Left Atrium Enlargement Is An Independent Predictor of Overall Mortality In Patients with Cardiac AL Amyloidosis.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4621-4621
Author(s):  
Dania Mohty ◽  
Philippe Pibarot ◽  
Nicole Darodes ◽  
Benedict Tanguy ◽  
David Lavergne ◽  
...  

Abstract Abstract 4621 Background: Primary systemic amyloidosis (AL) is a severe plasma cell disorder characterized by amyloid fibrils extracellular deposition in different organs. Myocardial involvement is frequent and has major impact on prognosis. Echocardiography (TTE) is the most common test performed when cardiac involvement is suspected. We hypothesized that a simple measurement of left atrium enlargement (LA) by TTE may provide an important risk marker for this disease. Methods and results: Between 1997 and 2010, 114 patients were diagnosed with systemic AL. Sixty one patients of the cohort (54%) had cardiac involvement on the basis of standard criteria defined as: mean LV wall thickness >12mm with no other cardiac causes. They had first TTE within 16 days of diagnosis. Patients were mainly treated with conventional chemotherapy (M-Dex) or with new agents for refractory or relapsing patients We retrospectively collected demographic baseline characteristics along with biological and echo data of these patients. Mean age was 61±11 years; 60% were male; 19% had hypertension. Mean left ventricular ejection fraction and mean LV wall thickness were respectively 58±13% and 15±3 mm. Mean follow up time was 2.1±2 years. None had significant valvular heart disease. LA enlargement was defined by M mode as > 40 mm in male and > 36 mm in female. Patients with enlarged LA had significantly more hypertension and lower ejection fraction and more hypertrophied LV walls (All P< 0.05). At 3 years, survival rate was markedly reduced in patients with enlarged LA vs. those with normal LA: 34±9% vs.75 ±9% (P =0.02). By multivariate analysis, after adjusting for age, gender, and presence of hypertension, LA enlargement remained an independent predictor of overall mortality at 3 years (HR=2.88; CI (1.12-8.84); P=0.03). Conclusion: In patients with systemic AL amyloidosis and cardiac involvement, LA enlargement, a surrogate marker of diastolic dysfunction and elevated LV filling pressure, is a powerful independent predictor of long-term mortality. Therefore LA enlargement may help to enhance risk stratification in patients presenting with this disease. Disclosures: No relevant conflicts of interest to declare.

2020 ◽  
Vol 19 (2) ◽  
pp. 181-187
Author(s):  
Jing Li ◽  
Yun Zhang ◽  
Weizhong Huangfu ◽  
Yuhong Ma

Using rat models of heart failure, we evaluated the effects of rosuvastatin and Huangqi granule alone and in combination on left ventricular end-diastolic dimension, left ventricular end-systolic dimension, left ventricular ejection fraction, left ventricular posterior wall thickness at end-diastole, and left ventricular posterior wall thickness at end-systole. Results showed that left ventricular end-diastolic dimension, left ventricular end-systolic dimension in the rosuvastatin + Huangqi granule group were significantly decreased (P ‹ 0.01), while left ventricular ejection fraction, left ventricular posterior wall thickness at end-diastole and left ventricular posterior wall thickness at end-systole were significantly increased (P ‹ 0.05). The serum IL-2, IFN-β, and TNF-α in rosuvastatin + Huangqi granule group were significantly lower than those in model group (P ‹ 0.05). However, the levels of S-methylglutathione and superoxide dismutase in rosuvastatin + Huangqi granule group were significantly higher, while nitric oxide was significantly lower than that in the model group (P ‹ 0.05). Also, compared to the model group, the apoptosis rate, and the autophagy protein LC3-II in the cardiomyocytes of rosuvastatin + Huangqi granule group was significantly decreased (P ‹ 0.01), while the level of p62 protein was significantly increased (P ‹ 0.01). The levels of AMPK and p-AMPK in cardiomyocytes were significantly lower in rosuvastatin + Huangqi granule group; however, the levels of mTOR and p-mTOR showed an opposite trend (P ‹ 0.05). To sum up, rosuvastatin + Huangqi granule could improve the cardiac function, decrease the level of oxidative stress, and inflammatory cytokines in rats with HF. The possible underlying mechanism might be inhibition of autophagy and reduced apoptosis in cardiomyocytes by regulating AMPK-mTOR signaling pathway.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Jing Ping Sun ◽  
Xianda Ni ◽  
Tingyan Xu ◽  
Min Xu ◽  
Xing Sheng Yang ◽  
...  

Purpose: We aimed to evaluate compensatory mechanisms in hypertrophic cardiomyopathy (HCM) patients (pts) with preserved left-ventricular (LV) ejection fraction (EF). Methods: Speckle-tracking echocardiography (Vivid E9, GE) was performed in 50 HCM with preserved LV EF (38 m; 49± 14 y, all LV EF > 55%) and 50 age, gender matched controls (38 m; 49±12 y). The global and segmental longitudinal (LS), circumferential (CS) and radial strain (RS) strains of endocardia (End), mid-wall and epicardia layers were analyzed using a novel layer-specific TTE. The ratio of End to epicardia strain (End/Epi) was calculated. Results: The LV EF were similar in pts and controls (64±8 vs 64±7 %, p=0.95). The diastolic function was significantly impaired in HCM pts compared with controls (E/E’:18.4±8.4 vs 8.6 ±2.4, p<0.0001). The absolute value of LS and CS was reserved at apical End layers (-34±7 vs -35±6, p=0.44); the remaining segments and LV global LS and CS of three layers were significantly smaller (LS,-16±5 vs -22±3; CS -24±8 vs -33±7; p<0.0001), and LS and CS End/Epi (1.7±0.3 vs 1.3±0.1, 3.4±1.1 vs 1.7±0.2 respectively, P <0.0001) was significantly higher in HCM pts than in controls. The RS and LV twist were preserved in all LV segments (27±10 vs 24±12, p=0.19; 20±8 vs 18±5, p=0.33; respectively). In HCM pts, the LV LS value at basal and middle levels revealed significant negative correlations with LV relative wall thickness (r=–0.65, –0.59 and –0.60, –0.54, respectively , p< 0.0001); and mild negative correlations (r=-0.33,-0.29, p<0.0001). The LV CS value at all levels revealed mild correlations with relative wall thickness (r=-0.22, p<0.05) . The LS were significantly reduced at the hypertrophic segments (Figure). Conclusions: In HCM patients with preserved LVEF, LV GLS was impaired, but apical End LS and basal End CS, LV RS as well as LV twist were maintained as the compensation for reduction LV LS and CS. The Bull’s eye of LS may help us to localize the lesion segments and define the type of HCM.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Heuze ◽  
L Legrand ◽  
A Diallo ◽  
M L Monin ◽  
C Ewenczyk ◽  
...  

Abstract Introduction Friedreich ataxia (FRDA) is a rare genetic sensory ataxia. The causal mutation is an expanded trinucleotide repeat (GAA) in the frataxin gene. Hypertrophic cardiomyopathy is associated with FRDA and is the major cause of early death before 40 years old. Patients with progressive decline of the left ventricular ejection fraction (LVEF) have the worse prognosis. Speckle tracking echography with 2D longitudinal myocardial strain (GLS) is recognized as a more effective technique than conventional LVEF in detecting subtle changes in LV function. Purpose Evaluate the prognostic value of global longitudinal strain (GLS) in patients with FRDA as compared to LVEF. Methods From 2003 to 2017 consecutive patients with genetically confirmed FRDA were included. Longitudinal strain analysis was retrospectively performed with Tomtec software. News were obtained until April 2018, no patient was lost during follow-up. Results The study included 156 patients (51% male) of 35±12 years (mean ± SD) with an age at disease onset of 17±11 years, age at wheelchair use of 26±10 years, and GAA repeat on the shorter allele of 590±241 pb. The following echocardiographic parameters were studied at baseline: LVEF 64±9%, GLS −19.8±5% (n=141), septal wall thickness (SWT) 11.4±2.5 mm, posterior wall thickness (PWT) 10.4±1.8 mm, LV end diastolic diameter (LVEDD) 44.4±6mm. Correlation between GLS and LVEF was 0.31 (p=0.0002). After a mean follow-up of 7.7±4.0 years, 17 (11%) patients died and the outcome (cardiac arrhythmia, heart failure, stroke or death) concerned 28 (18%) patients. In univariate analysis (Cox model), factors associated with mortality were: GLS (HR: 1.2; 95% CI 1.10–1.32, p=0.0001), LVEF (HR: 0.88; 95% CI 0.85–0.92, p<0.0001), GAA (HR: 1.28; 95% CI 1.11–1.47, p=0.0008), age at onset (HR: 0.84; 95% CI 0.76–0.94, p=0.002), LVMi (HR: 1.02; 95% CI 1.01–1.04, p=0.0078), SWT (HR: 1.18 95% CI 1.01–1.36, p=0.03) and LVEDD (HR: 1.09; 95% CI 1.00–1.19, p=0.04). In multivariate analysis LVEF was the only independent predictor of long-term mortality (HR: 0.93; 95% CI 0.88; 0.99, p=0.02). Similarly GLS was not an independent predictor of the composite outcome in multivariate analysis. Conclusion GLS is a predictor of morbimortality but is not superior to LVEF in FRDA patients. Further prospective studies are mandatory to assess the early predictive value of 2D GLS.


Author(s):  
V. I. Denesyuk ◽  
O. V. Denesyuk ◽  
N. O. Muzyka

Background. According to the national registries of European countries and epidemiological studies, the prevalence of chronic heart failure (CHF) among adults is 2,0-5,0%, and increases due to age, in people aged over 70 years old it is 10,0-20,0%.Objective. To find out the specific features of remodeling of the left atrium and change of vasodilation factors in ischemic heart failure with reduced and preserved left ventricular ejection fraction and to establish correlation relationships.Methods. A full clinical examination of 153 patients with CHF (105 men and 48 women) was conducted to achieve this objective. The surveyed patients underwent clinical examinations; spectrophotometric parameters: quantification of markers of vasodilation, metabolites of monoxide nitrogen – nitrates and nitrites with Gris reagent; content of endothelial nitric oxide synthase (eNOS) in serum - ELISA for the set of Nitric Oxide Synthase 3, Endothelial (NOS3) Human ELISA Kit (Cloud-Clone Corp, USA). Electrocardiographic (ECG) examination was conducted in 12 standard conventional leads on electrocardiograph by the Hungarian production Heart Screen 112 D.Results. The 1st group of the examined patients with reduced LV EF prevails III (significant) degree LA dilatation in 33 (70.21%) cases, II (moderate) degree of LA dilatation was determined in 14 (29.78%), and I (initial) degree was not defined at all. In the 2nd group of the patients with preserved LV EF mainly the II degree of LA dilatation was determined in 44 (44.51%) cases, and decreased LA dilation in 39 (36.79%) cases (p<0.01), and III degree of LA dilation was defined in 23 (21.69%) cases (p<0.01). In patients with stable coronary heart disease, complicated by heart failure with reduced LV EF and II degree of LA dilatation, eNOS levels in the serum was 449.00±39.91 pg/ml, whereas in patients with stable coronary heart disease, complicated by heart failure with preserved LV EF and II stage of LA dilatation – 673.56±50.98 pg/ml (p<0.01). At III stage of LA dilatation in patients of the 1st group level eNOS was 344.20±51.98 pg/ml in the patients of the 2nd group – 616.90±36.49 pg/ml (p<0.01). At the same degree and with LA dilation in the patients of the 2nd group eNOS was 750.27±99.85 pg/ml. Conclusions. The structural and functional changes of the left atrium and changing factors of vasodilation in patients with stable coronary artery disease of II-III functional classes complicated by heart failure of I-III functional classes are studied. It is established that in the examined patients with stable coronary heart disease complicated by heart failure with reduced left ventricular ejection fraction mainly III (significant) degree of dilatation of the left atrium was determined, while in patients with stable coronary heart disease, complicated by heart failure with preserved left ventricular ejection fraction mainly II (moderate) degree of dilation of the left atrium was determined. In comparison with the results of research among the patients with stable coronary heart disease, complicated by heart failure with reduced left ventricular ejection fraction, and a group of patients with preserved left ventricular ejection fraction, it was determined a significant decrease in eNOS, nitrites, total amount of nitrites and nitrates.


2021 ◽  
Author(s):  
Adrian Carlessi ◽  
Leonel Perello ◽  
Cristian Pantaley ◽  
Armando Borsini ◽  
Lucia Rossi ◽  
...  

Abstract Background The disease caused by coronavirus (COVID-19) affects the cardiovascular system, whether by direct viral aggression or indirectly through systemic inflammation and multiple organ compromise. A widely used method to determine cardiac injury is troponin measurement. The aim of this study is to evaluate the prevalence of cardiac involvement (CINV) in a population recovered from COVID-19, referred to cardiac MRI (CMR), who did not present troponin elevation. Methods There were 156 patients that recovered from COVID-19 and who did not present troponin elevation referred to CMR. CINV was considered to be the presence of: late gadolinium enhancement (LGE), edema, myocarditis, pericarditis, left ventricular systolic dysfunction (LVSD) and/or depressed right ventricular systolic dysfunction (RVSD). Results Prevalence of CINV was 28.8%, being more frequent in men (p = 0.002), in patients who required hospitalization (p = 0.04) and in those who experienced non-mild cases of infection (p = 0.007). RVSD (17.9%) and LVSD (13.4%) were the most frequent findings. The rate of myocarditis was 0.6%. LGE manifested in 7.1% of patients and its presence was related to less left ventricular ejection fraction (LVEF) (p = 0.0001) and right ventricular ejection fraction (RVEF) (p = 0.04). Conclusion In patients who recovered from COVID-19, 28.8% of CINV was found. It was more frequent in men, in patients who required admission and in patients with cases of non-mild infection. The patients that presented LGE had less LVEF and RVSF.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4872-4872
Author(s):  
Wesley Witteles ◽  
Ronald Witteles ◽  
Sally Arai ◽  
Richard Lafayette ◽  
Stanley Schrier

Abstract Abstract 4872 Backgroud Prognosis in AL amyloidosis is commonly viewed as dichotomous, based on the presence or absence of cardiac involvement. Patients with cardiac AL amyloidosis are reported to have a dismal prognosis, with a median survival of approximately 6 months. Furthermore, cardiac involvement is often considered a contraindication to hematopoietic cell transplant. Aims To compare the prognosis of patients with cardiac AL amyloidosis with and without presenting symptoms of congestive heart failure (CHF). Methods The study population consisted of individuals with biopsy-proven AL amyloidosis and cardiac involvement seen at the Stanford University Amyloid Center. Cardiac involvement was defined by the presence of amyloid deposits on endomyocardial biopsy or the combination of increased ventricular mass with reduced electrocardiographic voltage. Results A total of 27 patients (median age 64 years, 37% female) were evaluated. The 16 patients with CHF symptoms had a median age of 65 years with a median of 2 organs involved with amyloidosis. The 11 patients without CHF symptoms had a median age of 59 years with a median of 3 organs involved. At diagnosis, NT-proBNP was significantly higher in the group with CHF symptoms (15,938 vs. 2,538 pg/ml, P<0.001), as was the likelihood of detectable troponin I (86% s. 36%, P<0.02). Mean levels of the involved serum free light chain (179.7 vs. 47.9 mg/dL) were nonsignificantly higher in the cohort with CHF. Though there was no difference in the mean interventricular septal width between the two groups (1.5 cm), the mean systolic blood pressure (sBP) (91 vs. 115 mm Hg, P<0.001) and left ventricular ejection fraction (45 vs. 59%, p=0.02) were significantly lower in the group with CHF symptoms. A total of 6 patients (4 without CHF symptoms, 2 with CHF symptoms) received hematopoietic cell transplants, and 3 patients received heart transplants. Other therapies employed included oral melphalan (6 patients), dexamethasone (19 patients), lenalidomide (17 patients), and bortezomib (4 patients). After a median follow-up of 24 months, there were 9 deaths in the cohort with CHF symptoms vs. 0 in the group without CHF symptoms. Six-month survival (50% vs. 100%, P<0.01) and one-year survival (42% vs. 100%, P<0.01) were significantly lower in the group with CHF symptoms. Discussion CHF at presentation is the primary factor which predicts a poor prognosis in patients with cardiac AL amyloidosis. In our cohort, no patient without CHF symptoms at diagnosis later developed CHF, and thus new CHF symptoms do not appear to be a typical consequence of progressive disease. Conclusion Not all patients with cardiac AL amylodosis have an exceptionally poor prognosis. Many patients have ‘incidental’ cardiac involvement in the absence of CHF symptoms and appear to do well with both standard and intensive therapies. Disclosures Witteles: Celgene: Research Funding. Off Label Use: Melphalan, lenalidomide & bortezomib as therapy for AL amyloidosis.. Witteles:Celgene: Research Funding. Schrier:Celgene: Research Funding.


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