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Cardiac amyloidosis

Laroussi Mohamed-Salem, Juan José Santos-Mateo, Juan Sanchez-Serna, Álvaro Hernández-Vicente, Rafael Reyes-Marle, María I Castellón Sánchez, María Antonia Claver-Valderas, Emiliano Gonzalez-Vioque, Francisco J Haro-Del Moral, Pablo García-Pavía, Domingo A Pascual-Figal
BACKGROUND: Myocardial uptake of bone tracers has emerged as useful tool for the early detection of transthyretin amyloidosis (ATTR). The prevalence of wild-type ATTR (ATTRwt) in individuals remains to be established. METHODS: All whole body bone scans performed in individuals ≥ 75 years with no previous clinical suspicion of ATTR were revised in a population-based university hospital over a 7-year period (1509 studies corresponding to 1114 patients; 80...
June 6, 2018: International Journal of Cardiology
Antonio Vitarelli, Maria Teresa Petrucci, Silvia Lai, Carlo Gaudio, Lidia Capotosto, Enrico Mangieri, Serafino Ricci, Simone De Sio, Giovanni Truscelli, Federico Vozella, Mario Sergio Pergolini
The dataset presented in this article is related to the research article entitled "Biventricular assessment of light-chain amyloidosis using 3D speckle tracking echocardiography: Differentiation from other forms of myocardial hypertrophy" (Vitarelli et al., 2018) [1], which examined the potential utility of left ventricular (LV) and right ventricular (RV) deformation and rotational parameters derived from three-dimensional speckle-tracking echocardiography (3DSTE) to diagnose cardiac amyloidosis(CA) and differentiate this disease from other forms of myocardial hypertrophy...
June 2018: Data in Brief
Francesco Cappelli, Raffaele Martone, Giulia Taborchi, Sofia Morini, Simone Bartolini, Paola Angelotti, Silvia Farsetti, Carlo Di Mario, Federico Perfetto
Transthyretin-related (ATTR) cardiac amyloidosis is currently lacking a disease-modifying therapy. Despite demonstration of effectiveness in halting amyloid deposition, no study focused on epigallocatechin-3-gallate (EGCG) impact on patient survival. We sought to explore prognostic impact of EGCG in a cohort of lone cardiac ATTR patients. From the Florence Tuscan Regional Amyloid Centre database, we retrospectively selected ATTR patients treated with EGCG (675mg daily dose) for a minimum of 9 months, between March 2013 and December 2016...
June 7, 2018: Internal and Emergency Medicine
Sarah Pradel, Stéphanie Brun, Gérard Victor, Pierre Pascal, Pauline Fournier, David Ribes, Yoan Lavie-Badie, Michel Galinier, Didier Carrié, Isabelle Berry, Olivier Lairez
AIMS: The purpose of the study was to describe the pattern of 99m Tc-labeled phosphate agents myocardial uptake by scintigraphy and explore its impact on left ventricular (LV) functions in transthyretin cardiac amyloidosis (TTR-CA). METHODS: Fifty patients with TTR-CA underwent 99m Tc- hydroxymethylene-diphosphonate (99m Tc-HMDP) scintigraphy and echocardiography with measure of LV thickness, longitudinal strain (LS), systolic and diastolic functions. Cardiac retention by scintigraphy was assessed by visual scoring and the heart/whole body (H/WB) ratio was calculated by dividing counts in the heart by counts in late whole-body images...
June 7, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Miwa Ishida, Yoko Yamada, Tomohiro Mizutani, Atsuko Hara, Yoshiki Sekijima, Junya Ako, Takayuki Inomata
No abstract text is available yet for this article.
June 6, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
Ryan D Clay, James P Utz
No abstract text is available yet for this article.
June 2018: Mayo Clinic Proceedings
A Rancati, S Andreola, P Bailo, M Boracchi, P Fociani, G Gentile, R Zoja
Congo Red staining is usually used in diagnosing amyloidosis, a pathology characterized by the storage of abnormal proteins in several human organs. When assessed on samples fixated in formalin and embended in paraffin, this staining can undergo several artefacts, causing diagnostic and interpretative difficulties due to its weak stainability and a consequent reduced visibility of the amyloid. These complications, in time, requested several variations of this staining technique, especially in clinical practice, while in the forensic field no protocols has ever been adapted to cadaveric samples, a material that is already characteristically burdened by a peculiar stainability...
May 26, 2018: Forensic Science International
Andrew J Einstein, Igor Shuryak, Adam Castaño, Akiva Mintz, Mathew S Maurer, Sabahat Bokhari
BACKGROUND: Increasing recognition that transthyretin cardiac amyloidosis (ATTR-CA) is much more common than previously appreciated and the emergence of novel disease-modifying therapeutic agents have led to a paradigm shift in which ATTR-CA screening is considered in high-risk populations, such as patients with heart failure with preserved ejection fraction (HFpEF) or aortic stenosis. Radiation risk from 99m Tc-pyrophosphate (99m Tc-PYP) scintigraphy, a test with very high sensitivity and specificity for ATTR-CA, has not been previously determined...
May 30, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Pengguo Chen, Zhaohan Wang, Hui Liu, Donglin Liu, Zhibin Gong, Jin Qi, Jianfang Hu
Systemic amyloidosis is a rare disease involving multiple organs. It is difficult to establish diagnosis as the symptoms is diverse and non-specific. And without specific therapy the prognosis is very poor. We analyzed detailed clinical and laboratorial data of a 53-year-old male patient. The characteristic features included refractory pleural effusion, extraordinary hepatomegaly and cardiac failure. The illness lasted 9 months and therapy period spanned 4 months. Fine needle biopsy of liver, lung, heart, pancreas and kidney was performed...
May 11, 2018: Oncotarget
Bo Da Nam, Sung Mok Kim, Hye Na Jung, Yiseul Kim, Yeon Hyeon Choe
To compare inversion time (TI) parameters, native T1, and extracellular volume (ECV) on cardiac magnetic resonance (CMR) imaging between patients with cardiac amyloidosis (CA) or hypertrophic cardiomyopathy (HCMP). Forty six patients with biopsy-confirmed CA and 30 patients with HCMP who underwent CMR were included. T1 and TI values were measured in the septum and cavity of the left ventricle on T1 mapping and TI scout images. TI values were selected at nulling point for each myocardium and blood pool. Native T1, ECV, and TI interval values were significantly different between the CA (1170...
May 30, 2018: International Journal of Cardiovascular Imaging
Francisco Gual-Capllonch, Albert Teis, Elena Ferrer, Antoni Bayes-Genis
Doppler echocardiogram provides not only structural information about heart chambers, valves and pericardium but also contributes to functional assessment. A unique feature of this assessment is its variability, not only due to heart disease course but also related to extracardiac conditions. We present a case of primary amyloidosis with two atypical characteristics: left ventricular outflow obstruction and low-grade diastolic dysfunction. Nevertheless, a more typical picture of cardiac amyloidosis was observed in the evolution of the disease coinciding with a second cardiac stressor, emphasizing the necessity of not focusing our attention exclusively to the heart...
May 25, 2018: Echocardiography
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
Ketty Savino, Giuseppe Bagliani, Federico Crusco, Margherita Padeletti, Massimo Lombardi
Cardiovascular imaging has radically changed the management of patients with arrhythmogenic cardiomyopathies. This article focuses on the role of echocardiography and MRI in the diagnosis of these structural diseases. Cardiomyopathies with hypertrophic pattern (hypertrophic cardiomyopathy, restrictive cardiomyopathies, amyloidosis, Anderson-Fabry disease, and sarcoidosis), cardiomyopathies with dilated pattern, inflammatory cardiac diseases, and right ventricular arrhythmogenic cardiomyopathy are analyzed. Finally, anatomic predictors of arrhythmias and sudden cardiac death are discussed...
June 2018: Cardiac Electrophysiology Clinics
Steven J Promislow, Terrence D Ruddy
No abstract text is available yet for this article.
May 18, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
Marco Luciani, Luca Troncone, Federica Del Monte
Cardiac amyloidosis (CA) comprises a heterogeneous group of medical conditions affecting the myocardium. It presents with proteinaceous infiltration with variable degrees of severity, prevalence and evolution. Despite this heterogeneity, erroneous protein folding is the common pathophysiologic process, yielding the formation of a single misfolded protein (monomer) that progressively evolves and ultimately forms amyloid fibers. Additionally, by seeding out from the organs of origin, intermediates called oligomers metastasize and restart the process...
May 17, 2018: Acta Pharmacologica Sinica
Meidi El Issa, Malik El Issa, Besma Sidia
Amyloidosis is a rare disease, which can affect various organs, such as the kidneys, heart, liver, respiratory and gastrointestinal tracts, and the nervous system. It still has a bad prognosis nowadays, despite chemotherapy and the new biotherapies. Its physiopathology corresponds to an irreversible, extracellular accumulation of fibrillous proteins in the tissues. Notwithstanding the fact that a clear diagnosis can be made with histology (of solid injured organs or a subcutaneous biopsy), magnetic resonance imaging (MRI) can show various advantages, especially to prove cardiac involvement, with great specificity and sensibility as well...
March 15, 2018: Curēus
Tania Jain, Heidi E Kosiorek, Shu T Kung, Vishal S Shah, Amylou C Dueck, Veronica Gonzalez-Calle, Susan Luft, Craig B Reeder, Roberta Adams, Pierre Noel, Jeremy T Larsen, Joseph Mikhael, Leif Bergsagel, A Keith Stewart, Rafael Fonseca
BACKGROUND: The hematologic response is critical in patients with light chain amyloidosis because a good response is known to improve organ response and overall survival. We present a retrospective analysis to compare the hematologic and organ response in patients who received bortezomib-based therapy before autologous stem cell transplantation (ASCT) versus those who received non-bortezomib-based therapy before ASCT and those who underwent ASCT at diagnosis. PATIENTS AND METHODS: Of a total of 63 patients who underwent ASCT for light chain amyloidosis, 34 received bortezomib-based therapy before ASCT (Bor-ASCT) and 29 did not receive bortezomib therapy (non-Bor-ASCT)...
May 4, 2018: Clinical Lymphoma, Myeloma & Leukemia
Omar K Siddiqi, Vaishali Sanchorawala, Frederick L Ruberg
No abstract text is available yet for this article.
May 2018: Circulation. Cardiovascular Imaging
(no author information available yet)
BACKGROUND: Heart involvement is the most important prognostic determinant in AL amyloidosis patients. Echocardiography is a cornerstone for the diagnosis and provides important prognostic information. METHODS AND RESULTS: We studied 754 patients with AL amyloidosis who underwent echocardiographic assessment at the Mayo Clinic, including a Doppler-derived measurement of stroke volume (SV) within 30 days of their diagnosis to explore the prognostic role of echocardiographic variables in the context of a well-established soluble cardiac biomarker staging system...
May 2018: Circulation. Cardiovascular Imaging
Y F Wu, Y L Zhu, X W Yan
No abstract text is available yet for this article.
May 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
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