Cardiac amyloid

Michelle M Kittleson, Mathew S Maurer, Amrut V Ambardekar, Renee P Bullock-Palmer, Patricia P Chang, Howard J Eisen, Ajith P Nair, Jose Nativi-Nicolau, Frederick L Ruberg
Transthyretin amyloid cardiomyopathy (ATTR-CM) results in a restrictive cardiomyopathy caused by extracellular deposition of transthyretin, normally involved in the transportation of the hormone thyroxine and retinol-binding protein, in the myocardium. Enthusiasm about ATTR-CM has grown as a result of 3 simultaneous areas of advancement: Imaging techniques allow accurate noninvasive diagnosis of ATTR-CM without the need for confirmatory endomyocardial biopsies; observational studies indicate that the diagnosis of ATTR-CM may be underrecognized in a significant proportion of patients with heart failure; and on the basis of elucidation of the mechanisms of amyloid formation, therapies are now approved for treatment of ATTR-CM...
June 1, 2020: Circulation
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
Roberta Fenoglio, Simone Baldovino, Michela Ferro, Savino Sciascia, Gianluca Rabajoli, Giacomo Quattrocchio, Giulietta Beltrame, Carla Naretto, Daniela Rossi, Mirella Alpa, Antonella Barreca, Mario Giulio Papotti, Dario Roccatello
INTRODUCTION: AL amyloidosis is caused by a clone of plasma cell. Due to the impact of the disease on patient survival, careful evaluation of organ involvement is essential and treatment should be tailored to single patient's risk. AIM: We analyzed the clinical, laboratory and histological characteristics of 21 elderly patients (pts) (mean age 74.7 ± 7.97 years, range 55-81) with AL amyloidosis, including 17 patients (81%) with biopsy-proven renal involvement, who were ineligible for bone marrow transplantation, and evaluated the impact of renal impairment on survival...
May 29, 2020: Journal of Nephrology
P J Altshuler, M Helmers, A Iyengar, J Han, A Rios-Diaz, P Atluri
PURPOSE: Advances in amyloidosis management have improved patient longevity. Coupling potential increased transplantation for amyloid patients with graft scarcity, we intend to assess the use of extended-donor criteria (EDC) hearts for amyloidosis. METHODS: Retrospective review of heart transplants from the national Organ Procurement and Transplantation Network database from 1/'08 to 6/'19. Recipients were stratified first by amyloidosis diagnosis and subsequently by use of EDC grafts...
April 2020: Journal of Heart and Lung Transplantation
V K Randhawa, A Gabrovsek, E G Soltesz, M Z Tong, S Unai, L Chen, M M Mountis, J D Estep, W Tang, M Hanna
PURPOSE: Limited data exists on the use continuous-flow left ventricular assist device (CF-LVAD) in patients with cardiac amyloidosis (CA). The largest single center analysis on CF-LVAD support in patients with CA reports a 50% mortality at 6-months. Our primary aim was to examine the survival and morbidity of our patients with diagnosed CA and supported by CF-LVAD. METHODS: Data on demographics, type of cardiac amyloidosis, pre-implant echocardiographic features, and post-implant survival and morbidity outcomes was retrospectively extracted on patients with CA and supported by CF-LVAD at our institution from January 2009-October 2019...
April 2020: Journal of Heart and Lung Transplantation
B Di Giovanni, D Gustafson, M B Adamson, K Runeckles, D H Delgado
PURPOSE: Amyloid transthyretin (ATTR) cardiac amyloidosis (CA) is an increasingly recognized cause of restrictive cardiomyopathy and associated heart failure with preserved ejection fraction (HFpEF). Despite improved diagnostic techniques to identify this condition, many patients experience delayed diagnosis. Interpretation of routine cardiac biomarkers (i.e. troponins and brain natriuretic peptide (BNP)) and echocardiography in ATTR-CA is limited, particularly in relation to pyrophosphate (PYP) scintigraphy...
April 2020: Journal of Heart and Lung Transplantation
R Araujo-Gutierrez, A Bhimaraj, A Guha, I Hussain, M Kassi, J H Kim, B Trachtenberg
PURPOSE: After initial efforts at heart transplantation (HT) in patients with light chain amyloidosis were fraught with poor outcomes, emerging experience with modern chemotherapy regimens has led to a major improvement in outcomes in select centers. Given the systemic nature of the disease and the added immunosuppression in the form of chemotherapy, this study sought to analyze if AL patients undergoing HT had a higher burden of readmissions and infections non-amyloid heart transplant patients...
April 2020: Journal of Heart and Lung Transplantation
J P Li, P A Kingsford, G S Liu, J Nattiv, P A Genyk, A Wolfson, D Vucicevic, P Banankhah, A Salimbangon, M Fong, K Pandya, L Grazette, E C DePasquale, A S Vaidya
PURPOSE: Advanced amyloid cardiomyopathy (ACM) patients have high waitlist (WL) mortality. Given the greater emphasis and clarity for status exceptions for ACM patients in the new allocation system, we sought to assess whether this change in the allocation policy would affect the WL and post-transplant outcomes in ACM pts. METHODS: Thirty-five patients were identified in the UNOS database that underwent heart transplant (HT) with a prior diagnosis of ACM during a 6-month period immediately before and after the policy change...
April 2020: Journal of Heart and Lung Transplantation
J Lukac, S Syrjälä, M Saraswat, S Joenväärä, E Holmström, K Dhaygude, R Krebs, A Nykänen, R Renkonen, K Lemström
PURPOSE: Cardiac allografts are subjected to ischemic preservation before transplantation, and subsequent ischemia-reperfusion injury (IRI) after the surgery. IRI increases the risk for acute rejection, and more detailed detection methods for early allograft injury and rejection are therefore warranted. Systemic molecular phenotyping by proteomics is a novel method for investigating the underlaying pathophysiology. Plasma protein expressions reflect the ongoing clinical state of the patients, therefore enabling real-time screening of biomarkers for allograft injury or treatment efficacy...
April 2020: Journal of Heart and Lung Transplantation
E Bobbio, E Bollano, S Esmaily, C Thomsen, F Noborn, C Sihlbom, E Vorontsov, G Dellgren, G Larson, A Oldfors, K Karason
PURPOSE: Medical treatment for cardiac amyloidosis (CA) is evolving rapidly. Heart transplantation can be a valid option when followed by transplantation of bone marrow or liver, dependent on the type and origin of the amyloid protein. Thus, accurate typing of amyloidosis has implications for treatment, prognosis, and genetic counseling. Although non-invasive diagnostic techniques can type CA, endomyocardial biopsy (EMB) may be needed in the case of equivocal imaging findings or discordant data...
April 2020: Journal of Heart and Lung Transplantation
G S Ma, Q M Bui, M Kraushaar, V Escobedo, B Le, E Covarrubias, M Brambatti, A Kahn, C Bougault, M Taylor, E Adler
PURPOSE: Danon Disease (DD) is a rare X-linked disorder due to mutations in the Lysosomal Associated Membrane Protein 2 (LAMP-2) gene. Patients present with cardiac manifestations of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). The goal of this study is to characterize the longitudinal progression of hypertrophic phenotypes and descriptions of left ventricular strain in DD. METHODS: We created an international registry including 44 adult and pediatric patients with DD...
April 2020: Journal of Heart and Lung Transplantation
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
Alessia Gimelli, Alberto Aimo, Giuseppe Vergaro, Dario Genovesi, Valeria Santonato, Annette Kusch, Michele Emdin, Paolo Marzullo
Background: Tissue accumulation of misfolded transthyretin (TTR) may occur because of TTR gene mutations (variant amyloid TTR amyloidosis, ATTRv), or as an age-related phenomenon (wild-type ATTR, ATTRwt). Cardiac sympathetic denervation has been reported in ATTRv, but has never been investigated in ATTRwt. Methods: Fifteen consecutive patients with ATTRwt cardiomyopathy (81% men, median age 82 years, no one with prior myocardial infarction) underwent Cadmium Zinc Telluride tomographic imaging for amyloid burden (99m Tc-hydroxymethylene diphosphonate - 99m Tc-HMDP), innervation (123 I-metaiodobenzylguanidine - 123 I-MIBG ), and perfusion (99m Tc-tetrofosmin)...
May 22, 2020: Amyloid: the International Journal of Experimental and Clinical Investigation
Cesia Gallegos, Edward J Miller
The gold standard for diagnosis of cardiac amyloidosis (CA) is endomyocardial biopsy showing Congo red staining followed by mass spectroscopy, but the diagnosis can also be made with high certainty by demonstration of typical cardiac imaging features along with amyloid on biopsy of another involved organ. The use of cardiac imaging techniques to detect amyloid deposits may frequently obviate the need for invasive methods in order to ascertain the presence, and potentially the type, of amyloid deposition. PURPOSE OF REVIEW: We aim to review the evidence behind the development of novel positron emission tomography (PET) radiotracers for demonstrating cardiac amyloid deposition and potentially distinguishing between light-chain (AL) or transthyretin (ATTR) cardiac amyloidosis...
May 19, 2020: Current Cardiology Reports
Wenguang Li, Alan Lazarus, Hao Gao, Ana Martinez-Naharro, Marianna Fontana, Philip Hawkins, Swethajit Biswas, Robert Janiczek, Jennifer Cox, Colin Berry, Dirk Husmeier, Xiaoyu Luo
Deposition of amyloid in the heart can lead to cardiac dilation and impair its pumping ability. This ultimately leads to heart failure with worsening symptoms of breathlessness and fatigue due to the progressive loss of elasticity of the myocardium. Biomarkers linked to the clinical deterioration can be crucial in developing effective treatments. However, to date the progression of cardiac amyloidosis is poorly characterized. There is an urgent need to identify key predictors for disease progression and cardiac tissue function...
2020: Frontiers in Physiology
Sarah A M Cuddy, Paco E Bravo, Rodney H Falk, Samir El-Sady, Marie Foley Kijewski, Mi-Ae Park, Frederick L Ruberg, Vaishali Sanchorawala, Heather Landau, Andrew J Yee, Giada Bianchi, Marcelo F Di Carli, Su-Chun Cheng, Michael Jerosch-Herold, Raymond Y Kwong, Ronglih Liao, Sharmila Dorbala
OBJECTIVES: The purpose of this study was to determine phenotypes characterizing cardiac involvement in AL amyloidosis by using direct (fluorine-18-labeled florbetapir {[18 F]florbetapir} positron emission tomography [PET]/computed tomography) and indirect (echocardiography and cardiac magnetic resonance [CMR]) imaging biomarkers of AL amyloidosis. BACKGROUND: Cardiac involvement in systemic light chain amyloidosis (AL) is the main determinant of prognosis and, therefore, guides management...
May 8, 2020: JACC. Cardiovascular Imaging
Wengen Chen, Vasken Dilsizian
No abstract text is available yet for this article.
May 8, 2020: JACC. Cardiovascular Imaging
Sara Rosengren, Tor Skibsted Clemmensen, Lars Tolbod, Sven-Olof Granstam, Hans Eiskjær, Gerhard Wikström, Ola Vedin, Tanja Kero, Mark Lubberink, Hendrik J Harms, Frank A Flachskampf, Tomasz Baron, Kristina Carlson, Fabian Mikkelsen, Gunnar Antoni, Niels Frost Andersen, Steen Hvitfeldt Poulsen, Jens Sörensen
OBJECTIVES: This dual-site study evaluated the diagnostic accuracy of the method. BACKGROUND: Pittsburgh compound ([11 C]PIB) positron emission tomography (PIB-PET) has shown promise as a specific and noninvasive method for the diagnosis of cardiac amyloidosis (CA). METHODS: The study had 2 parts. In the initial study, 51 subjects were included, 36 patients with known CA and increased wall thickness (15 immunoglobulin light chain [AL] and 21 transthyretin [ATTR] amyloidosis) and 15 control patients (7 were nonamyloid hypertrophic and 8 healthy volunteers)...
May 8, 2020: JACC. Cardiovascular Imaging
Zohya Khalique, Pedro F Ferreira, Andrew D Scott, Sonia Nielles-Vallespin, Ana Martinez-Naharro, Marianna Fontana, Phillip Hawkins, David N Firmin, Dudley J Pennell
Background Cardiac amyloidosis (CA) is a disease of interstitial myocardial infiltration, usually by light chains or transthyretin. We used diffusion tensor cardiovascular magnetic resonance (DT-CMR) to noninvasively assess the effects of amyloid infiltration on the cardiac microstructure. Methods DT-CMR was performed at diastole and systole in 20 CA, 11 hypertrophic cardiomyopathy, and 10 control subjects with calculation of mean diffusivity, fractional anisotropy, and sheetlet orientation (secondary eigenvector angle)...
May 2020: Circulation. Cardiovascular Imaging
Osnat Itzhaki Ben Zadok, Ran Kornowski
Cardiac amyloidosis, the majority of cases of which are due to immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR), affects different aspects of the heart and cardiovascular system. Amyloid-induced cardiomyopathy, clinically manifesting with heart failure and electrophysiological abnormalities, has distinct characteristics compared to non-amyloid cardiomyopathies. Accordingly, specific management strategies are required. This paper will review the cardiovascular manifestations of patients with cardiac amyloidosis and their suggested treatment strategies, emphasizing the importance of multidisciplinary care...
May 14, 2020: Acta Haematologica
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