Pubblicazioni recenti - cardiac amyloidosis

• Impact of Delayed Diagnosis and Misdiagnosis for Patients with Transthyretin Amyloid Cardiomyopathy (ATTR-CM): A Targeted Literature Review.
  Cardiol Ther

  2021 Apr;():. doi: 10.1007/s40119-021-00219-5.
  
  Rozenbaum Mark H, Large Samuel, Bhambri Rahul, Stewart Michelle, Whelan Jo, van Doornewaard Alexander, Dasgupta Noel, Masri Ahmad, Nativi-Nicolau Jose,
  
  Abstract
  
  INTRODUCTION:
  Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive, fatal and under-recognized disease. This targeted literature review assessed the extent and consequences of diagnostic delay and misdiagnosis in ATTR-CM.
  
  METHODS:
  The Embase database was searched together with proceedings of eight cardiology conferences to identify publications or abstracts on ATTR-CM. Outcomes of interest were time from symptom onset to diagnosis, rates of delayed diagnosis and misdiagnosis, and costs, healthcare resource use or clinical outcomes whilst undiagnosed/misdiagnosed.
  
  RESULTS:
  Twenty-three articles were included. Weighted means of reported mean and median diagnostic delays were 6.1 and 3.4 years for wild-type (ATTRwt-CM) and 5.7 and 2.6 years for hereditary (ATTRv-CM). Misdiagnosis occurred in 34-57% of patients when reported. Evaluation and misdiagnosis by multiple healthcare providers before receiving an ATTR-CM diagnosis was common, and there was evidence that patients undergo unnecessary or inappropriate evaluations or treatments while misdiagnosed. Diagnostic "red flags" were reported to be underused. Data on the consequences of delay for patients and health systems were sparse, but given the progressive nature of ATTR-CM, delay is likely to have adverse consequences.
  
  CONCLUSION:
  ATTR-CM patients commonly experience diagnostic delay and misdiagnosis. Efforts are required to provide timely diagnosis so that patients can benefit from earlier access to new disease-modifying therapies.
  
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• IGVL gene region usage correlates with distinct clinical presentation in IgM vs non-IgM light chain amyloidosis.
  Blood Adv

  2021 Apr;5(8):2101-2105. doi: 10.1182/bloodadvances.2020003671.
  
  Sidana Surbhi, Dasari Surendra, Kourelis Taxiarchis V, Dispenzieri Angela, Murray David L, King Rebecca L, McPhail Ellen D, Ramirez-Alvarado Marina, Kumar Shaji K, Gertz Morie A,
  
  Abstract
  
  Patients with immunoglobulin M (IgM) light chain (AL) amyloidosis have a distinct clinical presentation compared with those with non-IgM amyloidosis. We hypothesized that differential immunoglobulin light-chain variable region (IGVL) gene usage may explain the differences in organ involvement, because IGVL usage correlates with organ tropism. IGVL usage was evaluated by mass spectrometry of amyloid deposits (IgM, n = 45; non-IgM, n = 391) and differed across the 2 groups. In the ? family, LV2-08 (13% vs 2%; P < .001) and LV2-14 (36% vs 10%; P < .001) usage was more common in IgM vs non-IgM amyloidosis, whereas LV1-44 (0% vs 10%; P = .02) and LV6-57 (2% vs 18%; P = .004) usage was less common. In the ? family, there was a trend toward higher KV4-01 (11% vs 4%; P = .06) usage in IgM amyloidosis. IGVL usage correlated with disease characteristics/organ tropism. LV2-14 (more common in IgM amyloidosis) has historically been associated with peripheral nerve involvement and lower light chain burden, which were more frequent in IgM amyloidosis. LV1-44 (less common in IgM), associated with cardiac involvement, was less frequent in IgM patients. LV6-57 (less common in IgM) is associated with t(11;14), which was less frequent in IgM patients. In conclusion, IGVL gene usage differs in patients with IgM vs non-IgM amyloidosis and may explain the distinct clinical presentation.
  
  © 2021 by The American Society of Hematology.
  
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• Cardiac manifestations in Finnish gelsolin amyloidosis patients.
  Amyloid

  2021 Apr;():1-5. doi: 10.1080/13506129.2021.1911798.
  
  Mustonen Tuuli, Holkeri Arttu, Holmström Miia, Atula Sari, Pakarinen Sami, Lehmonen Lauri, Kiuru-Enari Sari, Aro Aapo L,
  
  Abstract
  
  INTRODUCTION:
  Finnish gelsolin amyloidosis (AGel amyloidosis) is an inherited systemic amyloidosis with well-known ophthalmological, neurological and cutaneous symptoms. Additionally, cardiomyopathies, conduction disorders and need of cardiac pacemakers occur in some patients. This study focuses on electrocardiographic (ECG) findings in AGel amyloidosis and their relation to cardiac magnetic resonance (CMR) changes. We also assessed whether ECG abnormalities were associated with pacemaker implantation and mortality.
  
  MATERIALS AND METHODS:
  In this cohort study, 51 genetically verified AGel amyloidosis patients (mean age 66?years) without cardiac pacemakers underwent 12-lead ECG and CMR imaging with contrast agent in 2017. Patients were followed-up for 3?years.
  
  RESULTS:
  Conduction disturbances were found in 22 patients (43%). Nine (18%) presented with first-degree atrioventricular block, six (12%) with left anterior hemiblock, seven (14%) with left or right bundle branch block and two (4%) with non-specific intraventricular conduction delay. Low QRS voltage was present in two (4%) patients. Late gadolinium enhancement (LGE) concentrating on the interventricular septum and inferior parts of the heart was present in 19 (86%) patients with conduction abnormalities. During the follow-up, only one patient received a pacemaker, and one patient died.
  
  DISCUSSION:
  Conduction disorders and septal LGE are common in AGel amyloidosis, whereas other ECG and CMR findings typically observed in most common cardiac amyloidosis types were rare. Septal pathology seen in CMR may interfere with the cardiac conduction system in AGel amyloidosis, explaining conduction disorders, although pacemaker therapy is rarely required.
  
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• Cardiac Magnetic Resonance-Derived Extracellular Volume Mapping for the Quantification of Hepatic and Splenic Amyloid.
  Circ Cardiovasc Imaging

  2021 Apr;():CIRCIMAGING121012506. doi: 10.1161/CIRCIMAGING.121.012506.
  
  Chacko Liza, Boldrini Michele, Martone Raffaele, Law Steven, Martinez-Naharrro Ana, Hutt David F, Kotecha Tushar, Patel Rishi K, Razvi Yousuf, Rezk Tamer, Cohen Oliver C, Brown James T, Srikantharajah Mukunthan, Ganesananthan Sharmananthan, Lane Thirusha, Lachmann Helen J, Wechalekar Ashutosh D, Sachchithanantham Sajitha, Mahmood Shameem, Whelan Carol J, Knight Daniel S, Moon James C, Kellman Peter, Gillmore Julian D, Hawkins Philip N, Fontana Marianna,
  
  Abstract
  
  BACKGROUND:
  Systemic amyloidosis is characterized by amyloid deposition that can involve virtually any organ. Splenic and hepatic amyloidosis occurs in certain types, in some patients but not others, and may influence prognosis and treatment. SAP (serum amyloid P component) scintigraphy is uniquely able to identify and quantify amyloid in the liver and spleen, thus informing clinical management, but it is only available in 2 centers globally. The aims of this study were to examine the potential for extracellular volume (ECV) mapping performed during routine cardiac magnetic resonance to: (1) detect amyloid in the liver and spleen and (2) estimate amyloid load in these sites using SAP scintigraphy as the reference standard.
  
  METHODS:
  Five hundred thirty-three patients referred to the National Amyloidosis Centre, London, between 2015 and 2017 with suspected systemic amyloidosis who underwent SAP scintigraphy and cardiac magnetic resonance with T1 mapping were studied.
  
  RESULTS:
  The diagnostic performance of ECV to detect splenic and hepatic amyloidosis was high for both organs (liver: area under the curve, -0.917 [95% CI, 0.880-0.954]; liver ECV cutoff, 0.395; sensitivity, 90.7%; specificity, 77.7%; <0.001; spleen: area under the curve, -0.944 [95% CI, 0.925-0.964]; spleen ECV cutoff, 0.385; sensitivity, 93.6%; specificity, 87.5%; <0.001). There was good correlation between liver and spleen ECV and amyloid load assessed by SAP scintigraphy (r=0.504, <0.001; r=0.693, <0.001, respectively). There was high interobserver agreement for both the liver and spleen (ECV liver intraclass correlation coefficient, 0.991 [95% CI, 0.984-0.995]; <0.001; ECV spleen intraclass correlation coefficient, 0.995 [95% CI, 0.991-0.997]; <0.001) with little bias across a wide range of ECV values.
  
  CONCLUSIONS:
  Our study demonstrates that ECV measurements obtained during routine cardiac magnetic resonance scans in patients with suspected amyloidosis can identify and measure the magnitude of amyloid infiltration in the liver and spleen, providing important clues to amyloid type and offering a noninvasive measure of visceral amyloid burden that can help guide and track treatment.
  
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• Systemic light-chain amyloidosis incidentally diagnosed after subtotal parathyroidectomy and thyroid lobectomy.
  BMJ Case Rep

  2021 Apr;14(4):. doi: e241282.
  
  Tsai Karen, Yu Alice Chen, Livhits Masha J, Sajed Dipti, Leung Angela M, Cheung Dianne S,
  
  Abstract
  
  A 74-year-old woman with a history of primary hyperparathyroidism, thyroid nodules, atrial fibrillation and pacemaker placement for sick sinus syndrome presented with fatigue, constipation and persistent lower extremity oedema. She underwent subtotal parathyroidectomy and left thyroid lobectomy. Histopathology revealed amyloidosis affecting the thyroidand parathyroids confirmed by Congo Red Staining with Mayo Clinic subtyping of light chain kappa-type amyloidosis. She was found to have combined systolic and diastolic cardiac dysfunction, carpal tunnel neuropathy and pre-diabetes suggestive of systemic amyloidosis with involvement of the heart, nerves and pancreas. Congo red stain was positive for amyloidosis on bone marrow biopsy suggestive of a diagnosis of systemic amyloidosis. She was treated with daratumumab with good clinical response. This case illustrates the necessity of considering systemic amyloidosis in patients with incidentally discovered diffuse amyloid deposits on biopsy of an endocrine organ, as endocrine effects are a rare but likely underdiagnosed consequence of systemic amyloidosis.
  
  © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.
  
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• [Update of tafamidis for the treatment of transthyretin cardiac amyloidosis].
  Zhonghua Xin Xue Guan Bing Za Zhi

  2021 Apr;49(4):314-317. doi: 10.3760/cma.j.cn112148-20200920-00748.
  
  Tian Z, Zhang S Y,
  
  
  
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• A Case Report of Sick Sinus Syndrome as an Initial Presentation of Primary Amyloidosis.
  Cureus

  2021 Mar;13(3):e13922. doi: 10.7759/cureus.13922.
  
  Abdelazeem Basel, Malik Bilal, Baral Nischit, Gjeka Rudin, Kunadi Arvind,
  
  Abstract
  
  Primary light chain amyloidosis (AL amyloidosis) rarely presents as sick sinus syndrome (SSS), and only a few cases have been reported in the literature. A higher index of suspicion is needed to diagnose AL amyloidosis in patients presenting with SSS. Recognizing the electrocardiography (ECG) and transthoracic echocardiogram (TTE) findings for amyloidosis are crucial for early recognition, proper management, and to improve the patients' quality of life. A 79-year-old female initially presented with dyspnea and was diagnosed with SSS that required a pacemaker insertion. Ten days later, the patient had complained of dysphagia and difficulty swallowing. She underwent an esophagogastroduodenoscopy (EGD) to investigate further, and it revealed esophageal and duodenal ulcers, and biopsy was positive for amyloidosis. The patient was worked up for amyloidosis, including bone marrow biopsy, renal biopsy, frees light chains, and serum electrophoresis, which all confirmed the diagnosis of primary amyloidosis. Unfortunately, due to the terminal nature of her condition, the patient was discharged with comfort measures to hospice care.
  
  Copyright © 2021, Abdelazeem et al.
  
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• Cardiac Amyloidosis Presenting as Biventricular Systolic Heart Failure.
  Case Rep Cardiol

  2021 ;2021():6671469. doi: 10.1155/2021/6671469.
  
  Oye Monique, Richardson Aaron, Sadic Edin, Alkhasawneh Ahmad, Velarde Gladys,
  
  Abstract
  
  A previously healthy octogenarian presented with new onset heart failure symptoms. Comprehensive multimodality imaging including complete echocardiography with longitudinal strain analysis, cardiac magnetic resonance imaging (cMRI), nuclear medicine pyrophosphate (99-mcTcPYP) scan along with biomarker, monoclonal protein analysis, and fat pad biopsy confirmed diagnosis of transthyretin cardiac amyloidosis.
  
  Copyright © 2021 Monique Oye et al.
  
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• Cardiac amyloidosis characterization by kinetic model fitting on [18F]florbetaben PET images.
  J Nucl Cardiol

  2021 Apr;():. doi: 10.1007/s12350-021-02608-8.
  
  Santarelli M F, Genovesi D, Scipioni M, Positano V, Favilli B, Giorgetti A, Vergaro G, Landini L, Emdin M, Marzullo P,
  
  Abstract
  
  OBJECTIVE:
  To evaluate the feasibility of kinetic modeling-based approaches from [18F]-Flobetaben dynamic PET images as a non-invasive diagnostic method for cardiac amyloidosis (CA) and to identify the two AL- and ATTR-subtypes.
  
  METHODS AND RESULTS:
  Twenty-one patients with diagnoses of CA (11 patients with AL-subtype and 10 patients with ATTR-subtype of CA) and 15 Control patients with no-CA conditions underwent PET/CT imaging after [18F]Florbetaben bolus injection. A two-tissue-compartment (2TC) kinetic model was fitted to time-activity curves (TAC) obtained from left ventricle wall and left atrium cavity ROIs to estimate kinetic micro- and macro-parameters. Combinations of kinetic parameters were evaluated with the purpose of distinguishing Control subjects and CA patients, and to correctly label the last ones as AL- or ATTR-subtype. Resulting sensitivity, specificity, and accuracy for Control subjects were: 0.87, 0.9, 0.89; as far as CA patients, the sensitivity, specificity, and accuracy were respectively 0.9, 1, and 0.97 for AL-CA patients and 0.9, 0.92, 0.97 for ATTR-CA patients.
  
  CONCLUSION:
  Pharmacokinetic analysis based on a 2TC model allows cardiac amyloidosis characterization from dynamic [18F]Florbetaben PET images. Estimated model parameters allows to not only distinguish between Control subjects and patients, but also between AL- and ATTR-amyloid patients.
  
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• Transforming ATTR cardiac amyloidosis into a chronic disease: The enormous potential of quantitative SPECT to improve diagnosis, prognosis, and monitoring of disease progression.
  J Nucl Cardiol

  2021 Apr;():. doi: 10.1007/s12350-021-02587-w.
  
  Ayers Michael P, Peruri Adithya V, Bourque Jamieson M,
  
  
  
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• Narrative review of pharmacotherapy for transthyretin cardiac amyloid.
  Ann Transl Med

  2021 Mar;9(6):519. doi: 10.21037/atm-20-4636.
  
  Cruz Rodriguez Jose B, Tallaj Jose A,
  
  Abstract
  
  Treatment of cardiac amyloidosis is determined by the amyloid type and degree of involvement. Two types of amyloid commonly infiltrate the heart: immunoglobulin light-chain amyloid (AL), and transthyretin amyloid (ATTR), that encompasses other two forms, a hereditary form (hATTR), and a sporadic, age-related wild-type (wtATTR). The prevalence is expected to increase with aging population. The natural history of ATTR cardiomyopathy includes progressive heart failure (HF), complicated by arrhythmias and conduction system disease. New therapies options have been approved or are under investigation. We performed a narrative literature review, manually-searched the reference lists of included articles and relevant reviews. Treatment for cardiac ATTR should be directed towards alleviation of HF symptoms and to slow or stop progressive amyloid deposition. Conventional HF medications are poorly tolerated and may not alter the disease progression or symptoms, except perhaps with the administration of diuretics. There are three approaches of therapy for ATTR cardiomyopathy: tetramer stabilizers, inhibition of ATTR protein synthesis and clearance of deposited fibrils. Tafamidis diminishes the progression of cardiomyopathy, functional parameters, improves overall outcome in patients with early disease stages, irrespective of ATTR status and is well tolerated. Diflunisal has shown promising results in early studies, but at the expense of significant side effects. Two new agents, antisense oligonucleotides, patisiran and inotersen are under investigation in cardiac amyloidosis. Patisiran appears to be the most effective treatment for hATTR, although evidence is limited, with a relatively small cardiac subpopulation. Therapies considering clearance of amyloid fibrils from tissue remain experimental. In conclusion, tafamidis is the only approved agent for the treatment of ATTR cardiomyopathy although multiple other agents have shown promising early results and are undergoing clinical trials. Careful consideration of the type of ATTR, comorbidities and disease stage will be key in deciding the optimal therapy for ATTR patients.
  
  2021 Annals of Translational Medicine. All rights reserved.
  
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• Patisiran for advanced heart failure with hereditary transthyretin cardiac amyloidosis.
  J Cardiol Cases

  2021 Apr;23(4):177-180. doi: 10.1016/j.jccase.2021.01.007.
  
  Nakamura Makiko, Imamura Teruhiko, Kinugawa Koichiro,
  
  Abstract
  
  We experienced a 78-year-old woman who was diagnosed with hereditary transthyretin cardiac amyloidosis and administered patisiran for advanced heart failure refractory to tafamidis. The levels of N-terminal pro B-type natriuretic peptide and left ventricular mass index decreased following the six-month patisiran treatment without any complications. Patisiran might be a promising disease-modifying drug for hereditary transthyretin cardiac amyloidosis even in its advanced stage, although further evaluation in a large cohort is warranted.
  
  © 2021 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
  
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• Usefulness of electron microscopy in the diagnosis of wild-type transthyretin cardiac amyloidosis.
  J Cardiol Cases

  2021 Apr;23(4):166-169. doi: 10.1016/j.jccase.2020.11.023.
  
  Sunayama Isamu, Yoshimura Takahiro, Sonoura Takuryu, Nakamura Yuka, Morishita Yu, Sekihara Takayuki, Ishimi Masashi, Yamato Masashi, Hoshida Yoshihiko, Yasuoka Yoshinori,
  
  Abstract
  
  Wild-type transthyretin cardiac amyloidosis (ATTRwt) has been recognized as an important cause of heart failure with preserved ejection fraction; thus, its accurate diagnosis is crucial. Herein, we describe the case of a 76-year-old man who presented with dyspnea and palpitation. On observing the laboratory evaluations and clinical course, we suspected cardiac amyloidosis. However, optical microscopic analysis by Congo-red and direct fast scarlet staining revealed no amyloid deposits in the biopsy samples. Therefore, a more thorough investigation was pursued by examining the myocardial tissue under electron microscopy. We could recognize amyloid deposits between the myocardial fibers using electron microscopy. We submitted all the pathological specimens to a specialized facility for genetic testing to ensure the accurate diagnosis of the amyloidosis disease type. As a result, a biopsy sample from the minor salivary gland was stained with the Congo red stain. Anti-transthyretin antibody detected using immunohistochemical analysis of amyloidosis supported the presence of transthyretin form of amyloid proteins. Genetic testing revealed the absence of TTR gene mutations. The final diagnosis was ATTRwt. We believe that this case suggests the usefulness of electron microscopy in the diagnosis of ATTRwt and other related disorders. Further study is warranted to validate our findings.
  
  © 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
  
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• Ventricular tachyarrhythmias and sudden cardiac death in light-chain amyloidosis: a clash of cardio-toxicities?
  Br J Haematol

  2021 Apr;():. doi: 10.1111/bjh.17399.
  
  Zampieri Mattia, Allinovi Marco, Olivotto Iacopo, Antonioli Elisabetta, Gabriele Martina, Argirò Alessia, Fumagalli Carlo, Nardi Giulia, Di Mario Carlo, Vannucchi Alessandro M, Perfetto Federico, Cappelli Francesco,
  
  
  
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• Incidence rate of hospitalization and mortality in the first year following initial diagnosis of cardiac amyloidosis in the US claims databases.
  Curr Med Res Opin

  2021 Apr;():1. doi: 10.1080/03007995.2021.1913109.
  
  Wang Lu, Swerdel Joel N, Weaver James, Weiss Brendan, Pan Guohua, Yuan Zhong, DiBattiste Peter M,
  
  Abstract
  
  OBJECTIVE:
  This study aimed to determine rates of hospitalization and in-hospital mortality in the first year following amyloidosis diagnosis with cardiac involvement using observational databases.
  
  METHODS:
  Three administrative claims databases, IBM MarketScan® Commercial Claims and Encounters (CCAE), IBM MarketScan Multi-State Medicare Database (MDCR), and Optum's de-identified Clinformatics Data Mart Database (Optum) were analyzed. Adults ?18 years old, with a diagnosis of amyloidosis and evidence of cardiac involvement (i.e., heart failure, heart block, or cardiomyopathy) but no hepatic/renal failure prior to amyloidosis diagnosis were included for analysis. The primary analyses identified patients between 2010-01-01 and 2017-12-31 period. We calculated the rates of hospitalization and in-hospital mortality within 1 year after the initial diagnosis of amyloidosis. A sensitivity analysis was conducted for patients identified in Optum database during 2004-2011 period, which provided additional mortality information.
  
  RESULTS:
  A total of 419, 654, and 922 patients from CCAE, MDCR, and Optum were identified during 2010-2017 period, with mean age of 55.6, 77.8, and 74.2 years, respectively. Within 1 year following initial amyloidosis diagnosis, incidence rates (95% confidence interval) of hospitalization were 78.4 (66.3, 90.4), 78.6 (69.2, 87.9), and 61.2 (54.4, 68.0) per 100 person-years, rates of in-hospital mortality were 16.5 (11.8, 21.3), 8.4 (5.7, 11.0), and 17.7 (14.5, 21.0) per 100 person-years, in CCAE, MDCR, and Optum, respectively. The mortality rate from the sensitivity analysis among patients identified in Optum 2004-2011 period was higher compared with Optum 2010-2017 period.
  
  CONCLUSIONS:
  The results from this study indicate that amyloidosis with cardiac involvement is a condition with high rates of hospitalization and mortality in the first year after initial diagnosis. Future studies are needed to further evaluate the outcomes within the subtypes of amyloidosis and understand the risk factors associated with poor prognoses.
  
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• Periorbital ecchymosis and shoulder pad sign in transthyretin amyloidosis.
  BMJ Case Rep

  2021 Apr;14(4):. doi: e242614.
  
  Ono Ryohei, Kajiyama Takatsugu, Miyauchi Hideyuki, Kobayashi Yoshio,
  
  
  
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• Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases.
  Eur J Heart Fail

  2021 Apr;():. doi: 10.1002/ejhf.2140.
  
  Garcia-Pavia Pablo, Rapezzi Claudio, Adler Yehuda, Arad Michael, Basso Cristina, Brucato Antonio, Burazor Ivana, Caforio Alida L P, Damy Thibaud, Eriksson Urs, Fontana Marianna, Gillmore Julian D, Gonzalez-Lopez Esther, Grogan Martha, Heymans Stephane, Imazio Massimo, Kindermann Ingrid, Kristen Arnt V, Maurer Mathew S, Merlini Giampaolo, Pantazis Antonis, Pankuweit Sabine, Rigopoulos Angelos G, Linhart Ales,
  
  Abstract
  
  Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.
  
  © European Society of Cardiology 2021.
  
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• Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESC Working Group on Myocardial and Pericardial Diseases.
  Eur Heart J

  2021 Apr;():. doi: ehab072.
  
  Garcia-Pavia Pablo, Rapezzi Claudio, Adler Yehuda, Arad Michael, Basso Cristina, Brucato Antonio, Burazor Ivana, Caforio Alida L P, Damy Thibaud, Eriksson Urs, Fontana Marianna, Gillmore Julian D, Gonzalez-Lopez Esther, Grogan Martha, Heymans Stephane, Imazio Massimo, Kindermann Ingrid, Kristen Arnt V, Maurer Mathew S, Merlini Giampaolo, Pantazis Antonis, Pankuweit Sabine, Rigopoulos Angelos G, Linhart Ales,
  
  Abstract
  
  Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.
  
  © European Society of Cardiology 2021 This article has been co-published with permission in European Heart Journal (published by Oxford University Press on behalf of European Society of Cardiology) and European Journal of Heart Failure (published by John Wiley & Sons Ltd on behalf of European Society of Cardiology) These articles are identical except for minor stylistic and spelling differences in keeping with each journal?s style. Either citation can be used when citing this article.
  
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• Absolute quantitative analysis of cardiac amyloidosis using SPECT/CT with Tc-pyrophosphate.
  Amyloid

  2021 Apr;():1-2. doi: 10.1080/13506129.2021.1903418.
  
  Yanagisawa Shin, Takahashi Yusuke, Sasaki Jun, Takasone Ken, Yoshie Koji, Koyama Jun, Katoh Nagaaki, Yazaki Masahide, Kuwahara Koichiro, Fujinaga Yasunari, Sekijima Yoshiki,
  
  
  
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• Intermediate-term outcomes of heart transplantation for cardiac amyloidosis in the current era.
  Clin Transplant

  2021 Apr;():e14308. doi: 10.1111/ctr.14308.
  
  Vaidya Gaurang N, Patel Jignesh K, Kittleson Michelle, Chang David H, Kransdorf Evan, Geft Dael, Czer Lawrence, Vescio Robert, Esmailian Fardad, Kobashigawa Jon A,
  
  Abstract
  
  BACKGROUND:
  Cardiac amyloidosis (CA) has been historically noted with poor outcomes after heart transplant (HTx). However, strict patient selection, appropriate multi-organ transplant, and aggressive post-transplant therapy can result in favorable outcomes. We present the experience in the largest single-center cohort of CA patients post-HTx in the recent era.
  
  METHODS:
  Between January 2010 and December 2018, 51 CA patients underwent HTx-13 light-chain amyloidosis (AL) and 38 transthyretin amyloidosis (ATTR), 49 were included. Endpoints included 3-year survival, freedom from cardiac allograft vasculopathy (CAV), and freedom from non-fatal major adverse cardiac events (NF-MACE).
  
  RESULTS:
  Overall 3-year survival was 81.6% (69.2% for AL and 86% for ATTR) and was comparable to survival for patients transplanted for non-amyloid restrictive cardiomyopathy (RCM) in the same period (89%, p = .46). Three-year freedom from CAV (84% vs. 89%, p = .98), NF-MACE (82% vs. 83%, p = .96), and any-treated rejection (95% vs. 89%, p = .54) were also comparable in both groups. No recurrence in amyloid was noted in endomyocardial biopsies. Six patients (46%) with AL amyloidosis underwent autologous stem cell transplant 1-year post-HTx, and two patients (8%) with variant ATTR-CA underwent combined heart-liver transplant due to cardiac cirrhosis.
  
  CONCLUSION:
  In the current era, both AL and ATTR cardiac amyloidosis patients have acceptable outcomes after heart transplantation.
  
  © 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
  
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