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Articles de revues sur le sujet « Cardiac amyloidosi »

Auteur : Grafiati

Publié le 9 mars 2023

Mis à jour le 31 juillet 2025

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1

Guan, Jian, Shikha Mishra, Rodney H. Falk, and Ronglih Liao. "Current perspectives on cardiac amyloidosis." American Journal of Physiology-Heart and Circulatory Physiology 302, no. 3 (2012): H544—H552. http://dx.doi.org/10.1152/ajpheart.00815.2011.

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Amyloidosis represents a group of diseases in which proteins undergo misfolding to form insoluble fibrils with subsequent tissue deposition. While almost all deposited amyloid fibers share a common nonbranched morphology, the affected end organs, clinical presentation, treatment strategies, and prognosis vary greatly among this group of diseases and are largely dependent on the specific amyloid precursor protein. To date, at least 27 precursor proteins have been identified to result in either local tissue or systemic amyloidosis, with nine of them manifesting in cardiac deposition and resultin

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Zattar Piazera, Flávia, Lucas Dornas Xavier, Natalye Wynona Rosário Cunha, Taís Nunes dos Santos, and Alex Francisco Antônio de Souza Damaceno. "The challenge of clinical diagnosis of cardiac amyloidosis: Case report and literature review." International Journal of Case Reports and Images 15, no. 2 (2024): 10–14. http://dx.doi.org/10.5348/101461z01fp2024cr.

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Introduction: Cardiac amyloidosis is a disease caused by the deposition and extracellular accumulation of defective proteins (amyloids) in the myocardium, which can lead to conduction disorders, diastolic dysfunction, and restriction to ventricular filling resulting in reduction of systolic volume and of cardiac output. Case Report: The patient, a 62-year-old male with a history of ischemic stroke for two months, was referred to the outpatient clinic for investigation of thrombophilia due to echocardiographic changes suggestive of cardiac amyloidosis. Conclusion: Cardiac amyloidosis is an unde

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Niemiec, Małgorzata, Magdalena Balwierz, Bartosz Gruchlik, and Katarzyna Mizia-Stec. "Cardiac amyloidosis." Annales Academiae Medicae Silesiensis 78 (June 25, 2024): 146–54. http://dx.doi.org/10.18794/aams/183982.

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Amyloidoza jest rzadką chorobą charakteryzującą się nieprawidłowym gromadzeniem się białka amyloidowego w tkankach. Amyloidozę można podzielić na dwa główne podtypy: amyloidozę transtyretynową (ATTR-CA) i amyloidozę łańcuchów lekkich immunoglobulin (AL-CA). Nagromadzenie białka amyloidu w mięśniu sercowym może doprowadzić do zaburzeń przewodzenia, kardiomiopatii restrykcyjnej i w konsekwencji niewydolności serca. Objawy mogą obejmować spadek tolerancji wysiłku, duszność, obrzęki oraz omdlenia. Rozpoznanie opiera się na badaniach laboratoryjnych, obrazowych oraz biopsji. Leczenie koncentruje si

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Musetti, Veronica, Francesco Greco, Vincenzo Castiglione, et al. "Tissue Characterization in Cardiac Amyloidosis." Biomedicines 10, no. 12 (2022): 3054. http://dx.doi.org/10.3390/biomedicines10123054.

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Cardiac amyloidosis (CA) has long been considered a rare disease, but recent advancements in diagnostic tools have led to a reconsideration of the epidemiology of CA. Amyloid light-chain (AL) and transthyretin (ATTR) amyloidoses are the most common forms of cardiac amyloidosis. Due to the distinct treatments and the different prognoses, amyloid typing is crucial. Although a non-biopsy diagnosis can be obtained in ATTR amyloidosis when certain diagnostic criteria are fulfilled, tissue characterization still represents the gold standard for the diagnosis and typing of CA, particularly in AL amyl

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Ablasser, Klemens, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, and Peter P. Rainer. "Unfolding Cardiac Amyloidosis –From Pathophysiology to Cure." Current Medicinal Chemistry 26, no. 16 (2019): 2865–78. http://dx.doi.org/10.2174/0929867325666180104153338.

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Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium causes cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. Thus, the reversal of arrest of adverse cardiac remodeling is the target of current therapies. Here, we provide a condensed overview on the pathophysiology of AL and ATTR cardiac amyloidoses and describe treatments that are cu

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de Barros Correia, Edileide, Larissa Ventura Ribeiro Bruscky, Georgina del Cisne Jadán Luzuriaga, Bruno Vaz Kerges Bueno, and Marcus Vinicius Simões. "AMILOIDOSE CARDÍACA: O QUE HÁ DE NOVO." Revista da Sociedade de Cardiologia do Estado de São Paulo 33, no. 3 (2023): 294–303. http://dx.doi.org/10.29381/0103-8559/20233303294-303.

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Amiloidose se refere a um conjunto de doenças causadas pelo depósito de fragmentos anômalos de diferentes proteínas. O coração é um dos principais órgãos afetados, nas formas mais comuns de amiloidose, com os pacientes apresentando mau prognóstico e sobrevida curta, conforme o grau de acometimento. Embora considerada uma afecção rara, vários trabalhos têm demonstrado um aumento expressivo no número de diagnósticos nos últimos anos. Tem contribuído para isso o maior arsenal diagnóstico não invasivo, com novas ferramentas de avaliação mais específicas em exames já consagrados na prática clínica

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Yilmaz, A., J. Bauersachs, F. Bengel, et al. "Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK)." Clinical Research in Cardiology 110, no. 4 (2021): 479–506. http://dx.doi.org/10.1007/s00392-020-01799-3.

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AbstractSystemic forms of amyloidosis affecting the heart are mostly light-chain (AL) and transthyretin (ATTR) amyloidoses. The latter is caused by deposition of misfolded transthyretin, either in wild-type (ATTRwt) or mutant (ATTRv) conformation. For diagnostics, specific serum biomarkers and modern non-invasive imaging techniques, such as cardiovascular magnetic resonance imaging (CMR) and scintigraphic methods, are available today. These imaging techniques do not only complement conventional echocardiography, but also allow for accurate assessment of the extent of cardiac involvement, in ad

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Wisniowski, Brendan, and Ashutosh Wechalekar. "Confirming the Diagnosis of Amyloidosis." Acta Haematologica 143, no. 4 (2020): 312–21. http://dx.doi.org/10.1159/000508022.

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Amyloidosis is a general term for diseases characterised by the deposition of insoluble amyloid fibrils in organs or tissues, leading to organ dysfunction and, in many cases, death. Amyloid fibrils are derived from soluble precursor proteins, with the number of known amyloidogenic proteins increasing over time. The identity of the precursor protein often predicts the disease phenotype, although many of the amyloidoses have overlapping clinical features. Most patients with amyloidosis will require biopsy of an involved organ or tissue to confirm the diagnosis. Cardiac transthyretin amyloidosis,

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Herzog, Eyal, David Leibowitz, Moshe E. Gatt, Tali Koren, and Arthur Pollak. "Pathway for the Diagnosis and Management of Cardiac Amyloidosis." Critical Pathways in Cardiology: A Journal of Evidence-Based Medicine 22, no. 4 (2023): 114–19. http://dx.doi.org/10.1097/hpc.0000000000000331.

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The systemic amyloidoses are a broad spectrum of diseases that result from misfolding of proteins that aggregate into amyloid fibrils. In cardiac amyloidosis, amyloid fibrils accumulate in the interstitial space between cardiac myocytes causing cellular injury and impairing compliance. Current data suggest that cardiac amyloidosis is more common than previously thought. Advances in cardiac imaging, diagnostic strategies, and therapies have improved the recognition and treatment of cardiac amyloidosis. A position statement for the diagnosis and treatment of cardiac amyloidosis has been publishe

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Ruiz-Mori, Enrique, Leonor Ayala-Bustamante, Luis Taxa-Rojas, Cristian Pacheco-Román, Javier Alarcón-Santos, and Jorge Burgos-Bustamante. "Amiloidosis cardiaca: reporte de un caso." Horizonte Médico (Lima) 18, no. 4 (2018): 81–89. http://dx.doi.org/10.24265/horizmed.2018.v18n4.12.

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Triposkiadis, Filippos, Alexandros Briasoulis, and Andrew Xanthopoulos. "Amyloids and the Heart: An Update." Journal of Clinical Medicine 13, no. 23 (2024): 7210. http://dx.doi.org/10.3390/jcm13237210.

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Amyloids consist of fibrils that can be formed by a large variety of different precursor proteins. In localized amyloidosis, amyloids accumulate at the production site with a single organ being affected, whereas in systemic amyloidosis several organs are affected, with the heart being the most common, followed by the kidneys, liver, and the nervous system. The two most frequent systemic amyloidosis types affecting the heart in the vast majority (>95%) of cases are immunoglobulin light chain (AL) amyloidosis and transthyretin (TTR) amyloidosis (ATTR amyloidosis). Patients with amyloid cardio

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Ponomarev, B., K. R. Pashayeva, N. Y. Feyziyeva, and Stefka Gospodinova Radenska-Lopovok. "The features of cardiomyopathy in AL and ATTR amyloidosis." Rheumatology (Bulgaria) 32, no. 2 (2024): 15–20. https://doi.org/10.35465/32.2.2024.pp15-20.

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Amyloidoses are protein misfolding diseases characterized by the accumulation of amyloid fibrils in the interstitium leading to the damage of an affected organ. Cardiac involvement is quite common and results from light chain amyloidosis (AL) or transthyretin amyloidosis (ATTR), including two subtypes: wild type (ATTRwt) and variant ATTR (ATTRv), which can lead to hereditary cardiac amyloidosis. Heart damage leads to life-threatening cardiomyopathy (CM) with poor prognosis. Besides the alteration of the tissue caused by the deposition of amyloid fibrils, experimental studies reveal some comple

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Theodorakakou, Foteini, Despina Fotiou, Meletios A. Dimopoulos, and Efstathios Kastritis. "Solid Organ Transplantation in Amyloidosis." Acta Haematologica 143, no. 4 (2020): 352–64. http://dx.doi.org/10.1159/000508262.

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Amyloidosis comprises a diverse group of diseases characterized by misfolding of precursor proteins which eventually form amyloid aggregates and preceding intermediaries, which are deposited in target tissues causing progressive organ damage. In all forms of amyloidosis, vital organs may fail; depending on the specific amyloidosis type, this may occur rapidly or progress slowly. Beyond therapies to reduce the precursor protein (chemotherapy for light chain [AL] amyloidosis, anti-inflammatory therapy in serum A amyloidosis [AA], and antisense RNA therapy in transthyretin amyloidosis [ATTR]), o

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Steinhardt, Maximilian J., Vladimir Cejka, Mengmeng Chen, et al. "Safety and Tolerability of SGLT2 Inhibitors in Cardiac Amyloidosis—A Clinical Feasibility Study." Journal of Clinical Medicine 13, no. 1 (2024): 283. http://dx.doi.org/10.3390/jcm13010283.

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Sodium-glucose transport protein 2 inhibitors (SGLT2i) slow the progression of renal dysfunction and improve the prognosis of patients with heart failure. Amyloidosis constitutes an important subgroup for which evidence is lacking. Amyloidotic fibrils originating from misfolded transthyretin and light chains are the causal agents in ATTR and AL amyloidosis. In these most frequent subtypes, cardiac involvement is the most common organ manifestation. Because cardiac and renal function frequently deteriorate over time, even under best available treatment, SGLT2i emerge as a promising treatment op

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Kitchlu, Abhijat, Christopher T. Chan, Kenar D. Jhaveri, Diego Delgado, and Paul Tam. "Amyloidoses in Onco-Nephrology Practice: A Multidisciplinary Case-Based Conference Report." Canadian Journal of Kidney Health and Disease 10 (January 2023): 205435812311657. http://dx.doi.org/10.1177/20543581231165711.

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Introduction and Objective: Amyloidoses are a heterogeneous group of disorders resulting from deposition of amyloid fibrils into extracellular tissues. While the kidneys are one of the most frequent sites of amyloid deposition, amyloid deposits can also affect a wide range of organ systems, including the heart, liver, gastrointestinal tract, and peripheral nerves. The prognosis of amyloidosis, especially with cardiac involvement, remains poor; however, a collaborative approach applying new tools for diagnosis and management may improve outcomes. In September 2021, the Canadian Onco-Nephrology

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Sun, Yang, Yan-hong Zhao, Qiong-fen Wang, et al. "AL type renal amyloidosis with cardiac involvement: A case report and literature review." Medicine 104, no. 16 (2025): e42161. https://doi.org/10.1097/md.0000000000042161.

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Rationale: Systemic amyloidosis refers to a spectrum of diseases characterized by extracellular deposition of amyloids, with commonrenal involvement; however, simultaneous involvement of cardiac amyloidosis remains rare. The purpose of this report was to enhance the understanding of the diagnosis and treatment of light chain (AL)-type renal amyloidosis with cardiovascular involvement, reduce misdiagnosis and missed diagnosis, and enable timely and effective treatment for such patients. Patient concerns: A 74-year-old male patient was admitted to our hospital because of recurrent edema for over

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Eniseeva, E. S. "АМИЛОИДНАЯ КАРДИОМИОПАТИЯ: ДИАГНОСТИКА И ЛЕЧЕНИЕ". Baikal Medical Journal 2, № 4 (2023): 11–23. http://dx.doi.org/10.57256/2949-0715-2023-4-11-23.

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The review presents current data on heart disease in systemic amyloidosis. The article discusses variants of amyloi-dosis with common heart disease: AL-, ATTRwt- and ATTRv amyloidoses. Clinical signs of amyloid cardiomyopathy and extracardiac manifestations that allow one to suggest amyloidosis are described. Methods for diagnosing amyloid cardiomyopathy are presented. Echocardiographic diagnostic characters include increased thickness of the left ventricular wall, atrioventricular valve, right ventricular wall, thickening of the interatrial septum, and also diastolic dysfunction, and decrease

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Merlini, Giampaolo, David C. Seldin, and Morie A. Gertz. "Amyloidosis: Pathogenesis and New Therapeutic Options." Journal of Clinical Oncology 29, no. 14 (2011): 1924–33. http://dx.doi.org/10.1200/jco.2010.32.2271.

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The systemic amyloidoses are a group of complex diseases caused by tissue deposition of misfolded proteins that results in progressive organ damage. The most common type, immunoglobulin light chain amyloidosis (AL), is caused by clonal plasma cells that produce misfolded light chains. The purpose of this review is to provide up-to-date information on diagnosis and treatment options for AL amyloidosis. Early, accurate diagnosis is the key to effective therapy, and unequivocal identification of the amyloidogenic protein may require advanced technologies and expertise. Prognosis is dominated by t

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Czyżewska, Emilia, Agnieszka Wiśniewska, Anna Waszczuk-Gajda, and Olga Ciepiela. "The Role of Light Kappa and Lambda Chains in Heart Function Assessment in Patients with AL Amyloidosis." Journal of Clinical Medicine 10, no. 6 (2021): 1274. http://dx.doi.org/10.3390/jcm10061274.

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There are reports indicating that myocardial dysfunction in systemic immunoglobulin light chain amyloidosis (AL amyloidosis) stems not only from the amyloid deposit in the organ but also the cardiotoxicity of the amyloid precursor free light chains (FLCs) circulating in the blood. The aim of the study is to analyze the role of sFLC κ and λ in the assessment of heart involvement and the degree of myocardial damage in AL amyloidosis. The study involved 71 patients diagnosed with primary AL amyloidosis. The relationship between sFLC concentrations and cardiac biochemical and echocardiographic par

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Desjardins, Baptiste, Kénol Franck, Nathalie Deschamps, et al. "A comprehensive evaluation of cardiac amyloidosis epidemiology and diagnostics in French Guiana." PLOS One 20, no. 6 (2025): e0324955. https://doi.org/10.1371/journal.pone.0324955.

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Background Cardiac amyloidosis (CA) is a potentially fatal systemic disease that has received increasing attention in recent years. However, there is no data on its epidemiology in French Guiana. This study aimed to evaluate the epidemiological characteristics of cardiac amyloidosis and describe the regional diagnostic pathways in French Guiana. Methods We performed a multicenter retrospective study of Guianese patients with confirmed or suspected cardiac amyloidosis who were followed up in hospitals in French Guiana by private cardiologists. Results A total of 47 patients were included. The s

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Rameeva, A. S., V. V. Rameev, I. N. Bobkova, A. F. Safarova, Zh D. Kobalava, and S. V. Moiseev. "Leading Factors of Progression in Patients with Cardiac Amyloidosis." Rational Pharmacotherapy in Cardiology 18, no. 2 (2022): 143–52. http://dx.doi.org/10.20996/1819-6446-2022-04-02.

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Aim. To describe prognostic meaning of cardiac and other principal clinical manifestations of systemic AL-amyloidosis in their interrelations.Material and methods. It has been made long-time survival analysis of 147 patients with systemic AL-amyloidosis. In the special investigation group (n=58) of AL (n=55) and ATTR (n=3) amyloidotic cardiopathy patients there were evaluated prognostically important structural and functional changes in myocardium with standard and impulse-wave tissue dopplerometric echocardiography in comparison with NTproBNP serum levels.Results. Even though significantly in

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Pipitsa, Valsamaki. "ASSUMER on PYP Scan for Cardiac Amyloid Typing and Origin: Case-Specific Corroborating Clinical Background and Genetic Analysis." J Biomed Res Environ Sci 4, no. 1 (2023): 008–13. https://doi.org/10.37871/jbres1643.

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Suspicion for Cardiac Amyloidosis (CA) led a 75-year-old male patient with fatigue, dyspnea, and palpitations to be subjected to scintigraphic myocardial investigation with technetium-99m-pyrophosphate (99mTc-PYP, or PYP scan) in order to distinguish transthyretin-related CA (ATTR) from the light-chain Amyloidosis (AL) form. A characteristic anamnesis of bilateral carpal tunnel syndrome, vertebral canal stenosis, and senile cataract, as well as laboratory, Electrocardiographic (ECG), and echocardiographic exploration was compatible with a positive for ATTR PYP scan. Specific features on PYP sc

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del Cisne Jadán Luzuriag, Georgina, Fábio Fernandes, Natália de Melo Pereira, et al. "TRATAMENTO COM PATISIRANA EM PACIENTES COM AMILOIDOSE CARDÍACA POR TRANSTIRRETINA - APOLLO-B." Revista da Sociedade de Cardiologia do Estado de São Paulo 34, no. 1 (2024): 43–51. http://dx.doi.org/10.29381/0103-8559/2024340143-51.

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A amiloidose por transtirretina (ATTR) é uma doença associada ao acúmulo de depósito amiloide de fragmentos desta proteína, comumente associada a cardiomiopatia progressiva (CM-ATTR). A patisirana, um agente terapêutico de interferência no RNA, inibe a produção hepática da TTR. Métodos. Estudo randomizado, duplo-cego, de fase 3, que incluiu pacientes com CM-ATTR variante ou selvagem, numa razão 1:1, para receber patisirana (0,3 mg/kg) ou placebo uma vez a cada três semanas por 12 meses. O desfecho primário avaliou a diferença entre a distância percorrida no teste de caminhada de 6 minutos (TC6

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Danková, M., and E. Gonçalvesová. "Amyloidová kardiomyopatia – zriedkavá alebo neodhalená? / Cardiac amyloidosis – rare or underdiagnosed?" Cardiology letters 29, no. 01 (2020): 17–25. http://dx.doi.org/10.4149/cardiol_2020_1_17.

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Соколовский, N. Sokolovskiy, Козырев, et al. "Comparative Analysis of the Acyzol Effects in the Prevention of Models of Amyloidosis." Journal of New Medical Technologies 22, no. 2 (2015): 50–55. http://dx.doi.org/10.12737/11831.

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First, the authors obtained two models of the system cardiopatic amyloidosis in rats. The first model was created by a single injection of equal mixture of native egg albumin and Freund&#180;s complete adjuvant 0.2 ml per five points of injection (subcutaneously in the axillary and inguinal region, left and right intraperitoneally). The second model was created with a single introduction of a mixture of native egg albumin (40%), Freund&#180;s complete adjuvant (40%), and myocardial homogenate of rats (20%) at a dose of 0.2 ml in a similar five-point-injection. To prevent cardiac

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Koike, Haruki, and Masahisa Katsuno. "The Ultrastructure of Tissue Damage by Amyloid Fibrils." Molecules 26, no. 15 (2021): 4611. http://dx.doi.org/10.3390/molecules26154611.

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Amyloidosis is a group of diseases that includes Alzheimer’s disease, prion diseases, transthyretin (ATTR) amyloidosis, and immunoglobulin light chain (AL) amyloidosis. The mechanism of organ dysfunction resulting from amyloidosis has been a topic of debate. This review focuses on the ultrastructure of tissue damage resulting from amyloid deposition and therapeutic insights based on the pathophysiology of amyloidosis. Studies of nerve biopsy or cardiac autopsy specimens from patients with ATTR and AL amyloidoses show atrophy of cells near amyloid fibril aggregates. In addition to the stress or

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Laptseva, Natallia, Dominik C. Benz, Rahel Schwotzer, and Andreas J. Flammer. "Cardiac amyloidosis." Swiss Medical Weekly 154, no. 11 (2024): 4186. http://dx.doi.org/10.57187/s.4186.

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Cardiac amyloidosis is a disease characterised by the accumulation of amyloid protein in the heart tissue. There are several types of amyloidosis, but the most common types affecting the heart are ATTR amyloidosis (caused by transthyretin protein) and AL amyloidosis (caused by abnormal immunoglobulin light chains). Cardiac amyloidosis causes typical signs and symptoms of heart failure. Diagnosis involves a combination of imaging tests such as echocardiography and cardiac magnetic resonance imaging, as well as nuclear imaging scans and tissue biopsies to confirm the presence of amyloid deposits

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D'Souza, Anita, Keren Osman, Cristiana Costa Chase, Azah Borham, and Marianna Bruno. "The Hematologist's Role in Amyloidosis Management: Disease Awareness, Diagnostic Workup, and Practice Patterns." Blood 136, Supplement 1 (2020): 28–29. http://dx.doi.org/10.1182/blood-2020-137740.

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Introduction: Systemic amyloidoses are progressive, life-threatening diseases characterized by the deposition of amyloid protein fibrils of varying origin in different tissues/organ systems (Merlini G, et al. N Engl J Med 2003;349:583-96). The precursor amyloidogenic protein influences the disease's clinical course, and identification of the specific protein is essential because treatment varies substantially by subtype. While there are 36 known proteins that can aggregate as amyloid in humans (Benson MD, et al. Amyloid 2018;25:215-9), the two most prevalent protein subtypes causing cardiac am

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Itzhaki Ben Zadok, Osnat, and Ran Kornowski. "Cardiac Care of Patients with Cardiac Amyloidosis." Acta Haematologica 143, no. 4 (2020): 343–51. http://dx.doi.org/10.1159/000506919.

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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, emphasizin

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Savova, R., E. Kinova, and A. Goudev. "Cardiac amyloidosis – an underestimated etiology for heart failure with preserved ejection fraction. A literary review." Bulgarian Cardiology 30, no. (4) (2024): 7–19. https://doi.org/10.3897/bgcardio.30.e140741.

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Cardiac amyloidosis, once considered a rare disease, is a severe and progressive cardiomyopathy characterized by extracellular deposition of misfolded proteins in the ventricular myocardium. Early diagnosis of cardiac amyloidosis is essential for improving patient prognosis, as it allows for a broader range of therapeutic options that can enhance outcomes and/or prevent potentially irreversible loss of physical function and quality of life. Current data suggest that a majority of patients do not get a timely diagnosis. Patients with early-stage CA do not exhibit reduced global left ventricular

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Aydın Akpınar, Emine, Pınar Pelin Özcan, Zehra Pınar Koç, Adil Gümüş, and Gökçe Yavan. "Comparison of Static and Spect Images on Tc-99m-PYP Bone Scintigraphy in the Diagnosis of Cardiac Amyloidosis." Molecular Oncologic Imaging 4, no. 3 (2025): 39–45. https://doi.org/10.71286/moi.1534280.

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Amyloidosis is a systemic disease characterized by the extracellular accumulation of amyloid fibrils caused by misfolding of proteins. Cardiac amyloidosis has the worst prognosis of the type of organ involvements. The most common forms of cardiac amyloidosis are immunoglobulin light chain amyloidosis (AL) and transtretin (TTR) amyloidosis. The developing diagnostic methods give the clinician early diagnosis of cardiac amyloidosis. Scintigraphic imaging with Technetium (Tc-99m) labelled biphosponates may faciliate early detection of cardiac involvement and distinguish various forms of cardiac a

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del Cisne Jadán Luzuriaga, Georgina, Fábio Fernandes, Natália de Melo Pereira, et al. "TRATAMENTO COM PATISIRANA EM PACIENTES COM AMILOIDOSE CARDÍACA POR TRANSTIRRETINA - APOLLO-B." Revista da Sociedade de Cardiologia do Estado de São Paulo 34, no. 1 (2024): 43–45. http://dx.doi.org/10.29381/0103-8559/2024340143-5.

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A amiloidose por transtirretina (ATTR) é uma doença associada ao acúmulo de depósito amiloide de fragmentos desta proteína, comumente associada a cardiomiopatia progressiva (CM-ATTR). A patisirana, um agente terapêutico de interferência no RNA, inibe a produção hepática da TTR. Métodos. Estudo randomizado, duplo-cego, de fase 3, que incluiu pacientes com CM-ATTR variante ou selvagem, numa razão 1:1, para receber patisirana (0,3 mg/kg) ou placebo uma vez a cada três semanas por 12 meses. O desfecho primário avaliou a diferença entre a distância percorrida no teste de caminhada de 6 minutos (TC6

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Butorov, Ekaterina A., and Olga V. Stukalova. "Role of cardiac MRI in the diagnosis of cardiac amyloidosis. Clinical cases." Clinical review for general practice 2, no. 2 (2021): 16–20. http://dx.doi.org/10.47407/kr2021.2.2.00037.

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Purpose. The aim of this work is to show the capabilities of late gadolinium enhancement cardiac magnetic resonance imaging (MRI) in the diagnosis of a rare disease such as cardiac amyloidosis. Materials and methods. Demonstration of clinical cases detecting cardiac amyloidosis using MRI. Results. Contrast-enhanced cardiac MRI revealed patterns characteristic of cardiac amyloidosis. Conclusion. Cardiac MRI with late gadloinium enhancement is the method of choice in the diagnosis of cardiac amyloidosis.

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Cowan, Andrew J., Martha Skinner, J. Mark Sloan, et al. "Macroglossia – Not Always AL Amyloidosis." Blood 116, no. 21 (2010): 5007. http://dx.doi.org/10.1182/blood.v116.21.5007.5007.

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Abstract Abstract 5007 Introduction: Amyloidosis is characterized by extracellular deposition of abnormal insoluble fibrillar proteins. The two most frequent systemic amyloidoses are the light-chain (AL amyloidosis) and familial transthyretin (ATTR) forms. Clinical presentations often vary between the two types. Macroglossia is viewed as pathognomic of AL amyloidosis, and has not previously been described in patients with hereditary TTR amyloidosis. Here, we describe two cases of systemic amyloidosis with macroglossia in which immuno-electron microscopy diagnosed ATTR in one and AL in the othe

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Abe, Temidayo, Eric Y. Chang, Gabrielle De Allie, Taiwo Ajose, Chukwuemeka Nwokike, and Nicolas Bakinde. "Rapid decline in ejection fraction and persistent elevation of troponin associated with cardiac amyloidosis." SAGE Open Medical Case Reports 8 (January 2020): 2050313X2092325. http://dx.doi.org/10.1177/2050313x20923259.

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Cardiac amyloidosis is an increasingly recognized cause of heart failure. It remains underdiagnosed despite a significant morbidity and mortality rate. The mean survival in patients with cardiac amyloidosis is less than 1 year in untreated primary light-chain amyloidosis and less than 4 years in wild-type transthyretin amyloidosis. We report a unique case of a 78-year-old male with transthyretin cardiac amyloidosis, who presented with persistently elevated troponin and progressive heart failure unresponsive to conventional therapy. With this case, we would like to highlight the role of cardiac

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Li, Weijia, Dipan Uppal, Yu Chiang Wang, et al. "Nuclear Imaging for the Diagnosis of Cardiac Amyloidosis in 2021." Diagnostics 11, no. 6 (2021): 996. http://dx.doi.org/10.3390/diagnostics11060996.

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Cardiac amyloidosis is caused by the deposition of misfolded protein fibrils into the extracellular space of the heart. The diagnosis of cardiac amyloidosis remains challenging because of the heterogeneous manifestations of the disease. There are many different types of amyloidosis with light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis being the most common types of cardiac amyloidosis. Endomyocardial biopsy is considered the gold standard for diagnosing cardiac amyloidosis and differentiating amyloid subtypes, but its use is limited because of the invasive nature of the proced

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Wechalekar, Ashutosh D., Helen J. Lachmann, Hugh J. B. Goodman, Arthur Bradwell, Philip N. Hawkins, and Julian D. Gillmore. "AL amyloidosis associated with IgM paraproteinemia: clinical profile and treatment outcome." Blood 112, no. 10 (2008): 4009–16. http://dx.doi.org/10.1182/blood-2008-02-138156.

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AbstractAL amyloidosis associated with immunoglobulin M (IgM) paraproteinemia is rare. We report 103 consecutive such patients evaluated at the National Amyloidosis Centre (London, United Kingdom) between 1988 and 2006. Renal, cardiac, and lymph node amyloid was present in 53%, 35%, and 21% of patients, respectively, at presentation and 2 or more organs were involved in 54%. Seventy-three percent had an abnormal bone marrow infiltrate (lymphoid in 87%). The median IgM paraprotein was 8 g/L and serum free light chain (FLC) ratio was abnormal in 77 (88%) of 87. The abnormal FLC component was mor

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Di Nora, Concetta, Sandro Sponga, Chiara Nalli, et al. "Heart transplantation in cardiac amyloidosis." Vessel Plus 6 (2022): 46. http://dx.doi.org/10.20517/2574-1209.2021.126.

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It is known that the prognosis of patients affected by light-chain (AL) or transthyretin-related (TTR) amyloidosis is poor. TTR amyloidosis has usually shown a slower progression than AL amyloidosis, both hereditary TTR amyloidosis, where there is an inherited mutation in the DNA, and wild-type TTR amyloidosis, which usually affects the elderly. In this paper, the current literature about heart transplantation on cardiac amyloidosis patients is extensively reviewed. The two most frequent types of cardiac amyloidosis have been considered for heart transplantation: AL amyloidosis and wild-type T

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Bonderman, Diana, Gerhard Pölzl, Klemens Ablasser, et al. "Diagnosis and treatment of cardiac amyloidosis: an interdisciplinary consensus statement." Wiener klinische Wochenschrift 132, no. 23-24 (2020): 742–61. http://dx.doi.org/10.1007/s00508-020-01781-z.

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SummaryThe prevalence and significance of cardiac amyloidosis have been considerably underestimated in the past; however, the number of patients diagnosed with cardiac amyloidosis has increased significantly recently due to growing awareness of the disease, improved diagnostic capabilities and demographic trends. Specific therapies that improve patient prognosis have become available for certain types of cardiac amyloidosis. Thus, the earliest possible referral of patients with suspicion of cardiac amyloidosis to an experienced center is crucial to ensure rapid diagnosis, early initiation of t

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Mohan Krushna, Chenga, Praneeth Ulavala, and C. Prabhakar Raju. "Heart of the Matter: Understanding Cardiac Amyloidosis and Its Evolving Landscape." International Journal of Science and Research (IJSR) 12, no. 9 (2023): 1072–74. http://dx.doi.org/10.21275/mr23910164005.

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Demko, I. V., L. I. Pelinovskaya, Irina A. Soloveva, A. Yu Kraposhina, N. V. Gordeeva, and V. A. Mosina. "Primary cardiac amyloidosis." Clinical Medicine (Russian Journal) 95, no. 11 (2018): 971–76. http://dx.doi.org/10.18821/0023-2149-2017-95-11-971-976.

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Cardiac amyloidosis - the disease which is characterized by deposition of insoluble protein amyloid in intercellular space is one of the most severe implications of systemic amyloidosis. Primary cardiac amyloidosis possesses a wide range of clinical implications that complicates well-timed diagnostics and, respectively, treatment. The long latent current and the adverse forecast do extremely important diagnostics of cardiac amyloidosis at early stages of a disease. The modern concept of pathogenesis and morphology of an amyloidosis is covered in article, the clinical options of a current, diag

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Marzuki, Hadi, Erwin A. Soeriadi, Astri Astuti, Hendra Budiawan, and Achmad Hussein S. Kartamihardja. "Difference Uptake of 99mTc-Pyrophosphate vs 99mTc- Methylenediphosphate in Detecting Cardiac Amyloidosis; First Experience in Hasan Sadikin General Hospital." Journal of Medicine and Health 4, no. 1 (2022): 10. http://dx.doi.org/10.28932/jmh.v4i1.3640.

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Cardiac amyloidosis (CA) is a disorder caused by deposits of insoluble fibrils in the myocardium and an underdiagnosed cause of heart failure. Immunoglobulin Light Chain-Associated Amyloid (AL) and Amyloidosis Transthyretin-related (ATTR) are the two protein precursors that most commonly cause cardiac amyloidosis. It is important to distinguish between these two types of amyloidosis because the treatment and prognosis of each types is different. Recent advances in cardiac radionuclide imaging, in particular using bone-seeking agents such as 99mTc-Pyrophosphate (PYP) and 99mTc-Methylenediphosph

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Bukhari, Syed. "Prevalence, Predictors and Diagnosis of Cardiac Amyloidosis in Patients with Aortic Stenosis." Journal of Biomedical Research & Environmental Sciences 4, no. 9 (2023): 1277–80. http://dx.doi.org/10.37871/jbres1796.

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Cardiac amyloidosis and aortic stenosis often share a common clinical phenotype, and are associated with morbidity and mortality if untreated. Cardiac amyloidosis is present in ~15% of patients with aortic stenosis. Clinical suspicion for cardiac amyloidosis in aortic stenosis is often raised based on history (bilateral carpal tunnel syndrome, severe lumbar spinal stenosis, spontaneous biceps rupture), chronic troponin elevation, electrocardiographic features (low-voltage criteria, pseudoinfarct pattern), echocardiogram (bilateral ventricular hypertrophy, abnormal longitudinal strain) and magn

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Lu, P., H. Van Acker, and P. Waer. "Cardiac amyloidosis." Journal of the Belgian Society of Radiology 98, no. 1 (2015): 48. http://dx.doi.org/10.5334/jbr-btr.755.

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Yusuf, Syed Wamique, Amirreza Solhpour, Jose Banchs, et al. "Cardiac amyloidosis." Expert Review of Cardiovascular Therapy 12, no. 2 (2014): 265–77. http://dx.doi.org/10.1586/14779072.2014.876363.

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Gertz, Morie A. "Cardiac Amyloidosis." Heart Failure Clinics 18, no. 3 (2022): 479–88. http://dx.doi.org/10.1016/j.hfc.2022.02.005.

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Witteles, Ronald, and Bonnie Ky. "Cardiac Amyloidosis." JACC: CardioOncology 3, no. 4 (2021): 617–18. http://dx.doi.org/10.1016/j.jaccao.2021.09.002.

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Le, Dan, Rasheed Zaid, Jeffrey Dela Cruz, Faisal Nabi, and Dipan Shah. "Cardiac Amyloidosis." Methodist DeBakey Cardiovascular Journal 9, no. 4 (2013): 237. http://dx.doi.org/10.14797/mdcvj.454.

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Le, Dan, Rasheed Zaid, Jeffrey Dela Cruz, Faisal Nabi, Stephen Little, and Dipan Shah. "Cardiac Amyloidosis." Methodist DeBakey Cardiovascular Journal 10, no. 2 (2014): 130. http://dx.doi.org/10.14797/mdcvj.491.

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Elbdri, Salah, and Rachel Hajar. "Cardiac amyloidosis." Heart Views 22, no. 3 (2021): 231. http://dx.doi.org/10.4103/heartviews.heartviews_94_21.

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