scholarly journals 566 Transthyretin and light-chain cardiac amyloidosis: clinical characteristics, echocardiographic findings, and outcomes of a large cohort of patients in Northern Italy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Maria Grazia De Angelis ◽  
Daniela Tomasoni ◽  
Edoardo Pancaldi ◽  
Elisa Pezzola ◽  
Nicola Saccani ◽  
...  

Abstract Aims To describe the characteristics of a cohort of patients with cardiac amyloidosis (CA) and to compare the two most common phenotypes of CA, transthyretin (ATTR) and immunoglobulin light-chain (AL). Methods and results One-hundred and eighty patients [n = 115 (64%) men, 74 ± 11 years] were retrospectively included from January 2013 to April 2021 in a single centre in Northern Italy. The majority [n = 102 (57%)] had ATTR-CA, whereas 78 patients (43%) had AL-CA. ATTR-CA patients were older (79 ± 7 vs. 66 ± 10 years, P < 0.001) and with higher prevalence of cardiovascular comorbidities, compared to those with AL-CA. ATTR-CA patients had higher N-terminal pro-B-type natriuretic peptide (Nt-proBNP) and troponin levels, and lower haemoglobin and estimated glomerular filtration rate. Echocardiographic findings suggested a more advanced stage of the disease in the ATTR-CA subgroup [left ventricular ejection fraction (LVEF), 51 ± 10% vs. 60 ± 9%; global longitudinal strain (GLS), −11 ± 3% vs. −13 ± 4%; peak systolic wall motion velocity, 4.9 ± 1.7 vs. 6.4 ± 1.9; left ventricular mass index (LVMI) 316 ± 133 g/m2 vs. 157 ± 72 g/m2; left atrium volume index (LAVI) 48 ± 17 ml vs. 40 ± 16 ml; right ventricular diameter 31 ± 9 mm vs. 22 ± 5 mm; tricuspidal annular plane systolic excursion (TAPSE) 17 ± 5 vs. 19 ± 5; all P < 0.05). During a median follow-up of 15 (6–31) months, 68 (38%) patients died. All-cause death occurred in 31% vs. 46% patients with ATTR- and AL-CA, respectively. AL-CA was an independent predictor of mortality (adjusted hazard ratio 2.62, 95% confidence interval 1.55–4.43; P < 0.001). Other independent predictors of mortality were age, systolic blood pressure, Nt-proBNP, troponin and GLS. When cardiovascular (CV) death was considered, there was no significant difference between the two phenotypes (log rank P = 0.384). Conclusions Despite ATTR-CA patients showed worse baseline characteristics, suggesting a more advanced disease at presentation, AL-CA phenotype was associated with a higher risk of all-cause death. Of note, CV mortality was comparable between the two groups.

Author(s):  
Koki Nakanishi ◽  
Masao Daimon ◽  
Yuriko Yoshida ◽  
Naoko Sawada ◽  
Kazutoshi Hirose ◽  
...  

Abstract Purpose Although subclinical hypothyroidism (SCH) is a common clinical entity and carries independent risk for incident heart failure (HF), its possible association with subclinical cardiac dysfunction is unclear. Left ventricular global longitudinal strain (LVGLS) and left atrial (LA) phasic strain can unmask subclinical left heart abnormalities and are excellent predictors for HF. This study aimed to investigate the association between the presence of SCH and subclinical left heart dysfunction in a sample of the general population without overt cardiac disease. Methods We examined 1078 participants who voluntarily underwent extensive cardiovascular health check-ups, including laboratory tests and 2-dimensional speckle-tracking echocardiography to assess LVGLS and LA reservoir, conduit, and pump strain. SCH was defined as an elevated serum thyroid-stimulating hormone level with normal concentration of free thyroxine. Results Mean age was 62 ± 12 years, and 56% were men. Seventy-eight (7.2%) participants exhibited SCH. Individuals with SCH had significantly reduced LA reservoir (37.1 ± 6.6% vs 39.1 ± 6.6%; P = 0.011) and conduit strain (17.3 ± 6.3% vs 19.3 ± 6.6%; P = 0.012) compared with those with euthyroidism, whereas there was no significant difference in left ventricular ejection fraction, LA volume index, LVGLS, and LA pump strain between the 2 groups. In multivariable analyses, SCH remained associated with impaired LA reservoir strain, independent of age, traditional cardiovascular risk factors, and pertinent laboratory and echocardiographic parameters. including LVGLS (standardized β −0.054; P = 0.032). Conclusions In an unselected community-based cohort, individuals with SCH had significantly impaired LA phasic function. This association may be involved in the higher incidence of HF in subjects with SCH.


Open Heart ◽  
2020 ◽  
Vol 7 (2) ◽  
pp. e001346
Author(s):  
Aénora Roger-Rollé ◽  
Eve Cariou ◽  
Khailène Rguez ◽  
Pauline Fournier ◽  
Yoan Lavie-Badie ◽  
...  

BackgroundCardiac amyloidosis (CA) is a life-threatening restrictive cardiomyopathy. Identifying patients with a poor prognosis is essential to ensure appropriate care. The aim of this study was to compare myocardial work (MW) indices with standard echocardiographic parameters in predicting mortality among patients with CA.MethodsClinical, biological and transthoracic echocardiographic parameters were retrospectively compared among 118 patients with CA. Global work index (GWI) was calculated as the area of left ventricular pressure–strain loop. Global work efficiency (GWE) was defined as percentage ratio of constructive work to sum of constructive and wasted works. Sixty-one (52%) patients performed a cardiopulmonary exercise.ResultsGWI, GWE, global longitudinal strain (GLS), left ventricular ejection fraction (LVEF) and myocardial contraction fraction (MCF) were correlated with N-terminal prohormone brain natriuretic peptide (R=−0.518, R=−0.383, R=−0.553, R=−0.382 and R=−0.336, respectively; p<0.001). GWI and GLS were correlated with peak oxygen consumption (R=0.359 and R=0.313, respectively; p<0.05). Twenty-eight (24%) patients died during a median follow-up of 11 (4–19) months. The best cut-off values to predict all-cause mortality for GWI, GWE, GLS, LVEF and MCF were 937 mm Hg/%, 89%, 10%, 52% and 15%, respectively. The area under the receiver operator characteristic curve of GWE, GLS, GWI, LVEF and MCF were 0.689, 0.631, 0.626, 0.511 and 0.504, respectively.ConclusionIn CA population, MW indices are well correlated with known prognosis markers and are better than LVEF and MCF in predicting mortality. However, MW does not perform better than GLS.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
R Ramos Polo ◽  
S Moral Torres ◽  
C Tiron De Llano ◽  
M Morales Fornos ◽  
J M Frigola Marcet ◽  
...  

Abstract INTRODUCTION Differential diagnosis by echocardiography between cardiac amyloidosis (CA) and hypertrophic cardiomyopathy (HCM) is based on the evaluation of left ventricular ejection fraction (LVEF) and global longitudinal strain (GLS) of the entire myocardial wall. Nevertheless, histopathological studies describe a higher involvement of subendocardial tissue in CA. The aim of our study was to evaluate whether the subanalysis of the GLS by layers (subendocardial and subepicardial) and segments (apical and basal) can provide further information. METHODS Retrospective study including 33 consecutive patients diagnosed with CA (with histological confirmation and imaging tests) or HCM by established criteria. Advanced myocardial deformation analysis software was used for both subendocardial and subepicardial evaluation of the left ventricle wall by transthoracic echocardiography. RESULTS Seventeen patients (52%) had CA and sixteen (48%) had HCM. Differences were observed in LVEF (52.9 ± 10.9% vs 62.4 ±5.0%; p = 0.004), but not in the analysis of the entire wall GLS (-12.3 ± 4.9 vs -13.4 ± 2.8; p = 0.457) nor in the LVEF/GLS ratio (4.7 ± 1.4 vs 4.8 ± 1.1; p = 0.718). In the layered analysis there was no difference in subendocardial GLS (-16.2 ± 5.0 vs -16.4 ± 3.2%; p = 0.916) or subepicardial GLS (-11.7 ± 4.1 vs -11.6 ±2.7%; p = 0.945); however, the increase in GLS from base to apex was greater for CA than for HCM both at subepicardial level (increase: 101% vs 16%; p = 0.006) and subendocardial level (increase: 242% vs 114%; p = 0.006), with inversion of the greatest values for each group (Fig. 1).The ratio (apical GLS/basal GLS) was diagnostic predictor of CA (area under the curve = 86%; p = 0.002): a value &gt;2 presented a sensitivity of 84% and a specificity of 85% for the diagnosis of CA. CONCLUSIONS CA presents an impairment of both subendocardial and subepicardial deformation in transthoracic echocardiography. These patterns provide additional information on differential diagnosis with HCM. Abstract P940 Figure. Subendo vs subepicardial mean values


EP Europace ◽  
2020 ◽  
Vol 22 (8) ◽  
pp. 1216-1223
Author(s):  
Eun-Jeong Kim ◽  
Benjamin B Holmes ◽  
Shi Huang ◽  
Ricardo Lugo ◽  
Asad Al Aboud ◽  
...  

Abstract Aims Cardiac amyloidosis (CA) is associated with increased mortality due to arrhythmias, heart failure, and electromechanical dissociation. However, the role of an implantable cardioverter-defibrillator (ICD) remains unclear. We conducted case-control study to assess survival in CA patients with and without a primary prevention ICD and compared outcomes to an age, sex, and device implant year-matched non-CA group with primary prevention ICD. Methods and results There were 91 subjects with CA [mean age= 71.2 ± 10.2, female 22.0%, 49 AL with Mayo Stage 2.9 ± 1.0, 41 transthyretin amyloidosis (ATTR), 1 other] followed by Vanderbilt Amyloidosis centre. Patients with ICD (n = 23) were compared with those without (n = 68) and a non-amyloid group with ICD (n = 46). All subjects with ICD had implantation for primary prevention. Mean left ventricular ejection fraction was 36.2% ± 14.4% in CA with ICD, 41.0% ± 10.6% in CA without ICD, and 33.5% ± 14.4% in non-CA patients. Over 3.5 ± 3.1 years, 6 (26.1%) CA, and 12 (26.1%) non-CA subjects received ICD therapies (P = 0.71). Patients with CA had a significantly higher mortality (43.9% vs. 17.4%, P = 0.002) compared with the non-CA group. Mean time from device implantation to death was 21.8 months in AL and 22.8 months in ATTR patients. There was no significant difference in mortality between CA patients who did and did not receive an ICD (39.0% vs. 46.0%, P = 0.59). Conclusions Despite comparable event rates patients with CA had a significantly higher mortality and ICDs were not associated with longer survival. With the emergence of effective therapy for AL amyloidosis, further study of ICD is needed in this group.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Frigy ◽  
I A Szabo ◽  
L Kocsis ◽  
S Z Laszlo ◽  
H Gabor Kelemen ◽  
...  

Abstract Background Early repolarization pattern (ERP) takes part from the J-wave syndromes and is associated with enhanced ventricular arrhythmogenesis in susceptible individuals. The main reason of our study was, that possible existence of subtle structural and functional cardiac changes related to ERP is still not well estabilished. Methods We compared 32 echocardiographic parameters (standard measures and specle tracking derived strain) of 30 young men (mean age 21.5 years) with ERP and 32 age and body-mass matched young men without ERP. T-test and chi-square test were used for statistical analysis (significant difference if p &lt; 0.05). Results Only the presence of mild mitral regurgitation was significantly more frequent in the ERP group (36.7% vs. 9.5%, p = 0.01). There were no significant differences regarding the other parameters, e.g., the interventricular septum (9.5 mm vs. 9.4 mm, p = 0.97), the end-diastolic diameters of the left and right ventricles (45 mm vs. 46.6 mm, p = 0.11; 34.9 mm vs. 33.8 mm, p = 0.42), the left ventricular ejection fraction ( 62.4% vs. 60.9 %, p = 0,3), the longitudinal diameters of the left and right atrium (51.8 mm vs. 53.1 mm, p = 0.27; 36.8 mm vs. 36.1 mm, p = 0.20), and the left ventricular global longitudinal strain (-21.75% vs. -21.28, p = 0.77). Conclusion In young males with ERP the vast majority of echocardiographic parameters was not different from those measured in their counterparts without ERP. The existence and clinical significance of the more prevalent mild mitral regurgitation in the ERP group has to be confirmed in a larger cohort.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
F Guastafierro ◽  
S Hosseini ◽  
P S Heiniger ◽  
S Anwer ◽  
N Kuzo ◽  
...  

Abstract Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is frequently associated with mutations in genes coding for desmosomal proteins. In this study, we investigated the association of genetic status with ARVC progression as defined by echocardiographic parameters. Methods We tested 62 ARVC patients for their genetic profile. Accordingly, they were grouped in mutation positive (48 (77%) patients; median age 48.5 years; 33 (69%) males), and mutation negative (14 (23%) patients; median age 45 years; 10 (71%) males). Prevalent mutations were Desmoglein-2 (DSG2) in 16 (26%), Desmoplakin (DSP) in 14 (23%), and Plakophilin-2 (PKP2) in 9 (15%) patients. Results At baseline, there were no significant differences in clinical characteristics between the two groups. Patients were followed-up for a median time period of 1420 days, and there was no significant difference in the duration of follow-up between the two groups (p=0.05). In the mutation positive group, there was a significant increase in right ventricular end-diastolic area (p=0.002), right atrial short (p=0.008) and long (p=0.002) diameter, left atrial diameter (p=0.014), and a decrease in left ventricular ejection fraction (p=0.014) during follow up. Right ventricular functial parameters did not change significantly (tricuspid annular plane systolic excursion: p=0.24; fractional area change: p=0.088). In the mutation negative group, none of the aforementioned echocardiographic findings exhibited any significant difference during follow-up: right ventricular end-diastolic area (p=0.1); right atrial short (p=0.7) and long (p=0.9) diameter, left atrial diameter (p=0.6), and left ventricular ejection fraction (p=0.3). Similarly, right ventricular functional parameters did not change significantly (tricuspid annular plane systolic excursion: p=0.77; fractional area change: p=0.80. Results are summarized in the figure. Change in echocardiographic findings. Conclusions There is a strong association between echocardiographic progression of ARVC phenotype and the presence of a pathogenic mutation. Such mutations should be searched in all patients with an ARVC phenotype, and mutation positive individuals should be followed-up in shorter intervals.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Hubert ◽  
A Galard ◽  
V Le Rolle ◽  
E Galli ◽  
A Hernandez ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): Hospital university of Rennes INSERM - LTSI Background Non-invasive estimation of myocardial work by trans-thoracic echocardiography is a novel tool to analyze myocardial contraction efficiency during systole. Two methods are described, on using Left ventricular (LV) strain and a LV pressure estimation, and another with only LV strain integrals. The present study analyzes their utility in prediction of CRT-response. Methods and results: 243 patients implanted by a CRT according to current recommendations were retrospectively included in hospital university of Rennes. All patients had a complete trans-thoracic echocardiography at implantation and at 6-moths follow-up. Responders were defined as having a 15% decrease in indexed LV end-systolic volume at follow-up compared to baseline. Baseline characteristics are described in table 1. 25.1% were non-responders. In this group, there were more men, more ischemic cardiomyopathies with more dilated LV. Strain signals ware analyzed only in the most informative loop, the apical 4 cavities. Myocardial work estimation with LV pressure estimation was previously described. The 3 different integral of strain signal were represented in figure 1. According to ROC curves, myocardial work (particularly wasted work in septal wall with AUC = 0.718 ± 0.04) estimated with LV pressure estimation is better than strain integrals to predict LV positive remodeling (best AUC 0.631 ± 0.040) after CRT-implantation. Conclusion Left ventricular pressure estimation give useful information on top of strain curves for prediction for CRT-response. Table 1 Responders n = 182 Non-responders n = 61 Men (%) 109 (59.9%) 52 (85%) Ischemic cardiomyopathy (%) 42 (23.1%) 34 (55.7%) LVEF (%) 28 ± 6 28 ± 7 GLS (%) -9 ± 3 -7 ± 3 LVEDD (mm) 62 ± 8 67 ± 7 LVEDVi (ml/m2) 85 ± 34 88 ± 30 LVEF Left ventricular ejection fraction; GLS: global longitudinal strain; LVEDD: left ventricular end-diastolic diameter; LVEDVi: left ventricular end diastolic volume index Abstract Figure 1


Author(s):  
T. Hauser ◽  
◽  
V. Dornberger ◽  
U. Malzahn ◽  
S. J. Grebe ◽  
...  

AbstractHeart failure with preserved ejection fraction (HFpEF) is highly prevalent in patients on maintenance haemodialysis (HD) and lacks effective treatment. We investigated the effect of spironolactone on cardiac structure and function with a specific focus on diastolic function parameters. The MiREnDa trial examined the effect of 50 mg spironolactone once daily versus placebo on left ventricular mass index (LVMi) among 97 HD patients during 40 weeks of treatment. In this echocardiographic substudy, diastolic function was assessed using predefined structural and functional parameters including E/e’. Changes in the frequency of HFpEF were analysed using the comprehensive ‘HFA-PEFF score’. Complete echocardiographic assessment was available in 65 individuals (59.5 ± 13.0 years, 21.5% female) with preserved left ventricular ejection fraction (LVEF > 50%). At baseline, mean E/e’ was 15.2 ± 7.8 and 37 (56.9%) patients fulfilled the criteria of HFpEF according to the HFA-PEFF score. There was no significant difference in mean change of E/e’ between the spironolactone group and the placebo group (+ 0.93 ± 5.39 vs. + 1.52 ± 5.94, p = 0.68) or in mean change of left atrial volume index (LAVi) (1.9 ± 12.3 ml/m2 vs. 1.7 ± 14.1 ml/m2, p = 0.89). Furthermore, spironolactone had no significant effect on mean change in LVMi (+ 0.8 ± 14.2 g/m2 vs. + 2.7 ± 15.9 g/m2; p = 0.72) or NT-proBNP (p = 0.96). Treatment with spironolactone did not alter HFA-PEFF score class compared with placebo (p = 0.63). Treatment with 50 mg of spironolactone for 40 weeks had no significant effect on diastolic function parameters in HD patients.The trial has been registered at clinicaltrials.gov (NCT01691053; first posted Sep. 24, 2012).


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Germano Junior Ferruzzi ◽  
Valeria Visco ◽  
Francesco Loria ◽  
Gennaro Galasso ◽  
Guido Iaccarino ◽  
...  

Abstract Aims Left ventricular global longitudinal strain (GLS) detects subtle systolic abnormalities in various cardiovascular conditions, which represent significant risk factors for cognitive impairment and stroke. Specifically, GLS has emerged as a more precise myocardial function measure than left ventricular ejection fraction (LVEF). This study investigated the relationship of GLS with mild cognitive impairment (MCI) in hypertensive patients. Methods and results From February 2020 to October 2021 were enrolled hypertensive patients without atrial fibrillation and/or cerebrovascular and/or neurodegenerative diseases. Complete demographic, clinical characteristics, laboratory analyses, conventional echocardiographic parameters were collected. Finally, MCI was defined by accurate the Quick Mild Cognitive Impairment (QMCI) Screen corrected for age and education. This score explores spatial and temporal orientation, registration, delayed recall, clock design, logical memory, and verbal fluency in a brief time (5 min—score 0–100); a compliance questionnaire (Morisky medication adherence scale); a questionnaire on nutritional status (MNA). 81 hypertensive patients [66 ± 7.27 years; 9 (11%) female] were included in the study. Concerning echocardiographic evaluation, LVEF was 50.47 ± 9.95% and mean GLS was −16.00 ± 3.66. Mean QMCI corrected for age and education was 56.45 ± 9.37, and MCI was detected in 21 patients (26%). When comparing the patients with MCI (QMCItot &lt;49.4) and without MCI (QMCItot &gt;49.4), a statistically significant difference of GLS values was detected (no MCI: −16.52 ± 3.66 vs. MCI: −14.18 ± 3.23; P = 0.032); on the other hand, the two groups did not differ in LVEF (no MCI: 50.58 ± 9.70 vs. MCI: 48.86 ± 11.93; P = 0.864). Furthermore, excluding patients with FE ≥ 45% from the analysis, a statistically significant linear regression was observed between QMCI (corrected for age and education) and the GLS (P = 0.014) (Figure 1). Conclusions Compromised GLS, but not LV EF, is related to MCI in hypertensive patients who are free of clinical dementia, stroke, and neurodegenerative disease. Moreover, our study demonstrates for the first time the existence of a significant association between the QMCI and GLS; consequently, GLS could be an additional parameter in clinical practice for early recognition of MCI. However, studies on a larger population will be needed to confirm this association.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Dragos Alexandru ◽  

Background: Among the 4 hemodynamic subsets of severe AS with preserved EF, based on LV stroke volume index (SVi) and mean pressure gradients, normal flow with a low gradient (NF/LG) has been attributed to measurement errors, small body size or inconsistencies in guideline criteria and associated with a favorable prognosis. We hypothesized that AV weight (AVW) in NF/LG AS, would be lower than that in other hemodynamic groups. Methods: Between 2010-13, 403 consecutive patients (pts) undergoing AV replacement (AVR) for severe isolated AS, (mean age 76.8 (9.3) yrs, 56% men, AVA index 0.36 (0.10 cm2)/m2, EF = 61.3 ± 4.6%, 11% bicuspid valves, mean follow-up 18 ± 9 months), underwent intraoperative TEE and had the AV weighed after excision (AVW = 2.4 ± 0.9 g), and excised valves were collected in formaldehyde, dried and weighed. All echo variables were measured off-line by a single observer on a dedicated reading station (Agfa). Medical records were reviewed to extract clinical data and all-cause mortality determined from the Social Security Death Index. Analysis of covariance (ANCOVA), Chi-square test, and Cox proportional hazards regression models were used as appropriate. Results: (for unadjusted comparisons see table.) In models adjusted for age, gender BSA, EF and annular area, AVW and AVW index remained lower in NF/LG than that in the high gradient groups (p<.01 for both). Although the number of deaths was small, no significant difference in all-cause mortality or time from AVR to death was found among the 4 groups (p=0.27 and p=0.28, respectively). AVR improved survival to a similar degree regardless of hemodynamic phenotype. Conclusions: 1. Compared with high gradient groups, pts with normal flow-low gradient have a lower AVW and AVW/aortic annulus area, a lower severity of stenosis and less LV remodeling. 2. These findings support the recent recommendation to categorize these pts as having only moderate AS.


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