Cardiac amyloid

Seungho Choi, Inderjit Singh, Avtar K Singh, Mushfiquddin Khan, Jeseong Won
Asymmetric dimethylarginine (ADMA), an endogenous inhibitor and uncoupler of nitric oxide synthase, has gained attention as a risk factor for cardiac disease, metabolic syndrome, and cerebrovascular disease. In this study, we investigated the role of systemic ADMA overburden in cerebromicrovascular pathology associated with cognitive dysfunction using APPSwDI transgenic mice expressing human β-amyloid precursor protein Swedish (Tg-SwDI), a model of cerebrovascular β-amyloidosis. To induce systemic overburden of ADMA, Tg-SwDI mice were treated with a daily dose of exogenous ADMA...
April 2, 2020: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
M Teresa Cibeira, José T Ortiz-Pérez, Luis F Quintana, Carlos Fernádez de Larrea, Natalia Tovar, Joan Bladé
Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response. The decrease in the production of the amyloidogenic light chains is a required condition to obtain the organ response, which is commonly delayed. Meanwhile, supportive treatment is aimed to maintain quality of life of these patients and preserve their involved organs' function...
April 1, 2020: Acta Haematologica
Alejandra González-Duarte, Isabel Conceição, Leslie Amass, Marc F Botteman, John A Carter, Michelle Stewart
INTRODUCTION: Hereditary (variant) transthyretin amyloidosis (ATTRv) with polyneuropathy (ATTR-PN) is a rare genetic disorder that causes progressive autonomic and sensorimotor neuropathy, severe disability, and death within 10 years of onset. Previous studies have primarily focused on how baseline cardiac characteristics affect mortality, but the impact of non-cardiac baseline characteristics is less defined. METHODS: We systematically searched PubMed/Medline (1990-2019) to identify studies that assessed the impact of baseline ATTR-PN characteristics on survival...
March 31, 2020: Neurology and Therapy
Riccardo Caccialanza, Emanuele Cereda, Catherine Klersy, Paolo Milani, Silvia Cappello, Valentina Martinelli, Annalisa Turri, Marco Basset, Valeria Borioli, Mario Nuvolone, Marilisa Caraccia, Francesca Lavatelli, Sara Masi, Federica Lobascio, Andrea Foli, Giampaolo Merlini, Giovanni Palladini
Background: The aim of the present prospective study ( Identifier: NCT02111538) was to assess the prognostic value of phase angle (PhA), derived from bioimpedance vectorial analysis (BIVA), in patients affected by systemic amyloid light-chain (AL) amyloidosis. Methods: One hundred-twenty seven consecutive newly diagnosed, treatment-naïve patients with histologically confirmed AL amyloidosis were enrolled. Nutritional assessment including BIVA-derived PhA was performed before treatment initiation...
March 26, 2020: Amyloid: the International Journal of Experimental and Clinical Investigation
Eva Maria Greunz, Karin Lemberger, Jérôme Catinaud, Baptiste Chenet, Reinhold Paul Linke, Jan Hinrich Bräsen, Jessica Schmitz, Mads Frost Bertelsen
Nine cases of amyloidosis in caracals ( Caracal caracal ) from three different institutions in Europe were reviewed and evaluated histopathologically. The six males and three females died between 2008 and 2018 at an age of 6 yr ± 2.5 mo (median ± interquartile range). In two out of nine (2/9) animals, amyloidosis was an incidental postmortem finding; the animals died of bronchopneumonia and gastric ulceration due to Helicobacter spp., respectively. Seven (7/9) animals suffered from acute renal failure due to amyloidosis, one of them additionally of cardiac decompensation...
March 17, 2020: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Anahita Tavoosi, Boxin Yu, Nazanin Aghel, Gauri R Karur, Mini Pakkal, Rachel Wald, Bernd J Wintersperger, Diego H Delgado, Kate Hanneman
PURPOSE: To evaluate the diagnostic utility of the Look Locker inversion time (TI) sequence on cardiac magnetic resonance imaging in patients with suspected cardiac amyloidosis and to evaluate whether there are differences in the nulling pattern between amyloid types. MATERIALS AND METHODS: A total of 144 patients with suspected cardiac amyloidosis who had undergone cardiac magnetic resonance imaging were included in this retrospective study. Sixty-four had cardiac amyloidosis (62...
March 17, 2020: Journal of Thoracic Imaging
Varinder K Randhawa, Sneha Vakamudi, Dermot M Phelan, Christy J Samaras, Jesse K McKenney, Mazen Hanna, Antonio L Perez
Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis...
March 16, 2020: ESC Heart Failure
Sunil Kumar Dubey, K K Lakshmi, Kowthavarapu Venkata Krishna, Mukta Agrawal, Gautam Singhvi, Ranendra Narayana Saha, Swarnalat Saraf, Shailendra Saraf, Rahul Shukla, Amit Alexander
Alzheimer's disease, a progressive neurodegenerative disorder, is one of the leading causes of death in the USA, along with cancer and cardiac disorders. AD is characterized by various neurological factors like amyloid plaques, tau hyperphosphorylation, mitochondrial dysfunction, acetylcholine deficiency, etc. Together, impaired insulin signaling in the brain is also observed as essential factor to be considered in AD pathophysiology. Hence, currently researchers focused on studying the effect of brain insulin metabolism and relation of diabetes with AD...
March 9, 2020: Life Sciences
Nathan Malka, Mukedaisi Abulizi, Mounira Kharoubi, Silvia Oghina, Arnault Galat, Fabien Le Bras, Anissa Moktefi, Soulef Guendouz, Valérie Molinier-Frenkel, Pascale Fanen, Benoît Funalot, Jean-Pascal Lefaucheur, Paul Blanc-Durand, Jean-François Deux, Vincent Audard, Diane Bodez, Emmanuel Itti, Thibaud Damy
PURPOSE: Increased cardiac uptake (CU) on early-phase 99m Tc-HMDP scintigraphy has demonstrated diagnostic and prognostic values in amyloid transthyretin (ATTR) cardiac amyloidosis (CA). Extracardiac uptake (ECU) has been poorly studied. We assessed the clinical value of ECU, in combination with CU, on 99m Tc-HMDP scintigraphy using a novel Methodological Amyloidosis Diagnostic Index (MADI). METHODS: We reviewed all patients referred for suspicion of CA, who underwent 99m Tc-HMDP scintigraphy over an 8-year period...
March 10, 2020: European Journal of Nuclear Medicine and Molecular Imaging
Nowell M Fine, James A White, Victor Jimenez-Zepeda, Jonathan G Howlett
BACKGROUND: Right heart function is an important prognostic determinant in cardiac amyloidosis. In this study we characterized serial changes in right and left heart function and evaluated their prognostic significance. METHODS: Cardiac amyloidosis patients with baseline and follow-up echocardiograms were included. Right and left heart function measured at baseline, 1 year, and most recent follow-up were compared and correlated with all-cause mortality or cardiovascular hospitalization...
March 2020: Canadian Journal of Cardiology
Christopher C Cheung, Thomas M Roston, Jason G Andrade, Matthew T Bennett, Margot K Davis
Cardiac amyloidosis occurs secondarily to the deposition of insoluble protein fibrils in cardiac tissue leading to progressive myocardial dysfunction, clinical heart failure, and arrhythmia. In recent years, increasing awareness and improved screening have resulted in an increased prevalence of cardiac amyloidosis, with contemporary estimates reporting a prevalence of 18-55 cases per 100,000 person-years, accounting for > 13% of heart failure hospitalizations. The arrhythmic manifestations of cardiac amyloidosis can range from conduction-system disease and bradyarrhythmias to atrial fibrillation and sudden cardiac death...
March 2020: Canadian Journal of Cardiology
Sarah A M Cuddy, Rodney H Falk
The systemic amyloidoses are a group of diseases characterized by the deposition of amyloid, a material formed from misfolding of proteins, in one or more organs. The 2 commonest forms of amyloidosis are transthyretin amyloidosis (ATTR), derived from wild-type or mutant transthyretin, and light-chain (AL) amyloidosis, derived from abnormal circulating light chains produced by plasma cell dyscrasia. Both frequently involve the heart, producing an infiltrative cardiomyopathy with restrictive pathophysiology. Although advances in echocardiographic, magnetic resonance, and nuclear imaging have rendered diagnosis of cardiac amyloidosis easier, diagnosis is still often delayed...
March 2020: Canadian Journal of Cardiology
Bennett Di Giovanni, Dakota Gustafson, Mitchell B Adamson, Diego H Delgado
Amyloidosis is a term used to describe a group of rare heterogeneous diseases that ultimately result in the deposition and accumulation of misfolded proteins. These misfolded proteins, known as amyloids, are associated with a variety of precursor proteins that have amyloidogenic potential. Ultimately, the specific type of amyloidosis is dependent on multiple factors including genetic variability of precursor proteins and the tissue or organ in which the amyloid accumulates. Several types of amyloid have a predilection for the heart and thus contribute to cardiac amyloidosis, a major cause of restrictive cardiomyopathy...
March 2020: Canadian Journal of Cardiology
Nowell M Fine, Margot K Davis, Kim Anderson, Diego H Delgado, Genevieve Giraldeau, Abhijat Kitchlu, Rami Massie, Jane Narayan, Elizabeth Swiggum, Christopher P Venner, Anique Ducharme, Natalie J Galant, Christopher Hahn, Jonathan G Howlett, Lisa Mielniczuk, Marie-Claude Parent, Donna Reece, Virginie Royal, Mustafa Toma, Sean A Virani, Shelley Zieroth
Cardiac amyloidosis is an under-recognized and potentially fatal cause of heart failure and other cardiovascular manifestations. It is caused by deposition of misfolded precursor proteins as fibrillary amyloid deposits in cardiac tissues. The two primary subtypes of systemic amyloidosis causing cardiac involvement are immunoglobulin light chain (AL), a plasma cell dyscrasia, and transthyretin (ATTR), itself subdivided into a hereditary subtype caused by a gene mutation of the ATTR protein, and an age-related wild type, which occurs in the absence of a gene mutation...
March 2020: Canadian Journal of Cardiology
Hailey A Kresge, Dandan Liu, Deepak K Gupta, Elizabeth E Moore, Katie E Osborn, Lealani Mae Y Acosta, Susan P Bell, Kimberly R Pechman, Katherine A Gifford, Lisa A Mendes, Thomas J Wang, Kaj Blennow, Henrik Zetterberg, Timothy J Hohman, Angela L Jefferson
BACKGROUND: Subclinical cardiac dysfunction is associated with decreased cerebral blood flow, placing the aging brain at risk for Alzheimer's disease (AD) pathology and neurodegeneration. OBJECTIVE: This study investigates the association between subclinical cardiac dysfunction, measured by left ventricular ejection fraction (LVEF), and cerebrospinal fluid (CSF) biomarkers of AD and neurodegeneration. METHODS: Vanderbilt Memory & Aging Project participants free of dementia, stroke, and heart failure (n = 152, 72±6 years, 68% male) underwent echocardiogram to quantify LVEF and lumbar puncture to measure CSF levels of amyloid-β42 (Aβ42), phosphorylated tau (p-tau), and total tau (t-tau)...
March 2, 2020: Journal of Alzheimer's Disease: JAD
Dimitrios A Stakos, Kimon Stamatelopoulos, Dimitrios Bampatsias, Marco Sachse, Eleftherios Zormpas, Nikolaos I Vlachogiannis, Simon Tual-Chalot, Konstantinos Stellos
Aging-related cellular and molecular processes including low-grade inflammation are major players in the pathogenesis of cardiovascular disease (CVD) and Alzheimer's disease (AD). Epidemiological studies report an independent interaction between the development of dementia and the incidence of CVD in several populations, suggesting the presence of overlapping molecular mechanisms. Accumulating experimental and clinical evidence suggests that amyloid-beta (Aβ) peptides may function as a link among aging, CVD, and AD...
March 3, 2020: Journal of the American College of Cardiology
Marco Luigetti, Angela Romano, Andrea Di Paolantonio, Giulia Bisogni, Mario Sabatelli
Hereditary transthyretin amyloidosis (hATTR) with polyneuropathy (formerly known as Familial Amyloid Polyneuropathy) is a rare disease due to mutations in the gene encoding transthyretin ( TTR ) and characterized by multisystem extracellular deposition of amyloid, leading to dysfunction of different organs and tissues. hATTR amyloidosis represents a diagnostic challenge for neurologists considering the great variability in clinical presentation and multiorgan involvement. Generally, patients present with polyneuropathy, but clinicians should consider the frequent cardiac, ocular and renal impairment...
2020: Therapeutics and Clinical Risk Management
Arnt V Kristen
Cardiac amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of amyloid fibrils in many different organs finally resulting in organ failure. Cardiac involvement is common for immunoglobulin light chain amyloidosis (AL) or transthyretin amyloidosis (ATTR); the latter is caused by a transthyretin gene variant or wild-type protein. Precise diagnostic assessment including laboratory tests, electrocardiography, echocardiography, cardiac magnetic resonance imaging, biopsy, and/or bone scintigraphy is mandatory for definition of the amyloid type and finally for treatment initiation...
February 27, 2020: Herz
James J Nawarskas, Emily A Shephard
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a life-threatening condition in which amyloid fibrils accumulate in the heart, eventually leading to cardiac symptomatology and death. To date, treatment of this condition has been directed at symptom relief due to a lack of effective treatment options which target the cause of the disease. The discovery that amyloid deposition was a result of dissociation of the transthyretin protein structure allowed for the development of tafamidis, which acts by stabilizing the transthyretin tetramer...
February 24, 2020: Cardiology in Review
Riccardo Zucchi
The development of thyroid hormone (TH) analogues was prompted by the attempt to exploit the effects of TH on lipid metabolism, avoiding cardiac thyrotoxicosis. Analysis of the relative distribution of the  and  subtypes of nuclear thyroid hormone receptors (TR and TR), showed that TR and TR are responsible for cardiac and metabolic responses, respectively. Therefore, analogues with TR selectivity were developed, and four different compounds have been used in clinical trials: GC-1 (sobetirome), KB-2115 (eprotirome), MB07344/VK2809, and MGL-3196 (resmetirom)...
February 26, 2020: Thyroid: Official Journal of the American Thyroid Association
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