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Amyloid cardiomyopathy

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https://www.readbyqxmd.com/read/29915031/kinetic-analysis-of-the-multistep-aggregation-pathway-of-human-transthyretin
#1
Xun Sun, H Jane Dyson, Peter E Wright
Aggregation of transthyretin (TTR) is the causative agent for TTR cardiomyopathy and polyneuropathy amyloidoses. Aggregation is initiated by dissociation of the TTR tetramer into a monomeric intermediate, which self-assembles into amyloid. The coupled multiple-step equilibria and low-concentration, aggregation-prone intermediates are challenging to probe using conventional assays. We report a 19 F-NMR assay that leverages a highly sensitive trifluoroacetyl probe at a strategic site that gives distinct 19 F chemical shifts for the TTR tetramer and monomeric intermediate and enables direct quantification of their populations during the aggregation process...
June 18, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29791180/-heart-transplantation-and-follow-up-treatment-with-al-amyloidosis-in-5-patients
#2
Zdeněk Adam, Eva Ozábalová, Petr Němec, Helena Bedáňová, Milan Kuman, Jan Krejčí, Lenka Špinarová, Víta Žampachová, Zdeňka Čermáková, Luděk Pour, Marta Krejčí, Viera Sanecká, Martin Štork, Tomáš Pika, Jan Straub, Dagmar Adamová, Yvetta Stavařová, Zdeněk Král, Jiří Mayer
The prognosis for patients with cardiac impairment due to AL-amyloid deposition and severe cardiac insufficiency is poor, with a survival median in the order of months. The classical treatment of AL-amyloidosis in combination with cardiac insufficiency is very poorly tolerated and the treatment of such patients is associated with considerably higher mortality than among other patients with AL-amyloidosis. If, however, patients with an isolated or another dominating cardiac impairment, without severe damage to other organs and tissues, have a heart transplant performed, their cardiovascular condition will significantly improve as a result, along with their ability to tolerate any kind of treatment for AL-amyloidosis including that using high-dose chemotherapy with a transplant of autologous hematopoietic stem cells...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29779881/failure-of-tafamidis-to-halt-progression-of-ala36pro-ttr-oculomeningovascular-amyloidosis
#3
Fabrizio Salvi, Roberto Volpe, Francesca Pastorelli, Andrea Bianchi, Alessandra Vella, Claudio Rapezzi, Mario Mascalchi
Oculomeningovascular amyloidosis is a variant of transthyretin (TTR) amyloidotic polyneuropathy, which is associated with blindness and brain ischemia, microhemorrages, and siderosis due to prominent production of the abnormal TTR in the eye and in the choroid plexuses. Tafamidis is a TTR stabilizer that is orally administered and, by interfering with amyloid fibril formation and deposition, is capable of slowing progression of TTR polyneuropathy and of early-stage cardiomyopathy. However, the ocular manifestations of amyloid deposition progressed despite tafamidis therapy in Val30Met TTR amyloidosis, and the effects of tafamidis on meningovascular amyloidosis are unknown...
May 17, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29700090/cardiac-amyloidosis
#4
Ana Martinez-Naharro, Philip N Hawkins, Marianna Fontana
Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is paramount in light of recent therapeutic advances that can significantly improve prognosis...
April 1, 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29687207/diagnostic-accuracy-of-bone-scintigraphy-in-the-assessment-of-cardiac-transthyretin-related-amyloidosis-a-bivariate-meta-analysis
#5
Giorgio Treglia, Andor W J M Glaudemans, Francesco Bertagna, Bouke P C Hazenberg, Paola A Erba, Raffaele Giubbini, Luca Ceriani, John O Prior, Luca Giovanella, Riemer H J A Slart
PURPOSE: Cardiac transthyretin-related amyloidosis (ATTR) is a progressive and fatal cardiomyopathy. The diagnosis of this disease is frequently delayed or missed due to the limited specificity of echocardiography. An increasing amount of data in the literature demonstrate the ability of bone scintigraphy with bone-seeking radiopharmaceuticals to detect myocardial amyloid deposits, in particular in patients with ATTR. Therefore we performed a systematic review and bivariate meta-analysis of the diagnostic accuracy of bone scintigraphy in patients with suspected cardiac ATTR...
April 23, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29663861/amyloid-cardiomyopathy-a-hidden-heart-failure-cause-that-is-often-misdiagnosed
#6
Yale Tung-Chen, Miguel-Ángel Arnau
BACKGROUND: Congestive heart failure (CHF) is a very common condition, especially in the elderly, characterized by dyspnea, orthopnea, nocturnal paroxysmal dyspnea, and peripheral edema. CLINICAL PRESENTATION AND INTERVENTION: We report the case of a 76-year-old with CHF symptoms for the last 3 months. The ECG and transthoracic echocardiogram were suggestive of CHF due to amyloid cardiomyopathy. After cardiac MRI, a positive Congo red staining of subcutaneous fat aspiration and a negative genetic testing for mutant transthyretin, senile amyloid cardiomyopathy (ATTRw) was expected...
April 17, 2018: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29574594/endomyocardial-biopsy-at-the-time-of-ablation-or-device-implantation
#7
Ammar M Killu, Nishaki Mehta, Qi Zheng, Piotr Sobieszczyk, Usha B Tedrow, William G Stevenson, Roy M John
PURPOSE: Cardiomyopathies frequently lead to conduction system disease and/or arrhythmias necessitating device therapy, catheter ablation, or both. Endomyocardial biopsy (EMB) is avoided with recent right ventricle (RV) lead implants and optimal timing is uncertain. We determined outcomes of EMB at the time of ablation or device implantation procedures. METHODS: We retrospectively analyzed patients undergoing EMB during their electrophysiological procedure between January 2014 and July 2016...
March 24, 2018: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/29559999/recurrent-syncope-a-clue-in-amyloid-cardiomyopathy
#8
Julian A Marin-Acevedo, Catalina Sanchez-Alvarez, Ali A Alsaad, Ricardo J Pagán
Infiltrative cardiomyopathies include a variety of disorders that lead to myocardial thickening resulting in a constellation of clinical manifestations and eventually heart failure that could be the first clue to reach the diagnosis. Among the more described infiltrative diseases of the heart is amyloid cardiomyopathy. The disease usually presents with subtle, nonspecific symptoms. Herein, we illustrate a case of recurrent syncope as the initial presenting symptom for systemic amyloid with polyneuropathy and cardiomyopathy as a cause of syncope...
2018: Case Reports in Medicine
https://www.readbyqxmd.com/read/29544239/-transthyretin-familial-amyloid-polyneuropathy-disease-profile-of-a-multisystem-disorder
#9
Christoph Niemietz, Christoph Röcken, Matthias Schilling, Jörg Stypmann, Constantin E Uhlig, Hartmut H-J Schmidt
Transthyretin-related Familial Amyloid Polyneuropathy (ATTR Amyloidosis, former FAP, here called TTR-FAP) is a rare, progressive autosomal dominant inherited amyloid disease ending fatal within 5 - 15 years after final diagnosis. TTR-FAP is caused by mutations of transthyretin (TTR), which forms amyloid fibrils affecting peripheral and autonomic nerves, the heart and other organs. Due to the phenotypic heterogeneity and partly not specific enough clinical symptoms, diagnosis of TTR-FAP can be complicated...
March 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29524169/clinical-use-of-cardiac-pet-mri-current-state-of-the-art-and-potential-future-applications
#10
REVIEW
Patrick Krumm, Stefanie Mangold, Sergios Gatidis, Konstantin Nikolaou, Felix Nensa, Fabian Bamberg, Christian la Fougère
Combined PET/MRI is a novel imaging method integrating the advances of functional and morphological MR imaging with PET applications that include assessment of myocardial viability, perfusion, metabolism of inflammatory tissue and tumors, as well as amyloid deposition imaging. As such, PET/MRI is a promising tool to detect and characterize ischemic and non-ischemic cardiomyopathies. To date, the greatest benefit may be expected for diagnostic evaluation of systemic diseases and cardiac masses that remain unclear in cardiac MRI, as well as for clinical and scientific studies in the setting of ischemic cardiomyopathies...
May 2018: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/29448998/cardiac-amyloidosis-from-clinical-suspicion-to-morphological-diagnosis
#11
REVIEW
Pavla Flodrova, Patrik Flodr, Tomas Pika, Jiri Vymetal, Dusan Holub, Petr Dzubak, Marian Hajduch, Vlastimil Scudla
Amyloidosis is a heterogeneous group of diseases characterised by extracellular accumulation of amyloid in various tissues and organs of the body, leading to alteration and destruction of tissues. Heart involvement is the most important prognostic factor in patients with systemic amyloidosis and the diagnosis and typing of amyloid must be made properly. The clinical picture shows congestive heart failure with predominant right-sided heart failure symptoms in fully developed disease, various types of arrhythmias and characteristic electrocardiography and echocardiography findings...
April 2018: Pathology
https://www.readbyqxmd.com/read/29394116/normal-scores-of-deep-breathing-tests-beware-of-dysrhythmia-in-transthyretin-amyloidosis
#12
Urban Wiklund, Amir Kadkhodaee, Kennet Andersson, Ole B Suhr, Rolf Hörnsten
BACKGROUND: The heart rate (HR) response to paced deep breathing (DB) is a common test of cardiac autonomic function, where high heart rate variability (HRV) is considered to reflect normal autonomic function. We evaluated the DB test in patients with hereditary transthyretin amyloid (ATTRm) amyloidosis, where autonomic dysregulation and atrial arrhythmias are common. METHODS: Paced DB was performed during one minute (six breaths/min) in 165 recordings in adult ATTRm amyloidosis patients with the TTR Val30Met mutation, 42 hypertrophic cardiomyopathy (HCM) patients and 211 healthy subjects...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29259858/-in-vivo-quantification-of-amyloid-burden-in-ttr-related-cardiac-amyloidosis
#13
Alexander Marco Kollikowski, Florian Kahles, Svetlana Kintsler, Sandra Hamada, Sebastian Reith, Ruth Knüchel, Christoph Röcken, Felix Manuel Mottaghy, Nikolaus Marx, Mathias Burgmaier
Cardiac transthyretin-related (ATTR) amyloidosis is a severe cardiomyopathy for which therapeutic approaches are currently under development. Because non-invasive imaging techniques such as cardiac magnetic resonance imaging and echocardiography are non-specific, the diagnosis of ATTR amyloidosis is still based on myocardial biopsy. Thus, diagnosis of ATTR amyloidosis is difficult in patients refusing myocardial biopsy. Furthermore, myocardial biopsy does not allow 3D-mapping and quantification of myocardial ATTR amyloid...
November 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29249054/transthyretin-familial-amyloid-polyneuropathy-an-update
#14
Violaine Plante-Bordeneuve
Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a progressive, fatal, inherited disorder first identified in Portugal and now recognized in all continents. Over the past decade, thanks to the availability of the genetic test, our knowledge on the range of clinical expressions of this disorder has expanded, including different patterns and progression rates of the neuropathy, as well as aspects of the cardiomyopathy, which can be prominent. In the mean time, new tools are being developed to detect earlier TTR amyloid deposition such as cardiac scintigraphy with technetium-labelled pyrophosphate tracers or small nerve fiber alterations from skin biopsies, or using neurophysiological approaches as well as magnetic resonance neurography (MRN)...
April 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29246775/coronary-ectasia-in-amyloid-cardiomyopathy-and-neuropathy-due-to-the-transthyretin-mutation-c-323a-g
#15
Josef Finsterer, Claudia Stöllberger, Helmut Rauschka, Edmund Gatterer
BACKGROUND: atrial fibrillation(AF) is a frequent manifestation of cardiac involvement in genetic and wild-type transthyretin-related familial amyloidosis(TTR-FA). However, ectasia of coronary arteries and ablation for AF have not been reported in TTR-FA. METHODS AND RESULTS: A 65yo male developed progressive sensori-motor polyneuropathy since age 59y. At age 60y bifascicular block and myocardial thickening were recognised. At age 62y heart failure developed and work-up with cardiac MRI suggested amyloidosis but biopsy was non-informative...
March 2018: Heart & Lung: the Journal of Critical Care
https://www.readbyqxmd.com/read/29218105/systemic-inflammation-is-associated-with-myocardial-fibrosis-diastolic-dysfunction-and-cardiac-hypertrophy-in-patients-with-hypertrophic-cardiomyopathy
#16
Lu Fang, Andris H Ellims, Anna L Beale, Andrew J Taylor, Andrew Murphy, Anthony M Dart
BACKGROUND: Regional or diffuse fibrosis is an early feature of hypertrophic cardiomyopathy (HCM) and is related to poor prognosis. Previous studies have documented low-grade inflammation in HCM. The aim of this study was to examine the relationships between circulating inflammatory markers and myocardial fibrosis, systolic and diastolic dysfunction, and the degree of cardiac hypertrophy in HCM patients. METHODS AND RESULTS: Fifty HCM patients were recruited while 20 healthy subjects served as the control group...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/29217108/improved-outcomes-after-heart-transplantation-for-cardiac-amyloidosis-in-the-modern-era
#17
Arnt V Kristen, Michael M Kreusser, Patrick Blum, Stefan O Schönland, Lutz Frankenstein, Andreas O Dösch, Benjamin Knop, Matthias Helmschrott, Bastian Schmack, Arjang Ruhparwar, Ute Hegenbart, Hugo A Katus, Philip W J Raake
BACKGROUND: Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTR amyloidosis, and changed chemotherapy regimens for AL amyloidosis...
May 2018: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29142812/left-ventricle-relative-apical-sparing-in-cardiac-amyloidosis
#18
Marianna Nardozza, Elisabetta Chiodi, Donato Mele
Amyloidosis is a disease characterized by the extracellular deposition of the protein amyloid. It is a multiorgan disease, and cardiac involvement is not uncommon, generally in the form of a restrictive cardiomyopathy. Typical aspects of cardiac amyloidosis have been described at echocardiography and magnetic resonance imaging (MRI). In particular, the relative apical sparing at two-dimensional speckle-tracking echocardiography has been reported to be specific for cardiac amyloidosis. In our case, we report for the first time that this echocardiographic sign is related to lack of hyperenhancement at late gadolinium enhancement imaging in cardiac MRI...
October 2017: Journal of Cardiovascular Echography
https://www.readbyqxmd.com/read/29142197/proteotoxicity-in-cardiac-amyloidosis-amyloidogenic-light-chains-affect-the-levels-of-intracellular-proteins-in-human-heart-cells
#19
Esther Imperlini, Massimiliano Gnecchi, Paola Rognoni, Eduard Sabidò, Maria Chiara Ciuffreda, Giovanni Palladini, Guadalupe Espadas, Francesco Mattia Mancuso, Margherita Bozzola, Giuseppe Malpasso, Veronica Valentini, Giuseppina Palladini, Stefania Orrù, Giovanni Ferraro, Paolo Milani, Stefano Perlini, Francesco Salvatore, Giampaolo Merlini, Francesca Lavatelli
AL amyloidosis is characterized by widespread deposition of immunoglobulin light chains (LCs) as amyloid fibrils. Cardiac involvement is frequent and leads to life-threatening cardiomyopathy. Besides the tissue alteration caused by fibrils, clinical and experimental evidence indicates that cardiac damage is also caused by proteotoxicity of prefibrillar amyloidogenic species. As in other amyloidoses, the damage mechanisms at cellular level are complex and largely undefined. We have characterized the molecular changes in primary human cardiac fibroblasts (hCFs) exposed in vitro to soluble amyloidogenic cardiotoxic LCs from AL cardiomyopathy patients...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29119082/cardiac-manifestations-of-familial-mediterranean-fever
#20
REVIEW
Ahmad Alsarah, Osama Alsara, Heather S Laird-Fick
Familial Mediterranean fever (FMF) is autoinflammatory disorder characterized by sporadic attacks of fever, peritonitis, pleuritis, and arthritis. It is mainly seen in patients from Mediterranean origins, but it is now reported more frequently in Europe and North America due to immigration. To analyze the data on the cardiovascular manifestations in FMF patients, we searched PubMed using the terms "Familial Mediterranean Fever" or "FMF" in combination with other key words including "cardiovascular diseases" "pericardial diseases" "atherosclerosis" "coronary artery diseases" "cardiomyopathy" "pulmonary hypertension" or "valvular diseases...
October 2017: Avicenna Journal of Medicine
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