scholarly journals The Role of Light Kappa and Lambda Chains in Heart Function Assessment in Patients with AL Amyloidosis

2021 ◽  
Vol 10 (6) ◽  
pp. 1274
Author(s):  
Emilia Czyżewska ◽  
Agnieszka Wiśniewska ◽  
Anna Waszczuk-Gajda ◽  
Olga Ciepiela
Keyword(s):  
Troponin I ◽  
Heart Function ◽  
Myocardial Dysfunction ◽  
Interventricular Septum ◽  
Myocardial Damage ◽  
Amyloid Deposit ◽  
Al Amyloidosis ◽  
Heart Involvement ◽  
Immunoglobulin Light Chain Amyloidosis

There are reports indicating that myocardial dysfunction in systemic immunoglobulin light chain amyloidosis (AL amyloidosis) stems not only from the amyloid deposit in the organ but also the cardiotoxicity of the amyloid precursor free light chains (FLCs) circulating in the blood. The aim of the study is to analyze the role of sFLC κ and λ in the assessment of heart involvement and the degree of myocardial damage in AL amyloidosis. The study involved 71 patients diagnosed with primary AL amyloidosis. The relationship between sFLC concentrations and cardiac biochemical and echocardiographic parameters was assessed. The median concentrations of N-terminal pro b-type natriuretic peptide(NT-proBNP) and troponin I (TnI) were significantly higher in patients with amyloids formed from monoclonal λ chains compared to patients with monoclonal κ proliferation. In patients with heart involvement by amyloids formed from monoclonal FLC, the study demonstrated a statistically significant positive correlation between the concentration of monoclonal antibody λ chain and TnI (R = 0.688; p < 0.05), NT-proBNP (R = 0.449; p < 0.05), and the value of diastolic dimension of the interventricular septum (IVS; R = 0.496, p < 0.05). The above data indicate that the presence of monoclonal λ chains in patients with AL amyloidosis may be associated with more severe damage to cardiomyocytes and dysfunction of the myocardium.

Cardiac AL Amyloidosis – the role of serum Immunoglobulin Free Llight Chain assay (sFLC) in diagnosis and prognostic classification

2017 ◽  
Vol 53 (3) ◽  
pp. 175-184
Author(s):  
Emilia Czyżewska ◽  
Anna Rodziewicz-Lurzyńska ◽  
Dagna Bobilewicz
Keyword(s):  
Light Chain ◽  
Amyloid Fibrils ◽  
Cell Clone ◽  
Myocardial Dysfunction ◽  
Myocardial Damage ◽  
Amyloid Deposit ◽  
Al Amyloidosis ◽  
Neoplastic Process ◽  
The Difference ◽  
Immunoglobulin Light Chain Amyloidosis

Systemic immunoglobulin light chain amyloidosis (AL amyloidosis) is a cancer in which the amyloid fibrils are formed from amyloid immunoglobulin free light chain (FLC) κ or λ, produced by a plasma cell clone. The created protein adopts the structure of β-sheets, and depositing in tissues and organs leads to their function impairment. Cardiomyopathies are the main cause of death in patients with amyloidosis. Diagnosis of cardiac involvement is too late owing to nonspecific symptoms and no early characteristic changes observed in imaging studies. The currently valid prognostic classification of AL amyloidosis created by Kumar et al. is based on biochemical parameters evaluating function and the degree of myocardial damage: concentration of troponin (Tn) and N-terminal fragment of B-type natriuretic peptide (NT-proBNP) and the value of dFLC, representing the difference between the concentration of serum FLC (sFLC) involved and not involved in the neoplastic process. There are reports indicating that myocardial dysfunction in AL amyloidosis stems not only from the amyloid deposit in the organ, but it is also the result of the cardiotoxicity amyloid precursor – FLC, circulating in the blood.

Heart Function In Systemic and Localized AL Amyloidosis: Role of NT-ProBNP and MPC-1

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5006-5006
Author(s):  
Francesca Saltarelli ◽  
Alessandro Moscetti ◽  
Guglielmo Bruno ◽  
Bruno Monarca ◽  
Gerardo Salerno ◽  
...  
Keyword(s):  
Conflicts Of Interest ◽  
Heart Function ◽  
Systemic Disease ◽  
Plasma Concentrations ◽  
Serum Levels ◽  
Mononuclear Phagocytes ◽  
Systemic Amyloidosis ◽  
Al Amyloidosis ◽  
Heart Involvement ◽  
Localized Amyloidosis

Abstract Abstract 5006 In AL amyloidosis typical sites of amyloid buildup are heart, skin, gastrointestinal tract, liver, kidneys, and blood vessels. To evaluate the heart involvement in systemic and localized amyloidosis proBNP, peptide (NT-proBNP; 76 amino acids) and MPC-1 were investigated. NT-proBNP have been described as useful marker for the diagnosis heart disease, and its plasma concentrations correlate with the functional classification of patients according to the New York Heart Association (NYHA). MCP-1 is a chemokine that activates mononuclear phagocytes by promoting leukocyte–endothelium binding and migration to sites of inflammation. The MCP-1 levels seem to be related to the severity of cardiac alteration, as demonstrated by the coronary angiogram. NT-proBNP and MPC-1 serum levels were performed in systemic or localized AL amyloidosis to evaluate if there was a difference in the heart involvement. Blood samples were collected from 8 patients with systemic amyloidosis and from 4 patients with localized amyloidosis. To analyze the results of NT-proBNP and MPC-1, Mann-Whitney test was performed. NT-proBNP serum values were significantly (p=0.007) increased in systemic disease. Also, MPC-1 serum levels were significantly (p=0.004) higher in the patients with systemic disease (350.52±58.70 pg/ml) if compared to the group of localized amyloidosis (147.82±26.03 pg/ml). On the basis of our results, the heart seem to be functionally more involved in AL systemic amyloidosis than in localized disease, as demonstrated by the higher NT-proBNP and MPC-1 serum values. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Evaluation of myocardial injury patterns and ST changes among critical and non-critical patients with coronavirus-19 disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Anam Liaqat ◽  
Rao Saad Ali-Khan ◽  
Muhammad Asad ◽  
Zakia Rafique
Keyword(s):  
Mechanical Ventilation ◽  
Myocardial Injury ◽  
Troponin I ◽  
Myocardial Dysfunction ◽  
Invasive Mechanical Ventilation ◽  
Myocardial Damage ◽  
Military Hospital ◽  
Ecg Changes ◽  
Health Crisis ◽  
Critical Patients

AbstractNovel coronavirus disease (COVID-19) has led to a major public health crisis globally. Currently, myocardial damage is speculated to be associated with COVID-19, which can be seen as one of the main causes of death of patients with COVID-19. We therefore, aim to investigate the effects of COVID-19 disease on myocardial injury in hospitalized patients who have been tested positive for COVID-19 pneumonia in this study. A prospective study was conducted among 201 patients with COVID-19 in the Pakistan Military Hospital from April 1 to August 31, 2020, including non-critical cases and critical cases. COVID-19 patients were stratified as critical and non-critical according to the signs and symptoms severity; with those requiring intensive care and invasive mechanical ventilation as critical, and those did not requiring invasive mechanical ventilation as non-critical. A total of 201 COVID-19 patients with critical and non-critical categories presented with myocardial injury. All patients with myocardial injury had an elevation in CKMB and Troponin-I levels. Of these patients, 43.7% presented with new electrocardiography (ECG) changes, and ST depression was typically observed in 36.3% patients. In addition, 18.7% patients presented with abnormal echocardiography findings, with right ventricular dilatation and dysfunction commonly seen among critical group patients. Results analyzed by a logistic regression model showing COVID-19 direct contribution to myocardial injury in these patients. COVID-19 disease directly leads to cardiovascular damage among critical and non-critical patients. Myocardial injury is associated not only with abnormal ECG changes but also with myocardial dysfunction on echocardiography and more commonly observed among critical patients.

scholarly journals The Role of Lactate and Other Laboratory Markers on Detection of Subtle Myocardial Dysfunction in Critically ill Children

2021 ◽  
Vol 9 (T3) ◽  
pp. 265-269
Author(s):  
Munar Lubis ◽  
Aridamuriany Dwiputri Lubis ◽  
Badai Buana Nasution
Keyword(s):  
Critically Ill ◽  
Troponin I ◽  
Complete Blood Count ◽  
Myocardial Dysfunction ◽  
Gas Analysis ◽  
Critically Ill Children ◽  
Significant Finding ◽  
Early Assessment ◽  
Laboratory Markers

BACKGROUND: Critically ill patients have a high risk of developing life-threatening infections that can eventually lead to multi-organ failure. The cardiovascular system involvement could increase the mortality rate by 70-90%. Myocardial dysfunction is often accompanied by a state of metabolic acidosis, liver damage, kidney damage, and anemia. Therefore laboratory markers and elevated lactate levels may aid in the early assessment of a myocardial dysfunction AIM: The aim of this study was to prove the role of lactate and other laboratory markers on detection of subtle myocardial dysfunction (SMD) in critically ill children admitted to the Pediatric Intensive Care Unit (PICU). METHODS: An observasional cohort study in PICU Haji Adam Malik General Hospital, Medan. Assessment of complete blood count, kidney function, liver function, lactic acid, blood gas analysis, and troponin I within 48 hoursPICU admission. The results of the troponin value was said to be subtle myocardial dysfunction if the troponin I value is ≥ 0.4 ng/ml RESULT: 55 subjects were recruited in this study, 23 subject (41.1%) with SMD. Laboratory marker in SMD that has significant finding were lactate, AST, ALT, Hemoglobin (p = 0.003; p = 0.028; p = 0.01; p = 0.001, repectively). High lactate ( > 2.5 ng/ml) could be used as a predictor for SMD with sensitivity 74% and specificity 72%. Subject with SMD has significant association with mortality (p <0.001). CONCLUSION: Subtle myocardial dysfunction should be suspected in patient with blood lactate level > 2.5 ng/ml, with significant association between SMD and mortality.

Evaluation of Heart Function in Patients With Hemophilia

2015 ◽  
Vol 23 (4) ◽  
pp. 374-378 ◽  
Author(s):  
Hamid Amoozgar ◽  
Maedeh Fath ◽  
Parisa Jooya ◽  
Mehran Karimi
Keyword(s):  
Blood Pressure ◽  
Troponin I ◽  
Heart Function ◽  
Enzyme Level ◽  
Interventricular Septum ◽  
Tissue Doppler ◽  
Control Group ◽  
Cross Sectional ◽  
Diastolic Velocity ◽  
Significant Difference

There are conflicting reports about the protective effect of hemophilia on the occurrence of ischemic heart disease. This study focuses on evaluation of heart function in patients with hemophilia. Cross-sectional, case–control study was done on all patients with hemophilia A or B who came to hemophilia center, and data were compared to controls. The data were collected from their charts, and heart function was evaluated by 2-dimensional, Doppler and pulse tissue Doppler. The serum troponin I level was measured in all patients as a marker of myocardial damage. Fifty patients with hemophilia took part in this study. All of them were male with mean age 29.1 years. Systolic blood pressure (mean = 121.52 ± 11 vs 115.61 ± 9.81, P = .038) and diastolic (mean = 81.94 ± 4.51 vs 75.21 ± 3.95, P = .042) blood pressure were higher in the patients. Five (10%) patients had systolic hypertension and 7 (14%) patients had diastolic hypertension. The M-mode echocardiography results showed that interventricular septum in diastole in patients with hemophilia (mean 1.143 ± 0.29) was significantly thicker than the control group (mean 0.828 ± 0.22, P < .001). Tissue Doppler echocardiography showed that late diastolic velocity of septum (Aa; P = .030), systolic velocity (S) of lateral mitral valve ( P = .006), late diastolic velocity of lateral mitral (Aa) annulus ( P = .038), and late velocity of (Aa) tricuspid ( P = .004) had significant difference compared with the control group ( P < .05). Troponin enzyme level was < 0.1 in all patients. Patients with hemophilia had higher blood pressure and more hypertension. Echocardiographic study of patients with hemophilia showed some increase in septal thickness and changes in diastolic dysfunction

Study of Myocardial Dysfunction in Perinatal Asphyxia Field

2018 ◽  
Vol 2 (S1) ◽  
pp. e000127
Author(s):  
Kushali Tanna ◽  
K M Mehariya ◽  
Suchita Munsi ◽  
Charul Pujani
Keyword(s):  
Troponin I ◽  
Heart Diseases ◽  
Perinatal Asphyxia ◽  
Myocardial Dysfunction ◽  
Birth Asphyxia ◽  
Serum Levels ◽  
Spontaneous Respiration ◽  
Term Neonates ◽  
Myocardial Involvement ◽  
Ischemic Encephalopathy

Aims and Objectives: To study an incidence of myocardial dysfunction in neonates admitted with perinatal asphyxia, to find out its correlation with severity of birth asphyxia and its outcome. Methods: This prospective study was conducted among 40 term neonates admitted in NICU of Civil Hospital Ahmedabad who had suffered with perinatal asphyxia (defined by WHO ), resuscitated as per NRP guidelines-2015 including both intramural and extramural admissions and who developed to hypoxic ischemic encephalopathy as defined by Levene staging. Neonates with congenital heart diseases, major central nervous system malformations and neonatal sepsis were excluded. Myocardial involvement was assessed by clinical evaluation, ECG, Creatinine Kinase Total (25-200IU/L), CK-MB (0-25IU/L) and Troponin I (0-0.03ug/L) measurements. Results: Among 40 cases, 10(25%) neonates had moderate birth asphyxia while 30(75%) had severe birth asphyxia. Respiratory distress was observed in 34(77.5%), poor spontaneous respiration 4(10%),shock in 14(35%),CCF 19(47.5%) while ECG was abnormal in 30(76.7%). Serum levels of CPK Total, CPK- MB and Troponin I were raised in 34(85%), 32(80%) and 28 (70%) neonates, respectively.  Conclusion: There was a direct correlation between ECG changes and enzymatic levels which showed increasing abnormalities with increasing with severity of HIE.  

Clinical and Morphological Features of Myocardial Damage and the Course of Fulminant Myocarditis on the Background of СOVID-19, Diagnosis and Treatment Tactics

2020 ◽  
Vol 75 (5S) ◽  
pp. 414-425
Author(s):  
Olga S. Oynotkinova ◽  
Evgenii L. Nikonov ◽  
Oleg V. Zayratyants ◽  
Elena V. Rzhevskaya ◽  
Evgenii V. Krukov ◽  
...  
Keyword(s):  
Myocardial Dysfunction ◽  
Myocardial Damage ◽  
Heart Rhythm ◽  
Microvascular Dysfunction ◽  
Symptomatic Treatment ◽  
Clinical Situation ◽  
Diagnosis And Treatment ◽  
Screening Tests ◽  
Fulminant Myocarditis ◽  
Heart Rhythm Disorders

In a review article based on my own clinical experience of managing patients with acute myocardial injury and fulminant myocarditis, taking into account expert recommendations on the clinical treatment of myocardial damage associated with novel coronavirus infection a National clinical geriatric medical research center, division of cardiovascular diseases, the Chinese geriatrics society, Department of cardiology, Beijing Medical Association and European clinics discusses the pathogenesis, diagnosis and treatment of myocardial damage and FM patients, infected with SARS-CoV-2 in the context of the COVID-19 pandemic. Clinical features and diagnostic criteria are presented, including screening tests of markers of myocardial damage in the form of a highly sensitive troponin test, a natriuretic peptide. The article discusses in detail the pathogenesis and mechanisms of myocardial damage, including immune mechanisms, cytokine storm, systemic inflammation with macro- and microvascular dysfunction and the development of myocardial dysfunction with acute heart failure, hypotension, cardiogenic shock and/or life-threatening heart rhythm disorders caused by hypoxia and metabolic disorders at the cellular level. Features of the clinical course of fulminant myocarditis in infected patients (SARS-CoV-2) in the conditions of the COVID-19 pandemic are presented. For the first time, a detailed histo-morphological analysis of pathological myocardial injuries and complications is presented on the basis of unique autopsy material on post-mortem diagnostics of various pathoanatomic autopsies of those who died from COVID-19 in Moscow. Based on the clinical, functional and morphological material, the Protocol of etiopathogenetic treatment is presented. The basis of standard therapy is considered antiviral drugs, immunoglobulin G, the use of monoclonal antibodies to interleukin-6, anticoagulants, glucocorticoids, depending on the clinical situation, cardioprotectors and symptomatic treatment are recommended to maintain the heart, which in combination can achieve a certain clinical effectiveness. As adjuvant cardioprotective targeted therapy, the sodium salt of phosphocreatine is considered in order to preserve the myocardium, maintain its contractility and vital activity.

Bone disease as the first manifestation of systemic AL-amyloidosis

2020 ◽  
Vol 92 (7) ◽  
pp. 85-89
Author(s):  
L. P. Mendeleeva ◽  
I. G. Rekhtina ◽  
A. M. Kovrigina ◽  
I. E. Kostina ◽  
V. A. Khyshova ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Bone Marrow ◽  
Bone Disease ◽  
Plasma Cells ◽  
Interventricular Septum ◽  
Bone Destruction ◽  
Amyloid Deposition ◽  
Bone Lesions ◽  
Al Amyloidosis ◽  
Linear Type

Our case demonstrates severe bone disease in primary AL-amyloidosis without concomitant multiple myeloma. A 30-year-old man had spontaneous vertebral fracture Th8. A computed tomography scan suggested multiple foci of lesions in all the bones. In bone marrow and resected rib werent detected any tumor cells. After 15 years from the beginning of the disease, nephrotic syndrome developed. Based on the kidney biopsy, AL-amyloidosis was confirmed. Amyloid was also detected in the bowel and bone marrow. On the indirect signs (thickening of the interventricular septum 16 mm and increased NT-proBNP 2200 pg/ml), a cardial involvement was confirmed. In the bone marrow (from three sites) was found 2.85% clonal plasma cells with immunophenotype СD138+, СD38dim, СD19-, СD117+, СD81-, СD27-, СD56-. FISH method revealed polysomy 5,9,15 in 3% of the nuclei. Serum free light chain Kappa 575 mg/l (/44.9) was detected. Multiple foci of destruction with increased metabolic activity (SUVmax 3.6) were visualized on PET-CT, and an surgical intervention biopsy was performed from two foci. The number of plasma cells from the destruction foci was 2.5%, and massive amyloid deposition was detected. On CT scan foci of lesions differed from bone lesions at multiple myeloma. Bone fragments of point and linear type (button sequestration) were visualized in most of the destruction foci. The content of the lesion was low density. There was no extraossal spread from large zones of destruction. There was also spontaneous scarring of the some lesions (without therapy). Thus, the diagnosis of multiple myeloma was excluded on the basis based on x-ray signs, of the duration of osteodestructive syndrome (15 years), the absence of plasma infiltration in the bone marrow, including from foci of bone destruction by open biopsy. This observation proves the possibility of damage to the skeleton due to amyloid deposition and justifies the need to include AL-amyloidosis in the spectrum of differential diagnosis of diseases that occur with osteodestructive syndrome.

Estimation of the Safety of Intraoperative Fluid Therapy during Great Abdominal Surgery in Patients with Coronary Heart Disease

2020 ◽  
Vol 5 (5) ◽  
pp. 158-163
Author(s):  
V. I. Lysenko ◽  
◽  
E. A. Karpenko ◽  
Ya. V. Morozova
Keyword(s):  
Heart Failure ◽  
Coronary Heart Disease ◽  
Heart Disease ◽  
Fluid Therapy ◽  
Troponin I ◽  
Significant Proportion ◽  
Myocardial Damage ◽  
Enzyme Linked Immunosorbent Assay ◽  
Cardiac Complications ◽  
Fluid Regimen

The study of intraoperative fluid therapy tactics has been of great interest over the past few years, especially in people with concomitant coronary heart disease, as they make up a significant proportion of all surgical patients. The purpose of our study was to assess the risk of intraoperative myocardial damage in patients with concomitant coronary heart disease depending on the fluid regimen used based on monitoring of hemodynamic parameters, electrocardiogram and biomarkers of myocardial damage. Material and methods. The study involved 89 patients, who were divided into two groups depending on the tactics of intraoperative fluid therapy – restrictive and liberal. In order to detect cardiac complications at different stages, we assessed biomarkers of myocardial damage Troponin I, NT-proBNP by solid-phase enzyme-linked immunosorbent assay (ELISA). Results and discussion. Analysis of the obtained data showed that MINS (myocardial injury in noncardiac surgery) incidents were diagnosed in 5 patients (11.1%) in the first group and in 6 patients (13.6%) in the second. In patients of both groups there was an increase in NT-proBNP in the dynamics at all stages, and in the 2nd group, with a liberal regimen of intraoperative fluid therapy, it was more pronounced. It should be noted that the obtained values of NT-proBNP in all patients did not differ significantly from those allowed for this age group; such dynamics of NT-proBNP may indicate a relative risk of complications of liberal fluid therapy in patients with baseline heart failure. One of the important points when choosing the mode of fluid therapy in patients with high cardiac risk is the assessment of the initial volemic status and careful monitoring of water balance in the perioperative period with the desire for "zero" balance. The obtained dynamics of laboratory markers of myocardial damage indicates that in patients with a significant reduction in cardiac reserves compensated for heart failure, a restrictive fluid regimen is preferable, which is also confirmed by slight changes in the concentration of biomarkers. Conclusion. Thus, the study demonstrated the relative safety of selected fluid regimens in patients with concomitant coronary heart disease without signs of congestive heart failure

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