keyword
MENU ▼
Read by QxMD icon Read
search

Cardiac amyloidosis

keyword
https://www.readbyqxmd.com/read/29217108/improved-outcomes-after-heart-transplantation-for-cardiac-amyloidosis-in-the-modern-era
#1
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...
November 15, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29212969/-analysis-of-long-term-survivors-with-cardiac-al-amyloidosis
#2
Kumiko Kagawa, Yusaku Maeda, Masahiro Oura, Kimiko Sogabe, Hikaru Fujino, Mamiko Takahashi, Tomoko Maruhashi, Masami Iwasa, Kengo Udaka, Takeshi Harada, Takayuki Ise, Shiro Fujii, Shingen Nakamura, Hirokazu Miki, Shusuke Yagi, Kyoko Takeuchi, Shuji Ozaki, Masahiro Abe
Cardiac AL amyloidosis (CA) is generally known as a severe disease with very poor prognosis. Here we retrospectively examined seven patients with CA in our cohort who achieved long-term survival. All six patients who underwent high-dose melphalan and autologous stem cell transplantation (ASCT) survived for >3 years, whereas four patients survived for >5 years. Patients who underwent ASCT had prompt hematological responses, and five patients showed organ responses. ASCT helps to achieve a quick and deep hematological response required for long-term survival in patients with CA...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29212513/cardiac-amyloidosis-is-prevalent-in-older-patients-with-aortic-stenosis-and-carries-worse-prognosis
#3
João L Cavalcante, Shasank Rijal, Islam Abdelkarim, Andrew D Althouse, Michael S Sharbaugh, Yaron Fridman, Prem Soman, Daniel E Forman, John T Schindler, Thomas G Gleason, Joon S Lee, Erik B Schelbert
BACKGROUND: Non-invasive cardiac imaging allows detection of cardiac amyloidosis (CA) in patients with aortic stenosis (AS). Our objective was to estimate the prevalence of clinically suspected CA in patients with moderate and severe AS referred for cardiovascular magnetic resonance (CMR) in age and gender categories, and assess associations between AS-CA and all-cause mortality. METHODS: We retrospectively identified consecutive AS patients defined by echocardiography referred for further CMR assessment of valvular, myocardial, and aortic disease...
December 7, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29210840/conoscere-e-curare-il-cuore-1986-2016-how-the-heart-has-changed
#4
Alessandro Boccanelli
: The demographic transition, with longer life expectancy, is mainly due to prevention and care of atherosclerotic vascular and heart disease. The cardiovascular patient is now much older and often presents with coexisting geriatric syndromes that affect healthcare goals, outcomes, and the process of care. Numerous mechanisms at cellular level are responsible for cardiovascular aging such as replicative senescence, apoptosis, proteins misfolding, and inflammation. Aging-related modifications are increase in left-ventricular mass, increased left atrial size, myocardial collagen deposition, and calcium deposition in valvular structures and the coronary arteries...
November 27, 2017: Journal of Cardiovascular Medicine
https://www.readbyqxmd.com/read/29202938/prevalence-of-monoclonal-gammopathy-in-wild-type-transthyretin-amyloidosis
#5
Hallie I Geller, Avinainder Singh, Tara M Mirto, Robert Padera, Richard Mitchell, Jacob P Laubach, Rodney H Falk
OBJECTIVE: To evaluate the prevalence of monoclonal gammopathy (MG) in patients with wild-type transthyretin amyloidosis (ATTRwt) (formerly known as senile amyloidosis). PATIENTS AND METHODS: We retrospectively analyzed the serum protein electrophoresis and serum immunofixation results, free light chain (FLC) levels, and renal function of 113 consecutive patients with ATTRwt seen at the Brigham and Women's Hospital's Cardiac Amyloidosis Program between February 21, 2006, and November 9, 2016...
December 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29199249/utility-of-single-photon-emission-computed-tomography-computed-tomography-fusion-imaging-with-99-mtc-pyrophosphate-scintigraphy-in-the-assessment-of-cardiac-transthyretin-amyloidosis
#6
Noriko Tsuda, Shinya Shiraishi, Seitaro Oda, Koji Ogasawara, Fumi Sakamoto, Seiji Tomiguchi, Koichiro Fujisue, Seiji Takashio, Yasuhiro Izumiya, Kenichi Tsujita, Yukio Ando, Yasuyuki Yamashita
No abstract text is available yet for this article.
December 2, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29197924/echocardiography-as-the-first-diagnostic-clue-to-rapidly-progressive-systemic-al-amyloidosis-associated-with-multiple-site-thrombosis-a-case-report
#7
Bogdan Caloian, Dumitru Zdrenghea, Sorin Claudiu Man, Simona Costea, Mihnea Zdrenghea, Dana Pop
AL-amyloidosis is a rare, but complex disease, with a severe prognosis, cardiac involvement being found in half of the patients. The rapid increase of the LV wall thickness predicts an unfavorable evolution. We report the case of a 63-year-old man diagnosed with AL-amyloidosis, with cardiac involvement, associated with multiple site thrombosis.  Specific echocardiographic methods like tissue Doppler imaging and speckle tracking provided crucial diagnostic and prognostic information.
November 29, 2017: Medical Ultrasonography
https://www.readbyqxmd.com/read/29196671/concurrent-structural-and-biophysical-traits-link-with-immunoglobulin-light-chains-amyloid-propensity
#8
Luca Oberti, Paola Rognoni, Alberto Barbiroli, Francesca Lavatelli, Rosaria Russo, Martina Maritan, Giovanni Palladini, Martino Bolognesi, Giampaolo Merlini, Stefano Ricagno
Light chain amyloidosis (AL), the most common systemic amyloidosis, is caused by the overproduction and the aggregation of monoclonal immunoglobulin light chains (LC) in target organs. Due to genetic rearrangement and somatic hypermutation, virtually, each AL patient presents a different amyloidogenic LC. Because of such complexity, the fine molecular determinants of LC aggregation propensity and proteotoxicity are, to date, unclear; significantly, their decoding requires investigating large sets of cases. Aiming to achieve generalizable observations, we systematically characterised a pool of thirteen sequence-diverse full length LCs...
December 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29182024/first-nationwide-survey-on-systemic-wild-type-attr-amyloidosis-in-japan
#9
Yoshiki Sekijima, Masahide Yazaki, Mitsuharu Ueda, Haruki Koike, Masahito Yamada, Yukio Ando
OBJECTIVE: A nationwide survey on systemic wild-type ATTR (ATTRwt) amyloidosis was conducted to elucidate the frequency, clinical picture and possible diagnostic issues of ATTRwt amyloidosis in Japan. METHODS: A questionnaire was sent to 4629 clinical departments across Japan. A total of 2341 (50.6%) responses were returned completed for further analysis. RESULTS: Fifty-one patients with ATTRwt amyloidosis (82% male) were identified between January 2012 and December 2014...
November 28, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29174954/cell-milieu-significantly-affects-the-fate-of-aapoai-amyloidogenic-variants-predestination-or-serendipity
#10
Rosa Gaglione, Giovanni Smaldone, Rocco Di Girolamo, Renata Piccoli, Emilia Pedone, Angela Arciello
BACKGROUND: Specific apolipoprotein A-I variants are associated to severe hereditary amyloidoses. The organ distribution of AApoAI amyloidosis seems to depend on the position of the mutation, since mutations in residues from 1 to 75 are mainly associated to hepatic and renal amyloidosis, while mutations in residues from 173 to 178 are mostly responsible for cardiac, laryngeal, and cutaneous amyloidosis. Molecular bases of this tissue specificity are still poorly understood, but it is increasingly emerging that protein destabilization induced by amyloidogenic mutations is neither necessary nor sufficient for amyloidosis development...
November 22, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29173805/cardiac-amyloidosis-an-updated-review-with-emphasis-on-diagnosis-and-future-directions
#11
Sukhdeep Bhogal, Vatsal Ladia, Puja Sitwala, Emilie Cook, Kailash Bajaj, Vijay Ramu, Carl J Lavie, Timir K Paul
Cardiac amyloidosis occurs because of abnormal protein (amyloid) deposition in the cardiac tissue. Even with advanced diagnostic techniques and treatments, the prognosis of amyloidosis remains poor. The diagnosis of cardiac amyloidosis particularly needs to be in the differential in patients presenting with heart failure with preserved ejection fraction. This entity remains underdiagnosed due to lack of suspicion on the part of many clinicians. Involvement of cardiac tissue is the utmost determinant factor for available treatment options and prognosis...
January 2018: Current Problems in Cardiology
https://www.readbyqxmd.com/read/29166521/case-for-diagnosis-systemic-light-chain-amyloidosis-with-cutaneous-involvement
#12
João Renato Vianna Gontijo, Jackson Machado Pinto, Maysa Carla de Paula
Systemic light chain amiloydosis is a rare disease. Due to its typical cutaneous lesions, dermatologists play an essential role in its diagnosis. Clinical manifestations vary according to the affected organ and are often unspecific. Definitive diagnosis is achieved through biopsy. We report a patient with palpebral amyloidosis, typical bilateral ecchymoses and cardiac involvement, without plasma cell dyscrasia or lymphomas. The patient died shortly after the diagnosis.
September 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29164000/late-ventricular-potentials-in-familial-mediterranean-fever-with-and-without-aa-amyloidosis
#13
Udi Nussinovitch, Avi Livneh
Objective: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by episodic and chronic inflammation that may lead to both accelerated coronary atherosclerosis and cardiac AA amyloidosis. We hypothesized that late ventricular potentials (LPs), an established electrocardiographic susceptibility marker of ventricular arrhythmias, will be more common in FMF than in the adjusted normal population due to these two types of inflammation-associated cardiac effects...
September 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/29159946/extracellular-volume-with-bolus-only-technique-in-amyloidosis-patients-diagnostic-accuracy-correlation-with-other-clinical-cardiac-measures-and-ability-to-track-changes-in-amyloid-load-over-time
#14
Giulia Zumbo, Sharon V Barton, Douglas Thompson, Min Sun, Amna Abdel-Gadir, Thomas A Treibel, Daniel Knight, Ana Martinez-Naharro, Lane Thirusha, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Extracellular volume (ECV) by T1 mapping requires the contrast agent distribution to be at equilibrium. This can be achieved either definitively with a primed contrast infusion (infusion ECV), or sufficiently with a delay postbolus (bolus-only ECV). For large ECV, the bolus-only approach measures higher than the infusion ECV, causing some uncertainty in diseases such as amyloidosis. PURPOSE: To characterize the relationship between the bolus-only and current gold-standard infusion ECV in patients with amyloidosis...
November 21, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29153741/left-atrial-function-in-patients-with-light-chain-amyloidosis-a-transthoracic-3d-speckle-tracking-imaging-study
#15
Dania Mohty, Vincent Petitalot, Julien Magne, Bahaa M Fadel, Cyrille Boulogne, Dounia Rouabhia, Chahrazed ElHamel, David Lavergne, Thibaud Damy, Victor Aboyans, Arnaud Jaccard
BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography...
November 16, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/29153245/frequency-of-and-prognostic-significance-of-cardiac-involvement-at-presentation-in-hereditary-transthyretin-derived-amyloidosis-and-the-value-of-n-terminal-pro-b-type-natriuretic-peptide
#16
Sebastiaan H C Klaassen, Jasper Tromp, Hans L A Nienhuis, Peter van der Meer, Maarten P van den Berg, Hans Blokzijl, Dirk J van Veldhuisen, Bouke P C Hazenberg
The aim of this study is to assess the prevalence of cardiac involvement in hereditary transthyretin-derived (ATTRm) amyloidosis at the time of diagnosis and to determine the diagnostic and clinical value of N-terminal pro-B-type natriuretic peptide (NT-proBNP). The University Medical Center Groningen is the national center of expertise for amyloidosis. All consecutive patients between 1994 and 2016 with ATTRm amyloidosis were followed prospectively. Baseline was set at the time of the first positive biopsy...
October 14, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/29148151/inflammation-in-cardiac-amyloidosis-prognostic-marker-or-therapeutic-target
#17
EDITORIAL
Gianfranco Sinagra, Enrico Fabris
No abstract text is available yet for this article.
November 16, 2017: European Journal of Heart Failure
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/29141071/usefulness-of-total-12-lead-qrs-voltage-as-a-clue-to-diagnosis-of-patients-with-cardiac-sarcoidosis-severe-enough-to-warrant-orthotopic-heart-transplant
#20
William C Roberts, Tiffany M Becker, Shelley A Hall
Importance: Severe heart failure caused by cardiac sarcoidosis is difficult to diagnosis without biopsy. Objective: To assess whether total electrocardiographic 12-lead QRS voltage may be a clue to diagnosis. Design, Setting, Participants: Case-series study with cases collected at Baylor University Medical Center at Dallas, Dallas, Texas, from January 13, 2005, to January 24, 2017. The clinical records of 16 patients with severe heart failure caused by cardiac sarcoidosis were studied...
November 15, 2017: JAMA Cardiology
keyword
keyword
3056
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"