GLS % -17 4 -20 2 0.003 LV MD ms 52 38 29 8 0.000

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Koziel ◽  
J Boidol ◽  
J Klys ◽  
K Miszalski-Jamka ◽  
Z Kalarus ◽  
...  

Abstract Background Myocarditis may be a challenging diagnosis because of diversity of clinical presentations. Thus, the clinical course and ventricular remodelling in this disease seems to be unpredictable. Aims To assess predictors of left ventricular remodelling in patients after active myocarditis. Methods Database from a high-volume, tertiary cardiology center was analysed to identify patients with active myocarditis, based on clinical presentation and ≥1 diagnostic criteria from different categories (including electrocardiography/ holter, elevated troponin T/I levels, functional or structural abnormalities on cardiac imaging or tissue characterization by cardiac magnetic resonance) between 2016 and 2019. Left ventricle global longitudinal strain (GLS), mechanical dispersion (standard deviation of time to peak longitudinal strain derived from all left ventricle segments in 3 apical views) were calculated. Response (left ventricular positive remodeling measured by transthoracic echocardiography) was defined as end-systolic volume (ESV) reduction ≥15% from a baseline value or end-diastolic volume (EDV) reduction ≥15%. Results 61 consecutive patients were enrolled. The median follow-up was 1.4 years (range: 0.3–4.0). During entire follow-up period mortality rate in patients was 1.6%. Multivariate Cox regression model including significant baseline differences as covariates reported that QRS durations (HR 1.31, 95% CI 1.17–1.57, P=0.049) and mechanical dispersion (HR 1.03, 95% CI 1.01–1.07, P=0.036) were independently associated with left ventricular positive remodelling with ESV reduction. Mechanical dispersion (HR 1.04, 95% CI 1.02–1.06, P=0.040) was independently associated with left ventricular positive remodelling with EDV reduction. Conclusions Mechanical dispersion and QRS duration are independent predictors of left ventricular remodelling in patients after active myocarditis. Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
D Sirico ◽  
P Costenaro ◽  
C Di Chiara ◽  
D Dona" ◽  
S Cozzani ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Evidence suggests that clinical manifestations of children’s COVID-19 may be less severe. However, it has been described the pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) which resembles other inflammatory conditions (i.e. Kawasaki disease). Patients affected by PIMS-TS showed cardiac involvement with myocardial injury, reduced left ventricle systolic function and coronary artery abnormalities. Little is known regarding cardiac involvement in pediatric patients with asymptomatic or mildly symptomatic SARS-CoV-2 infection. Methods We analyzed 23 pediatric patients (13males, 56%) with diagnosis of SARS-CoV-2 infection based on PCR analysis of nasopharingeal swab (NPS), and asymptomatic or only mildly symptomatic for COVID-19. Patients underwent standard transthoracic echocardiogram (TTE) within 2-3 month from diagnosis and after negative NPS for SARS-CoV-2. We performed offline analysis with GE EchoPAC software to measure global longitudinal strain (GLS) of the LV using 2D speckle tracking imaging. Therefore, we compared the results with a matched group of 23 controls (13males, 56%). Results Cases and controls were similar regarding age (5.9 ± 4.1years vs. 6.4 ± 4.4 years, p = 0.63), body surface area (0.98 ± 0.3m2 vs. 0.8 ± 0.4m2, p = 0.17), LV FS (37.9 ± 5.9% vs. 36.4 ± 8.3%, p = 0.74) and LV biplane EF (63.9 ± 5.2% vs. 66.4 ± 5.3%, p = 0.11). GLS analysis showed significant strain reduction of the LV mid-wall segments and of the basal anterior, posterior and septal inferior segments among cases compared to controls. Furthermore, in the case group there were 7 subjects (30%) with a strain below 16.5% in at least 3 segments. Conclusion SARS-CoV-2 infection may affect LV deformation in asymptomatic or only mildly symptomatic children, showing a peculiar pattern with lower longitudinal strain in all mid-wall segments of LV compared to control subjects. The clinical significance of this findings is unclear and follow-up is needed to verify the reversibility of this alterations.


Author(s):  
Giuseppina Novo ◽  
DANIELA DI LISI ◽  
Diego Bellavia ◽  
Eluisa La Franca ◽  
Maria Gabriella Carmina ◽  
...  

Background: Myocarditis have variable clinical presentation, evolution and prognosis. Aim of our study was to evaluate the value of speckle tracking echocardiography and cardiac magnetic resonance (CMR) in the prediction of ventricular arrhythmias and other cardiovascular adverse events in patients with acute myocarditis, at hospital admission. Methods: 70 patients (62 M, 8 F; mean age 31,3±13,2) with myocarditis and preserved left ventricle ejection fraction (LVEF) were enrolled. Electrocardiogram (ECG), continuous ECG monitoring, echocardiography with measurement of global longitudinal strain of the left ventricle (GLS), mechanical dispersion (MD) and CMR with quantitative measurement of delayed enhancement (DE) were performed. Adverse events were assessed (arrhythmias, heart failure, cardiogenic shock, syncope) during in-hospital stay. Results: We found a significant greater amount of DE mass in patients with cardiac arrhythmias (p = 0,01), but not of edema (p = 0,57). GLS was significantly impaired in patients with ventricular arrhythmias (p = 0,04), conversely MD was not significantly prolonged in this setting (p = 0,16). GLS > - 19.2% (sens 100%, specif 55,7%) and a DE mass >9,9 gr (sens 100%; specif. 58,6%) had the best sensitivity and specificity to identify patients with cardiac arrhythmias. Compared to GLS, DE mass showed a stronger association with ventricular arrhythmias (p < 0,001). Conclusions: in our study DE mass and GLS were associated with ventricular arrhythmias in patients with acute myocarditis and preserved LVEF. DE showed the stronger association with the occurrence of ventricular arrhythmias.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Hui Wang ◽  
Ruili Li ◽  
Zhen Zhou ◽  
Hong Jiang ◽  
Zixu Yan ◽  
...  

Abstract Background Coronavirus disease 2019 (COVID-19) induces myocardial injury, either direct myocarditis or indirect injury due to systemic inflammatory response. Myocardial involvement has been proved to be one of the primary manifestations of COVID-19 infection, according to laboratory test, autopsy, and cardiovascular magnetic resonance (CMR). However, the middle-term outcome of cardiac involvement after the patients were discharged from the hospital is yet unknown. The present study aimed to evaluate mid-term cardiac sequelae in recovered COVID-19 patients by CMR Methods A total of 47 recovered COVID-19 patients were prospectively recruited and underwent CMR examination. The CMR protocol consisted of black blood fat-suppressed T2 weighted imaging, T2 star mapping, left ventricle (LV) cine imaging, pre- and post-contrast T1 mapping, and late gadolinium enhancement (LGE). LGE were assessed in mixed both recovered COVID-19 patients and healthy controls. The LV and right ventricle (RV) function and LV mass were assessed and compared with healthy controls. Results A total of 44 recovered COVID-19 patients and 31 healthy controls were studied. LGE was found in 13 (30%) of COVID-19 patients. All LGE lesions were located in the mid myocardium and/or sub-epicardium with a scattered distribution. Further analysis showed that LGE-positive patients had significantly decreased LV peak global circumferential strain (GCS), RV peak GCS, RV peak global longitudinal strain (GLS) as compared to non-LGE patients (p < 0.05), while no difference was found between the non-LGE patients and healthy controls. Conclusion Myocardium injury existed in 30% of COVID-19 patients. These patients have depressed LV GCS and peak RV strains at the 3-month follow-up. CMR can monitor the COVID-19-induced myocarditis progression, and CMR strain analysis is a sensitive tool to evaluate the recovery of LV and RV dysfunction.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Boidol ◽  
M Koziel ◽  
K Miszalski-Jamka ◽  
J Klys ◽  
Z Kalarus ◽  
...  

Abstract Background The clinical course and ventricular remodeling in inflammatory myocardial disease could be unpredictable. No single functional parameter has been confirmed as a powerful predictor of clinical course and functional recovery assessment in patients with acute inflammatory myocardial disease. Purpose The aim of the study was to assess the mechanical properties of the myocardium in patients with active myocarditis at baseline and follow-up. Methods Database from a high volume, tertiary cardiology center was analysed to identify patients with active myocarditis, based on clinical presentation and ≥1 diagnostic criteria from different categories (including electrocardiography/holter, elevated troponin T/I levels, functional or structural abnormalities on cardiac imaging or tissue characterization by cardiac magnetic resonance) between 2016 and 2019. Conventional and speckle tracking echocardiography including global longitudinal strain (GLS) mechanical dispersion (MD) was completed at baseline and at 17±13 months follow-up. MD was calculated as a standard deviation of time to peak longitudinal strain derived from all left ventricle segments in 3 apical views. Results 61 consecutive patients [50 M, 11F, end-diastolic volume 212±84 ml, end-systolic volume 130±90ml, ejection fraction (EF) 42±16%] were enrolled. During the entire follow-up 1 patient died at early observation. Implantable cardioverter-defibrillator was implanted in 5 patients (primary prevention 4, secondary 1), cardiac resynchronization therapy pacemaker in 1 patient. Despite of significant global improvement (EF 42±16% vs 52±10%, p&lt;0.001) the limited regional improvement was noticed (GLS 14±6% vs 15±4%, p = NS; MD 47±18 ms vs 45±20 ms, p=NS) in all patients at 17±13 months follow-up. There was a strong negative association between GLS and MD at baseline (Figure 1), and slightly weaker at follow-up (R=0.47, Pearson's correlation). Moreover, the GLS correlated well the change of MD in each individual patient. Conclusions Mechanical dispersion and global longitudinal strain may serve as an additional markers of myocardial damage and potential predictive markers in non ischemic cardiomyopathy patients with proven inflammatory origin. Funding Acknowledgement Type of funding source: None


Author(s):  
Erika N Aagaard ◽  
Brede Kvisvik ◽  
Mohammad O Pervez ◽  
Magnus N Lyngbakken ◽  
Trygve Berge ◽  
...  

Abstract Aims Increased left ventricular mechanical dispersion by 2D speckle tracking echocardiography predicts ventricular arrhythmias in ischaemic heart disease and heart failure. However, little is known about mechanical dispersion in the general population. We aimed to study mechanical dispersion in the general population and in diseases associated with increased risk of cardiovascular disease. Methods and results The present cross-sectional study consists of 2529 subjects born in 1950 included in the Akershus Cardiac Examination (ACE) 1950 study. Global longitudinal strain (GLS) was assessed from 17 strain segments, and mechanical dispersion calculated as the standard deviation of contraction duration of all segments. The cohort was divided according to the median value of mechanical dispersion, and multivariable linear regression models were performed with mechanical dispersion as the dependent variable. The prevalence of coronary artery disease (CAD), hypertension, obesity, and diabetes (P < 0.01 for all) was significantly higher in subjects with supra-median mechanical dispersion. In a multivariable clinical model, CAD (B = 7.05), hypertension (B = 4.15; both P < 0.001), diabetes (B = 3.39), and obesity (B = 1.89; both P < 0.05) were independently associated with increasing mechanical dispersion. When echocardiographic indices were added to the multivariable model, CAD (B = 4.38; P < 0.01) and hypertension (B = 2.86; P < 0.001) remained significant in addition to peak early diastolic tissue velocity e’ (B = −2.00), GLS (B = 1.68), and ejection fraction (B = 0.22; P < 0.001 for all). Conclusion In a general middle-aged population, prevalent CAD and hypertension were associated with increasing mechanical dispersion, possibly indicating elevated risk of fatal arrhythmias and sudden cardiac death. Albeit weaker, systolic and diastolic dysfunction, were also associated with increasing mechanical dispersion.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Jun Tanaka ◽  
Satoshi Nakatani ◽  
Makoto Amaki ◽  
Hideaki Kanzaki ◽  
Shuji Hashimoto ◽  
...  

Dilated cardiomyopathy (DCM) is histologically characterized by myocardial fibrosis and cellular hypertrophy. These myocardial changes may affect timing of regional peak contraction. We investigated whether there was a correlation between intraventricular septal (IVS) intramural asynchrony based on tissue velocity imaging and the histologic changes. We obtained apical 4-chamber tissue velocity images in 27 normal subjects and 22 patients with DCM undergoing myocardial biopsy using Vivid 7 (GE). We set 6 tandem regions of interest (ROIs) on the mid level of the IVS and measured the time to peak myocardial contraction (Ts) from the QRS onset at each ROI. We defined the standard deviation of Ts (Ts-SD) as an index of IVS asynchrony. Further, we analyzed a correlation between Ts-SD and the degree of myocardial fibrosis and cellular hypertrophy. The degree of myocardial fibrosis and cellular hypertrophy was graded qualitatively (0 to 3 scale) according to the percent of fibrosis occupied of the tissue sample and cell size. Ts-SD was larger in patients with DCM compared with normal subjects (13.9±12.3 vs. 6.2±4.0 ms, p<0.05), suggesting asynchronous contraction of IVS in DCM. Ts-SD showed a significant correlation with the degree of myocardial fibrosis (grade 1, 6.9±3.5 ms; grade 2, 15.7±3.5 ms; grade 3, 25.9±5.3 ms; p<0.05), while not with that of cellular hypertrophy (p=0.50). There was intramural asynchrony in IVS in DCM. This method could predict the degree of myocardial fibrosis noninvasively. Because myocardial fibrosis is the substrate for heart failure in DCM, the present method should provide clinically important information.


2021 ◽  
Vol 20 (Supplement_1) ◽  
Author(s):  
O Germanova ◽  
O Tereshina

Abstract Funding Acknowledgements Type of funding sources: None. Background. New COVID-19 disease is well known by its primary damage of respiratory system. However, it is also possible to involve other organs and systems. Purpose. To determine the cardiac involvement in patients with COVID-19 pneumonia. Materials and methods. We performed echocardiography with speckle tracking in 48 patients. All of them had serologically verified COVID-19 pneumonia with lung involvement 5-60% on CT. None of them were treated by antimalarial drugs. 28 of them were men and 20 women, mean age - 45 ± 4,4 years old. We performed echocardiography in 1 month period from onset of the disease. Results. Most of the patients (30 patients) had enlargement of right heart chambers. Despite of right ventricle enlargement, in none of the patients had reduced right ventricle function (TAPSE 22 ± 2 mm, right ventricle strain -21,2 ± 1,5%). The mean systolic pulmonary artery pressure was slightly elevated (38 ± 2 mm Hg). Left ventricle was enlarged in 9 patients (with mean end diastolic volume 82 ± 5 ml/m2). In 25 of them, the ejection fraction was decreased (44 ± 4%). In all patients with COVID-19 pneumonia, the changes in circumferential and longitudinal strain were observed. Both parameters were decreased. The mean value of circumferential strain was -16,3 ± 1,9%. The longitudinal strain parameter was -14,2 ± 1,4%. 25 patients had enlargement of left atrium (&gt;40ml/m2) and the reservoir strain of left atrium was decreased (-23,3 ± 1,5%)  in 29 of them. After 2 months from the onset of the disease, we noticed the normalization of echocardiography data in all patients. Nevertheless, we observed cardiac structural and functional changes in all patients, troponins levels were increased only in 12 patients. Conclusion. We observed cardiac involvement in different degree in all of patients with COVID-19 pneumonia. Changes characterized by atriums and ventricles enlargement, and left ventricle contractile dysfunction.  Due to the fact that only part of the patients with COVID-19 with cardiac changes had elevated troponins, we believe that the mechanism of these pathological changes need further research.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Dimosthenis Pandis ◽  
Marc Miller ◽  
Ahmed El-Eshmawi ◽  
Ioulia A Grapsa ◽  
Percy Boateng ◽  
...  

Introduction: Asymptomatic patients on active surveillance for degenerative mitral regurgitation are at risk of ventricular arrhythmia and sudden death. Hypothesis: Abnormal myocardial mechanics may precede ventricular remodeling and may help identify at-risk patients. Methods: Multi-directional myocardial mechanics and LV dyssynchrony were assessed in 204 consecutive patients awaiting surgical mitral repair for severe degenerative MR in a quaternary mitral reference center. Results: The mean age was 58 ±12.5 years and 40% were females. The mean EF was 63% ± 6% and 92% had compensated LV function (EF>60% and LVESD<4cm) and only 24% had elevated LV filling pressures (E/e'≥13). Indexed LV wall stress-to-LVEDD, relative wall thickness and indexed LV mass-to-BSA were similar amongst males-females, although males had higher mean blood pressure (94 Vs. 90, P=0.02) . The peak global longitudinal strain (GLS) was -25.2% ± 3.3% and the mid-ventricular circumferential and radial strains were -33.5%±6.7% and 56%±25% respectively. Ventricular ectopy was present in 24.5% of patients and only 17% had atrial fibrillation (Afib) despite the significantly dilated left atria (mean LAVi 70±26.6 ml/m 2 ). The median LV mechanical dispersion was 40msec (IQR 30.7-56.5) but increased significantly with ventricular ectopy (65msec, P<0.01) and further influenced by concomitant Afib (p=0.001 for 2-factor interaction). Diastolic LV function did not correlate with dispersion (r=0.02 and 0.01 for E/A and E/e', P=NS) but was associated with the duration of LV diastolic filling (mean 502±140msec; r=0.2, P=0.004). Interpapillary radial strain delay was noted in the study cohort (mean delay 52.8msec, range 0-335msec) while intepapillary activation delay was manifested with concomitant ventricular ectopy (mean time-to-peak LS delay 57.5±48msec). Conclusions: Left ventricular dyssynchrony manifested by increased mechanical dispersion and imbalanced interpapillary mechanics are observed prior to overt chamber remodeling in significant degenerative MR and is associated with ventricular ectopy. Further studies are needed to assess the related clinical implications and potential impact on risk stratification in this patient group.


Circulation ◽  
2014 ◽  
Vol 129 (suppl_1) ◽  
Author(s):  
Michael L Chuang ◽  
Philimon Gona ◽  
Connie W Tsao ◽  
Carol J Salton ◽  
Warren J Manning ◽  
...  

Introduction: Myocardial contraction fraction (MCF) is the ratio of left ventricular (LV) stroke volume to myocardial volume, and thus a measure of LV pumping capacity per unit of myocardium. We sought to determine whether MCF measured using current steady-state free precession (SSFP) cardiac magnetic resonance (CMR) sequences was an independent predictor of incident “hard” cardiovascular disease (CVD) events, defined by myocardial infarction (MI), stroke, unstable angina (UA), hospitalized heart failure (HF) or CVD death in a community dwelling cohort initially free of these CVD events. Methods: 1794 members of the Framingham Heart Study Offspring cohort (aged 65±9 years) underwent CMR between 2002-2006 using a 1.5-Tesla system with contiguous multislice SSFP cine imaging to encompass the left ventricle. MCF was determined from the cine images by a single observer blinded to participant characteristics. We tracked incident hard CVD events over median 6.5-year follow up and used Cox proportional hazards models (adjusted for age, sex, body mass index, systolic blood pressure, diabetes, dyslipidemia, smoking, treatment for hypertension) to determine hazard of hard CVD events per increment (0.10) of MCF. Results: MCF was determined in 1776 (99%) Offspring (835 men). Overall, MCF was greater in women (0.92±0.14 vs. 0.78±0.15 for men), p<0.0001. There were 60 incident hard CVD events during follow up. Incident hard events included 26 MI, 2 UA, 13 stroke, 14 hospitalized HF and 5 CVD deaths. Offspring experiencing an incident event had lower MCF (0.78±0.19 vs. 0.86±0.15 for those free of events), p=0.002. On MV-adjusted Cox proportional hazards analyses, a greater MCF was protective against hard CVD events, HR [95% confidence intervals] = 0.76 [0.63 - 0.93] per 0.10 increment of MCF. Conclusion: Over 6.5-year follow-up, greater MCF is protective against major adverse CVD events, even after adjustment for traditional CVD risk factors in a community dwelling cohort of middle-aged and older predominantly European-descended adults. Determination of MCF requires only knowledge of LV stroke volume and myocardial volume, both of which are routinely determined in a standard CMR examination of the left ventricle, and thus imposes no additional scan-time or analysis burden. While MCF may be clinically useful for prediction of risk for incident hard CVD events, its potential value in younger age groups and other ethnicities remains to be determined.


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