Vidyani Suryadevara, Michael Kluppel, Federica Del Monte, Monte S Willis
Alzheimer disease (AD), characterized by deterioration of cognitive capabilities, is prevalent among 44 million people worldwide. Beyond memory deficits, the most common AD co-morbidities include swallowing defects (muscle), fractures (bone, muscle), and heart failure. The underlying causes of these co-morbidities and their role in AD pathophysiology are currently unknown. This review is the first to summarize the emerging picture of the cardiac and musculoskeletal deficits in human AD. We present the involvement of the heart, characterized by diastolic heart failure, the presence of amyloid deposits, and electrophysiological changes compared to age-matched controls...
May 11, 2020: American Journal of Pathology
Muhammed Kermali, Raveena Kaur Khalsa, Kiran Pillai, Zahra Ismail, Amer Harky
Aims: As of the 28th April 2020, the COVID-19 pandemic has infiltrated over 200 countries and affected over three million confirmed people. We review different biomarkers to evaluate if they are able to predict clinical outcomes and correlate with the severity of COVID-19 disease. Methods: A systematic review of the literature was carried out to identify relevant articles using six different databases. Keywords to refine the search included 'COVID-19', 'SARS-CoV2', 'Biomarkers', among others...
May 13, 2020: Life Sciences
Sofia Möckelind, Jan Axelsson, Björn Pilebro, Per Lindqvist, Ole B Suhr, Torbjörn Sundström
Background: Hereditary transthyretin amyloid (ATTRv) is a systemic amyloidosis with mainly neurological and cardiac symptoms. The aim of this study was to evaluate the outcome of [18 F]Flutemetamol PET/CT-scan of the heart in long-term survivors with ATTRV30M amyloidosis. Methods: Twenty-one patients with ATTRV30M amyloidosis and predominantly neurological symptoms, mainly negative on cardiac 99m technetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD)-scintigraphy, were examined with a dynamic [18 F]Flutemetamol PET/CT-scan...
May 13, 2020: Amyloid: the International Journal of Experimental and Clinical Investigation
Boyangzi Li, Monica Ahluwalia, Navneet Narula, Andre L Moreira, Daniel G Swistel, Daniele Massera, Mark V Sherrid
Cardiac amyloid A (AA) amyloidosis is rare. We present the case of a 72-year-old woman with obstructive hypertrophic cardiomyopathy (HCM) and biopsy-proven renal AA amyloidosis whose dyspnea and exercise intolerance had worsened over the previous year. Her AA amyloidosis was suspected to be secondary to chronic diverticulitis for which she had undergone hemicolectomy and sigmoidectomy 3 years prior. Echocardiographic findings were consistent with worsening left ventricular outflow tract obstruction at rest...
March 7, 2020: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Christopher D Barrett, Kevin M Alexander, Hongyu Zhao, Francois Haddad, Paul Cheng, Ronglih Liao, Matthew T Wheeler, Michaela Liedtke, Stanley Schrier, Sally Arai, Dana Weisshaar, Ronald M Witteles
OBJECTIVES: The purpose of this study is to report outcomes after heart transplantation in patients with cardiac amyloidosis based on a large single-center experience. BACKGROUND: Cardiac amyloidosis causes significant morbidity and mortality, often leading to restrictive cardiomyopathy, progressive heart failure, and death. Historically, heart transplantation outcomes have been worse in patients with cardiac amyloidosis compared with other heart failure populations, in part due to the systemic nature of the disease...
May 4, 2020: JACC. Heart Failure
Rudolf A Werner, Frank M Bengel, Thorsten Derlin
BACKGROUND: Positron-emission-tomography (PET) using the radiopharmaceutical 18F-fluorodeoxyglucose (FDG) has become an established and validated molecular imaging modality for characterization of the inflammatory activity of atherosclerotic plaque. In the latest years, new innovative radiopharmaceuticals and applications have emerged, providing specific information on atherosclerotic plaque biology, particularly focused on inflammatory processes. OBJECTIVE: To review and highlight recent evidence on the role of PET for atherosclerosis imaging using emerging radiotracers...
May 4, 2020: Current Radiopharmaceuticals
Nicholas S Hendren, Lori R Roth, Justin L Grodin
PURPOSE OF REVIEW: Transthyretin amyloidosis is an increasingly recognized cause of restrictive cardiomyopathy related to amyloid fibril deposition in cardiac tissues. As treatment therapies have emerged for transthyretin amyloidosis (ATTR), so has interest in using biomarkers to identify disease prior to advanced presentation. RECENT FINDINGS: Lower levels of transthyretin and retinol binding protein-4 have been demonstrated in patients with pathogenic mutations of transthyretin either with or without clinical disease...
April 30, 2020: Current Heart Failure Reports
Stefano Tambuzzi, Salvatore Andreola, Michele Boracchi, Paolo Fociani, Guendalina Gentile, Riccardo Zoja
In a previous study, we presented a case of an elderly woman's sudden death, in which microscopic examinations showed intramyocardial eosinophilic material suspected for amyloid, but not definable as such to the classic Congo Red staining. To overcome the arisen interpretative and diagnostic difficulties, we experimentally modified the classic Congo Red staining, using a specific one for corpse. The finding of a low-intensity positivity allowed us to formulate a very likely diagnosis of occult lethal cardiac amyloidosis...
April 2020: Journal of Forensic and Legal Medicine
Anvesh K R Dasari, Ivan Hung, Brian Michael, Zhehong Gan, Jeffery W Kelly, Lawreen H Connors, Robert G Griffin, Kwang Hun Lim
Structural characterization of misfolded protein ag-gregates is essential to understanding molecular mechanism of protein aggregation associated with various protein misfolding disorders. Here, we re-port structural analyses of ex vivo transthyretin ag-gregates extracted from human cardiac tissue. Comparative structural analyses of in vitro and ex vivo transthyretin aggregates using various bio-physical techniques revealed that cardiac transthy-retin amyloid has similar structural features to those of in vitro transthyretin amyloid...
April 27, 2020: Biochemistry
Naoko Sawada, Masao Daimon, Hiroyuki Abe, Tetsuo Ushiku, Tomoko S Kato, Hiroyuki Morita, Issei Komuro
No abstract text is available yet for this article.
April 2020: CASE: Cardiovascular Imaging Case Reports
Ariane Vieira Scarlatelli Macedo, Pedro Vellosa Schwartzmann, Breno Moreno de Gusmão, Marcelo Dantas Tavares de Melo, Otávio Rizzi Coelho-Filho
Cardiac amyloidosis is associated with a high mortality rate, a long delay between the first signs and the diagnosis but a short interval between diagnosis and death. This scenario has changed recently due to improved disease awareness among doctors and significant progress in diagnosis thanks to multimodal imaging and a multidisciplinary approach. Therefore, during the last few years, we have had access to specific therapies for those patients. Those therapies are quite different depending on the type of amyloidosis, but there has been real progress...
April 23, 2020: Current Treatment Options in Oncology
Ángela López-Sainz, Aitor Hernandez-Hernandez, Esther Gonzalez-Lopez, Fernando Domínguez, Maria Alejandra Restrepo-Cordoba, Marta Cobo-Marcos, Manuel Gómez-Bueno, Francisco Jose Hernandez-Perez, Juan Francisco Oteo, Jesus G Mirelis, Miguel Angel Cavero, Vanessa Moñivas, Susana Mingo Santos, F Javier de Haro-Del Moral, Isabel Krsnik, Clara Salas, Belén Bornstein, Ana Briceño, Juan Antonio López, Jesús Vázquez, Luis Alonso-Pulpón, Javier Segovia, Pablo Garcia-Pavia
INTRODUCTION AND OBJECTIVES: Cardiac amyloidosis (CA) is produced by amyloid fiber deposition in the myocardium. The most frequent forms are those caused by light chains (AL) and transthyretin (ATTR). Our objective was to describe the diagnosis, treatment and outcomes of CA in a specialized Spanish center. METHODS: We included all patients diagnosed with CA in Hospital Universitario Puerta de Hierro Majadahonda from May 2008 to September 2018. We analyzed their clinical characteristics, outcomes, and survival...
April 18, 2020: Revista Española de Cardiología
Ahmed Fathala
Cardiac amyloidosis (CA) is an important cause of restrictive cardiomyopathy and heart failure with preserved ejection fraction (HFpEF). At present, 3 bone-seeking tracers, 99m Tc-diphosphono-1,2-propanodicarboxylic acid (99m Tc-DPD), 99m Tc-pyrophosphate (99m Tc-PYP), and 99m Tc-hydroxymethylene diphosphonate (99m Tc-HMDP), have been evaluated for detecting CA, but they are not widely available. In contrast, methylene diphosphate (MDP) is widely available. However, only sporadic case reports have shown that MDP can accumulate in patients with CA...
June 2020: Radiology Case Reports
Paul R Scully, Kush P Patel, Thomas A Treibel, George D Thornton, Rebecca K Hughes, Sucharitha Chadalavada, Michail Katsoulis, Neil Hartman, Marianna Fontana, Francesca Pugliese, Nikant Sabharwal, James D Newton, Andrew Kelion, Muhiddin Ozkor, Simon Kennon, Michael Mullen, Guy Lloyd, Leon J Menezes, Philip N Hawkins, James C Moon
AIMS: Cardiac amyloidosis is common in elderly patients with aortic stenosis (AS) referred for transcatheter aortic valve implantation (TAVI). We hypothesized that patients with dual aortic stenosis and cardiac amyloid pathology (AS-amyloid) would have different baseline characteristics, periprocedural and mortality outcomes. METHODS AND RESULTS: Patients aged ≥75 with severe AS referred for TAVI at two sites underwent blinded bone scintigraphy prior to intervention (Perugini Grade 0 negative, 1-3 increasingly positive)...
April 8, 2020: European Heart Journal
Adam S Hafeez, Anthony A Bavry
Transthyretin amyloid cardiomyopathy (ATTR-CM) continues to be an easily overlooked, life-threatening, yet treatable cause of heart failure. Furthermore, its elusive diagnosis leads to late or misdiagnosis. As therapeutic advancements such as tafamidis usher in a promising new era in the management of ATTR-CM, the need for disease awareness and efficient diagnostic evaluation is crucial. With newer inexpensive imaging modalities and techniques, such as longitudinal strain imaging, T1 mapping on cardiac magnetic resonance imaging, and cardiac scintigraphy, the diagnosis of ATTR-CM no longer requires invasive evaluation with tissue biopsy...
April 7, 2020: Cardiology and Therapy
Ahamed Shaheer Ahmed, Sampath Kumar, Gautam Sharma, Sudheer Arava
A 50-year-old woman presented with complaints of palpitations and breathlessness of 6 months' duration. She was being treated elsewhere as a case of dilated cardiomyopathy. On evaluation she had racoon eyes, poor progression of R wave on ECG and low-voltage complexes in the limb leads. Echocardiography revealed biventricular hypertrophy, diastolic dysfunction and moderate systolic dysfunction. Cardiac MRI showed features suggestive of amyloidosis. Bone marrow biopsy revealed raised plasma cell count, and endomyocardial biopsy showed amyloid deposits in the myocardium...
April 6, 2020: BMJ Case Reports
Fangfang Zhang, Christopher J Lyon, Robert J Walls, Bo Ning, Jia Fan, Tony Y Hu
Improvements in long-term cancer survival rates have resulted in an increase in the prevalence of chemotherapy-linked cardiac failure, but treatment-induced cardiac injuries may not be detected until long after therapy. Monitoring cardiac function is recommended; however, cardiovascular injury in cancer patients differs from those with primary cardiac dysfunction, which limits the utility of traditional cardiac biomarkers. Here we examined plasma levels of peptides produced by cathepsin B, which is released during chemotherapy-induced cardiac injury...
October 11, 2019: ACS Pharmacology & Translational Science
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
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