SOSTIENICI
Pubblicazioni recenti - cardiac amyloidosis
-
COVID-19 Exposure Unmasking Systemic Amyloidosis With Hepatic Predominance.
Cureus2022 Nov;14(11):e31092. doi: e31092.
Russe-Russe Jose R, Abramowitz Chiya, Pellegrini James R, Alvarez Betancourt Alejandro, Cohen Ricky, Baldino Michael, Crandall Ronald, Kagolanu Deepthi, Mejia Jose, Rizvon Kaleem,
Abstract
Amyloidosis is characterized by depositing insoluble fibrillar proteins that misfold into beta-pleated sheets. This phenomenon occurs on a systemic or local level and may interfere with the function of various organs, including the heart, kidneys, and liver. Among those presenting with amyloidosis, hepatic, gastrointestinal, renal, cardiac, vitreous, and immunological involvement may occur. These manifestations are linked to several clinical presentations, varying from abdominal pain and hepatomegaly to restrictive cardiomyopathy and chronic renal failure. The two most common types of amyloid proteins are amyloid light chain (AL) and serum amyloid A (AA) proteins. AL produced by immunoglobulin light chains kappa and lambda (?, ?) circulate systemically and accumulate in organs. At the same time, serum AA proteins are acute-phase reactants seen in infectious, chronic inflammatory states. In an immune-mediated infection such as COVID-19, serum AA levels may be a predictive factor of disease severity and a valuable biomarker to monitor the clinical course of COVID-19 patients. This report highlights a case in which infection with COVID-19 provoked an effective immune response that may have contributed to the accelerated progression of systemic amyloidosis with hepatic involvement. The study further investigates the involvement of AL and AA proteins in COVID-19 infections, including their role in synergistically exacerbating an already grueling clinical course.
Copyright © 2022, Russe-Russe et al.
Guarda su PubMed
-
Systemic amyloidosis journey from diagnosis to outcomes: a twelve-year real-world experience of a single center in a middle-income country.
Orphanet J Rare Dis2022 Dec;17(1):425. doi: 10.1186/s13023-022-02584-3.
Szor Roberta Shcolnik, Fernandes Fabio, Lino Angelina Maria Martins, Mendonça Leonardo Oliveira, Seguro Fernanda Salles, Feitosa Valkercyo Araujo, Castelli Jussara Bianchi, Jorge Lecticia Barbosa, de Oliveira Alves Lucas Bassolli, de Menezes Neves Precil Diego Miranda, de Oliveira Souza Evandro, Cavalcante Livia Barreira, Malheiros Denise, Kalil Jorge, Martinez Gracia Aparecida, Rocha Vanderson,
Abstract
BACKGROUND:
Systemic amyloidosis is caused by the deposition of misfolded protein aggregates in tissues, leading to progressive organ dysfunction and death. Epidemiological studies originate predominantly from high-income countries, with few data from Latin America. Due to the non-specific clinical manifestations, diagnosing amyloidosis is often challenging and patients experience a long journey and delay in diagnosis. This study aimed to assess clinical and laboratory characteristics, the diagnostic journey, and outcomes of patients with biopsy-proven systemic amyloidosis diagnosed between 2009 and 2020 at a university referral center in a middle-income Latin American country. Patients´ medical records were retrospectively reviewed.
RESULTS:
One hundred and forty-three patients were included. The median age at diagnosis was 60 years and 54% were male. Until the diagnosis, most of the patients (52%) were seen by at least 3 specialists, the main ones being: general practitioners (57%), nephrologists (45%), and cardiologists (38%). The most common manifestations were renal (54%) and cardiac (41%) disorders, and cachexia was seen in 36% of patients. In 72% of the cases,???2 biopsies were required until the final diagnosis. The median time from symptoms onset to diagnosis was 10.9 months, and most patients (75%) had???2 organs involved. The following subtypes were identified: AL (68%), ATTR (13%), AA (8%), AFib (4%), and inconclusive (7%). Median OS was 74.3 months in the non-AL subgroup and 18.5 months in AL. Among AL patients, those with advanced cardiac stage had the worst outcome [median OS 8.6 months versus 52.3 for stage III versus I-II, respectively (p?0.001)]. AL subtype, cardiac involvement, and ECOG???2 were identified as independent risk factors for reduced survival.
CONCLUSIONS:
Systemic amyloidosis is still an underdiagnosed condition and the delay in its recognition leads to poor outcomes. Medical education, better diagnostic tools, improvement in access to therapies, and establishment of referral centers may improve patient outcomes in middle-income countries.
© 2022. The Author(s).
Guarda su PubMed
-
Prognostic value of left ventricular global constructive work in patients with cardiac amyloidosis.
Int J Cardiovasc Imaging2022 Dec;():. doi: 10.1007/s10554-022-02762-1.
Geers Jolien, Luchian Maria-Luiza, Motoc Andreea, De Winter Jari, Roosens Bram, Bjerke Maria, Van Eeckhaut Ann, Wittens Mandy M J, Demeester Simke, Forsyth Ramses, de Ravel Thomy, Bissay Véronique, Schots Rik, Verbrugge Frederik H, Weytjens Caroline, Weets Ilse, Cosyns Bernard, Droogmans Steven,
Abstract
PURPOSE:
The aim of the present study was to evaluate the role of ejection fraction (EF), left ventricular (LV) global longitudinal strain (LVGLS) and global constructive work (GCW) as prognostic variables in patients with cardiac amyloidosis (CA).
METHODS:
CA patients were retrospectively identified between 2015 and 2021 at a tertiary care hospital. Comprehensive clinical, biochemical, and imaging evaluation including two-dimensional (2D) echocardiography with myocardial work (MW) analysis was performed. A clinical combined endpoint was defined as all-cause mortality and heart failure readmission.
RESULTS:
70 patients were followed for 16 (7-37) months and 37 (52.9%) reached the combined endpoint. Patient with versus without clinical events had a significantly lower LVEF (40.71% vs. 48.01%, p?=?0.039), LVGLS (-9.26 vs. -11.32, p?=?0.034) and GCW (1034.47mmHg% vs. 1424.86mmHg%, p?=?0.011). Multivariable analysis showed that LVEF ( odds ratio (OR): 0.904; 95% confidence interval (CI): 0.839-0.973, p?=?0.007), LVGLS ( OR: 0.620; 95% CI: 0.415-0.926, p?=?0.020) and GCW ( OR: 0.995; 95% CI: 0.990-0.999, p?=?0.016) were significant predictors of outcome, but the model including GCW had the best discriminative ability to predict the combined endpoint (C-index?=?0.888). A GCW less than 1443mmHg% was able to predict the clinical endpoint with a sensitivity of 94% and a specificity of 64% (Area under the curve (AUC): 0.771 (95% CI: 0.581-0.961; p?=?0.005)).
CONCLUSION:
In CA patients, GCW may be of additional prognostic value to LVEF and GLS in predicting heart failure hospitalization and all-cause mortality.
© 2022. The Author(s), under exclusive licence to Springer Nature B.V.
Guarda su PubMed
-
Differential diagnosis of common etiologies of left ventricular hypertrophy using a hybrid CNN-LSTM model.
Sci Rep2022 Dec;12(1):20998. doi: 10.1038/s41598-022-25467-w.
Hwang In-Chang, Choi Dongjun, Choi You-Jung, Ju Lia, Kim Myeongju, Hong Ji-Eun, Lee Hyun-Jung, Yoon Yeonyee E, Park Jun-Bean, Lee Seung-Pyo, Kim Hyung-Kwan, Kim Yong-Jin, Cho Goo-Yeong,
Abstract
Differential diagnosis of left ventricular hypertrophy (LVH) is often obscure on echocardiography and requires numerous additional tests. We aimed to develop a deep learning algorithm to aid in the differentiation of common etiologies of LVH (i.e. hypertensive heart disease [HHD], hypertrophic cardiomyopathy [HCM], and light-chain cardiac amyloidosis [ALCA]) on echocardiographic images. Echocardiograms in 5 standard views (parasternal long-axis, parasternal short-axis, apical 4-chamber, apical 2-chamber, and apical 3-chamber) were obtained from 930 subjects: 112 with HHD, 191 with HCM, 81 with ALCA and 546 normal subjects. The study population was divided into training (n?=?620), validation (n?=?155), and test sets (n?=?155). A convolutional neural network-long short-term memory (CNN-LSTM) algorithm was constructed to independently classify the 3 diagnoses on each view, and the final diagnosis was made by an aggregate network based on the simultaneously predicted probabilities of HCM, HCM, and ALCA. Diagnostic performance of the algorithm was evaluated by the area under the receiver operating characteristic curve (AUC), and accuracy was evaluated by the confusion matrix. The deep learning algorithm was trained and verified using the training and validation sets, respectively. In the test set, the average AUC across the five standard views was 0.962, 0.982 and 0.996 for HHD, HCM and CA, respectively. The overall diagnostic accuracy was significantly higher for the deep learning algorithm (92.3%) than for echocardiography specialists (80.0% and 80.6%). In the present study, we developed a deep learning algorithm for the differential diagnosis of 3 common LVH etiologies (HHD, HCM and ALCA) by applying a hybrid CNN-LSTM model and aggregate network to standard echocardiographic images. The high diagnostic performance of our deep learning algorithm suggests that the use of deep learning can improve the diagnostic process in patients with LVH.
© 2022. The Author(s).
Guarda su PubMed
-
Wild-Type Transthyretin Amyloid Cardiomyopathy: The Gordian-Knot of Novel Therapeutic Regimens.
Cardiol Rev;31(1):36-41. doi: 10.1097/CRD.0000000000000427.
Zegkos Thomas, Gossios Thomas, Ntelios Dimitris, Parcharidou Despoina, Karvounis Haralampos, Efthimiadis Georgios,
Abstract
Wild-type TTR amyloidosis (wtATTR) represents a disease difficult to diagnose with poor prognosis. Increased clinical suspicion is key, allowing for timely diagnosis. Until recently, only off-label therapies were available but recent introduction of disease specific therapy has shown potential to alter the natural history of the disease. Tafamidis, the only currently approved drug for the therapy of wtATTR, provided significantly better survival and quality of life. However, not all subgroups of patients derived equal benefit. This, along with the increased cost of treatment raised question on whether treatment should be invariably administered through the wtATTR population. This review aims to summarize current evidence on the natural history and staging systems for wtATTR, as well as available treatment options. Special consideration is given to the selection process of patients who would be expected to gain maximum benefit from tafamidis treatment, based on an ethical and cost-effective point of view.
Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.
Guarda su PubMed
-
99mTc-Pyrophosphate Scintigraphy Can Image Tracer Uptake in Skeletal Trunk Muscles of Transthyretin Cardiac Amyloidosis.
Clin Nucl Med2023 Jan;48(1):18-24. doi: 10.1097/RLU.0000000000004397.
Takahashi Koji, Hiratsuka Yoshiyasu, Sasaki Daisuke, Sakaue Tomoki, Enomoto Daijiro, Morioka Hiroe, Uemura Shigeki, Okura Takafumi, Ikeda Shuntaro, Kono Taizo, Iwamura Takaaki, Yamamura Nobuhisa, Kitazawa Sohei, Ueda Mitsuharu,
Abstract
PURPOSE:
99mTc-pyrophosphate (99mTc-PYP) uptake in the skeletal muscles is minimal in patients with transthyretin cardiac amyloidosis (ATTR-CA) when assessed qualitatively and quantitatively. We previously demonstrated moderate- to high-grade 99mTc-PYP uptake in the subcutaneous abdominal fat of some patients with ATTR-CA and showed that this abnormal finding could reflect the regional amyloid burden of this tissue. We aimed to investigate the frequency of 99mTc-PYP uptake in skeletal trunk muscles of patients with ATTR-CA.
METHODS:
Chest- and abdomen-centered 99mTc-PYP scintigraphy images were obtained 2 hours after IV injections of the tracer (20 mCi) in 36 patients with ATTR-CA. The frequency of 99mTc-PYP uptake in the following 11 skeletal trunk muscles was investigated: pectoralis major, deltoid, subscapularis, infraspinatus, trapezius, latissimus dorsi, erector spinae, psoas major, abdominal oblique, rectus abdominis, and the gluteus muscles.
RESULTS:
Ten of the 11 muscles were involved in patients with the highest number of 99mTc-PYP uptake in the skeletal trunk muscles examined, whereas no muscle was involved in a patient with the least uptake. The muscle with the highest rate of 99mTc-PYP uptake, observed in 34 of 36 patients (94.4%), was the abdominal oblique. No tracer uptake was observed in the psoas major. The frequency of radiotracer uptake in the remaining examined muscles was between those of abdominal oblique and psoas major muscles.
CONCLUSIONS:
Radiotracer uptake was often detectable in some skeletal trunk muscles of ATTR-CA, although the muscles of patients examined and the skeletal trunk muscles of 1 patient showed heterogeneity in the uptake of 99mTc-PYP.
Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.
Guarda su PubMed
-
Coefficient of R-R interval variations under deep breathing load in patients with wild-type transthyretin amyloid cardiomyopathy: A case-control study.
Health Sci Rep2023 Jan;6(1):e938. doi: e938.
Nagayoshi Yasuhiro, Kawano Hiroaki, Nishihara Taiki, Morikawa Kei, Nagano Haruka, Hanatani Shinsuke, Sakaino Naritsugu, Tsujita Kenichi,
Abstract
BACKGROUND AND AIMS:
An autonomic nervous disorder is an important characteristic of cardiac amyloidosis; however, the prevalence of autonomic dysfunction in wild-type transthyretin amyloidosis (ATTR) has not been established. Analysis of the R-R interval coefficient of variation (CVR-R) is a noninvasive method to measure parasympathetic activity. We aimed to assess autonomic dysfunction of ATTR and determine the utility of CVR-R for the detection of ATTR in other cardiac diseases.
METHODS:
This is a single-center, retrospective, case-control study. Fifty patients with heart failure (HF) were studied. The etiologies of HF were as follows: ATTR, ?=?10; previous myocardial infarction (MI), ?=?20; and left ventricular hypertrophy (LVH) due to other disease processes (e.g., aortic stenosis), ?=?20. We measured the CVR-R at rest (CVR-R), CVR-R with deep breaths (CVR-R), and the change rate (CVR-R. The relative change formula is as follows: CVR-R?=?(CVR-R?-?CVR-R)/CVR-R ?100 (%).
RESULTS:
There was no difference in the CVR-R levels among the three groups. The CVR-R levels in the ATTR group were significantly lower (ATTR: -8.77 [-43.8 to 10.9]; LVH: 67.4 [38.7 to 89.4]; MI: 83.7 [60.4 to 142.9]). Based on the receiver operative characteristic curve analysis to identify ATTR in HF, the best cut-off value for the CVR-R was 19.7 (area under the curve: 0.848).
CONCLUSION:
Our data suggested autonomic dysfunction in patients with ATTR. Measurement of the CVR-R in HF patients may be a convenient support tool for the detection of ATTR.
© 2022 The Authors. Health Science Reports published by Wiley Periodicals LLC.
Guarda su PubMed
-
Single Center Experience of Autologous Stem Cell Transplantation in Patients with Systemic Light Chain Amyloidosis in Korea.
Clin Lymphoma Myeloma Leuk2022 Oct;():. doi: S2152-2650(22)01713-X.
Kim Hye Ryeon, Yoon Sang Eun, Kim Darae, Choi Jin-Oh, Min Ju-Hong, Kim Byung Jun, Kim Jung Sun, Lee Jung Eun, Choi Joon Young, Jeon Eun-Seok, Kim Seok Jin, Kim Kihyun,
Abstract
BACKGROUND:
Systemic light chains is the most common systemic amyloidosis. In patients with AL amyloidosis, the prognosis is influenced by the extent of organ damage, especially cardiac involvement. Autologous stem cell transplantation (ASCT) is a highly effective treatment for AL amyloidosis for selective patient METHODS: One hundred patients treated with ASCT for AL amyloidosis were reviewed in the Samsung Medical Center amyloidosis cohort. The cardiac, renal, and hematologic response was analyzed, and survival results compared based on organ involvement and hematologic response.
RESULTS:
The most common involved organ was kidney (n = 62) followed by heart (n = 50). The organ response rate was 44.0% and 37.1% in the patients with cardiac and renal involvement, respectively. In hematologic response, overall response rate (ORR) was 79.0%, including 48.0% complete response (CR). Median overall survival (OS) in patients with and without hematologic CR were not reached and 64.2 months (95% CI, 19.5 to 109.0), respectively (P
CONCLUSIONS:
ASCT is an effective treatment option for eligible patients with AL amyloidosis. Achieving hematologic CR is essential for long-term survival.
Copyright © 2022. Published by Elsevier Inc.
Guarda su PubMed
-
Real-Life Evaluation of an Algorithm for the Diagnosis of Cardiac Amyloidosis.
Mayo Clin Proc2022 Dec;():. doi: S0025-6196(22)00511-0.
Bézard Mélanie, Kharoubi Mounira, Galat Arnault, Le Bras Fabien, Poullot Elsa, Molinier-Frenkel Valérie, Fanen Pascale, Funalot Benoit, Moktefi Anissa, Abulizi Mukedaisi, Deux Jean-François, Lemonnier François, Guendouz Soulef, Chalard Coraline, Zaroui Amira, Itti Emmanuel, Hittinger Luc, Teiger Emmanuel, Oghina Silvia, Damy Thibaud,
Abstract
OBJECTIVE:
To evaluate the real-life use of a modified Gillmore algorithm with a "one-stop-shop" approach, bone scintigraphy (BS), a monoclonal gammopathy test (GT), a salivary gland biopsy (SGB), and genetic testing performed at the same time for the diagnosis of cardiac amyloidosis at the French National Reference Centre for Cardiac Amyloidosis (Henri Mondor Hospital, Créteil, France).
METHODS:
This retrospective cohort study included a total of 1222 patients with suspected amyloidosis who underwent BS and GT between June 2008 and May 2019.
RESULTS:
Of 1222 patients, 349 had no cardiac uptake on BS and negative GT (BS-/GT-), 276 were BS-/GT positive (GT+), 420 patients were BS+/GT-, and 177 were BS+/GT+. Our one-stop-shop check-up enabled us to diagnose 892 (72.9%) patients; 330 (27.0%) patients required additional examinations, such as mass spectrometry and/or a cardiac biopsy. This subset notably included 112 patients with amyloid light chain amyloidosis. More than 64% of the patients with transthyretin amyloidosis or another type of amyloidosis were diagnosed during the one-stop shop visit. Sensitivity and specificity of BS for transthyretin amyloidosis diagnosis was 99% and 96%, respectively. For amyloid light chain diagnosis, sensitivity and specificity were 100% and 76%, respectively, for GT and 54% and 100%, respectively, for SGB. Of 910 transthyretin genetic tests, 205 (17%) detected mutations.
CONCLUSION:
The results of our real-life cohort study confirmed the ability of a one-stop-shop approach with a modified Gillmore algorithm to diagnose cardiac amyloidosis and the interest of simultaneous testing for earlier diagnosis. The SGB has diagnostic value because it is easy, quick, and less invasive than a cardiac biopsy.
Copyright © 2022 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
Guarda su PubMed
-
Racial Differences in Atrial Fibrillation Management Between White Patients and Black Patients in Transthyretin Cardiac Amyloid.
Am J Cardiol2022 Nov;187():164-170. doi: 10.1016/j.amjcard.2022.10.039.
Mitrani Lindsey R, Tumasian Robert A, Vilches Silvia, De Los Santos Jeffeny, Gonzalez-Lopez Esther, Caponetti Angelo Giuseppe, Saturi Giulia, Mirelis Jesus G, Longhi Simone, Gagliardi Christian, Goldsmith Jeff, Rapezzi Claudio, García-Pavía Pablo, Maurer Mathew S,
Abstract
Black patients have higher rates of stroke than White patients. Paradoxically, atrial fibrillation (AF) affects twice as many White patients compared with Black patients. Transthyretin cardiac amyloidosis (ATTR-CA) is associated with both AF and strokes. We hypothesized that although Black patients with ATTR-CA have a lower incidence of AF, when diagnosed with AF, they have increased thromboembolic events. Patients with ATTR-CA (n = 558) at 3 international centers were retrospectively identified. We compared baseline characteristics, presence of AF, outcomes of thromboembolism (stroke, transient ischemic attack, and peripheral embolism), major bleed, and mortality by race. Of all patients, 367 of 488 White patients (75%) were diagnosed with AF compared with 39 of 70 Black patients (56%) (p = 0.001). Black patients with AF had a hazard ratio of 5.78 (95% confidence interval 2.30 to 14.50) for time to first thromboembolic event compared with White patients. There were no racial differences in major bleeding. Black patients with AF more often lacked anticoagulation (p = 0.038) and had higher incidence of labile international normalized ratio (p
Copyright © 2022 Elsevier Inc. All rights reserved.
Guarda su PubMed
-
Understanding AL amyloidosis with a little help from models.
Front Immunol2022 ;13():1008449. doi: 1008449.
Martinez-Rivas Gemma, Bender Sébastien, Sirac Christophe,
Abstract
Monoclonal immunoglobulin (Ig) light chain amyloidosis (AL) is a rare but severe disease that may occur when a B or plasma cell clone secretes an excess of free Ig light chains (LCs). Some of these LCs tend to aggregate into organized fibrils with a ?-sheet structure, the so-called amyloid fibrils, and deposit into the extracellular compartment of organs, such as the heart or kidneys, causing their dysfunction. Recent findings have confirmed that the core of the amyloid fibrils is constituted by the variable (V) domain of the LCs, but the mechanisms underlying the unfolding and aggregation of this fragment and its deposition are still unclear. Moreover, in addition to the mechanical constraints exerted by the massive accumulation of amyloid fibrils in organs, the direct toxicity of these variable domain LCs, full-length light chains, or primary amyloid precursors (oligomers) seems to play a role in the pathogenesis of the disease. Many studies have focused on these topics, but the variability of this disease, in which each LC presents unique properties, and the extent and complexity of affected organs make its study very difficult. Accordingly, several groups have focused on the development of animal models for years, with some encouraging but mostly disappointing results. In this review, we discuss the experimental models that have been used to better understand the unknowns of this pathology with an emphasis on approaches. We also focus on why reliable AL amyloidosis animal models remain so difficult to obtain and what this tells us about the pathophysiology of the disease.
Copyright © 2022 Martinez-Rivas, Bender and Sirac.
Guarda su PubMed
-
Myocardial Strain Imaging Using Feature Tracking Method of Cardiac MRI: Our Initial Experience of This Novel Parameter as an Additional Diagnostic Tool.
Indian J Radiol Imaging2022 Dec;32(4):479-487. doi: 10.1055/s-0042-1748760.
Chudgar Priya D, Burkule Nitin J, Kamat Nikhil V, Rege Gautam M, Jantre Mansi N,
Abstract
?Left ventricular ejection fraction (LVEF) is used as quantitative parameter to evaluate myocardial function. However, interobserver variation, limited reproducibility, and dependence on pre-load and after-load reduces its accuracy. The fall in LVEF occurs very late, when myocardial dysfunction is already advanced. Myocardial strain measurements (especially global longitudinal strain) is a new parameter to detect myocardial dysfunction before derangements in LVEF. The aim of this article is to share our experience of this novel diagnostic tool. ?Feature tracking method of strain assessment is performed using routine long and short axis cine images of cardiac MRI (CMR). Dedicated post-processing CMR software can perform off-line analysis and provide results in form of color-coded maps, percentage values as well as strain over time curve for each myocardial segments. ?Global longitudinal strain (GLS) is more sensitive than LVEF and can identify sub-clinical left ventricular (LV) dysfunction in various cardiomyopathies. It is also an important prognostic marker in serial assessment of heart failure patients. Regional differences in strain parameters can provide clues in hypertrophic cardiomyopathy as well as amyloidosis. GLS is recommended as routine measurement in patients undergoing chemotherapy to detect LV dysfunction prior to fall in LVEF. Strain imaging can be applied to guide placement of the LV pacing lead in patients receiving cardiac resynchronization therapy. More clinical data is needed to evaluate its role in ischemic heart disease. ?Strain imaging can identify LV dysfunction earlier than conventional methods and this opens a new perspective in risk stratification and therapeutic decision-making of various cardiac pathologies.
Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).
Guarda su PubMed
-
Left ventricular assist device in cardiac amyloidosis: friend or foe?
Heart Fail Rev2022 Dec;():. doi: 10.1007/s10741-022-10288-w.
Sciaccaluga Carlotta, De Carli Giuseppe, Fusi Chiara, Stefanini Andrea, Mandoli Giulia E, Giacomin Elisa, D'Ascenzi Flavio, Focardi Marta, Valente Serafina, Cameli Matteo,
Abstract
The prevalence of cardiac amyloidosis has progressively increased over the last years, being recognized as a significant cause of heart failure. In fact, the management of advanced heart failure is a cornerstone treatment of amyloid cardiomyopathy due to the frequent delay in its diagnosis. Left ventricular assist devices (LVADs) have been gaining importance in the scenario of end-stage heart failure, representing an alternative to heart transplant. However, only few studies have investigated the role of LVAD in restrictive cardiomyopathies such as cardiac amyloidosis, since there are several problems to consider. In fact, both anatomical factors and the restrictive physiology of this condition make LVAD implant a relevant challenge in this subset of patients. Furthermore, due to the systemic involvement of amyloidosis, several factors have to be considered after LVAD implant, such as an increased risk of bleeding and right ventricular failure. This review attempts to summarize the current evidence of LVAD in cardiac amyloidosis, especially focusing on the challenges that this cardiomyopathy imposes both to the implant and to its management thereafter.
© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Guarda su PubMed
-
New quantitative indices of cardiac amyloidosis with Tc-pyrophosphate scintigraphy.
Jpn J Radiol2022 Nov;():. doi: 10.1007/s11604-022-01364-0.
Matsuda Noritake, Otsuka Hideki, Otani Tamaki, Azane Shota, Kunikane Yamato, Otomi Yoichi, Ueki Yuya, Kubota Masahiro, Amano Masafumi, Yagi Shusuke, Sata Masataka, Harada Masafumi,
Abstract
PURPOSE:
Amyloid light chain (AL) and transthyretin (ATTR) are the major subtypes of cardiac amyloidosis (CA). Tc-pyrophosphate (PYP) scintigraphy is used to differentiate ATTR from other CA subtypes. We adapted the standardized uptake value (SUV) for Tc-PYP and proposed two quantitative indices, amyloid deposition volume (AmyDV) and total amyloid uptake (TAU). This study aimed to evaluate the utility of these quantitative indices in differentiating ATTR from non-ATTRs.
MATERIALS AND METHODS:
Before the SUV measurement, the Becquerel calibration factor (BCF) of Tc was obtained by a phantom experiment. Thirty-two patients who had undergone hybrid SPECT/CT imaging 3 h after injection of Tc-PYP (370 MBq) were studied. CT attenuation correction for image reconstruction was applied in all. We calculated SUV, AmyDV, and TAU using a quantitative analysis software program for bone SPECT (GI-BONE) and analyzed AmyDV using two methods: threshold method (set 40%); and constant value method (average SUV of ribs). We assessed the diagnostic ability of heart-to-contralateral lung (H/CL) ratio, SUV, AmyDV, and TAU to differentiate ATTR from non-ATTR using receiver operating characteristic (ROC) analysis.
RESULTS:
Statistically significant differences in all quantitative indices were observed between ATTR and non-ATTR. The area under the curve of each quantitative index for discriminating between ATTR and non-ATTR were as follows: H/CL, 0.997; SUV, 0.953; SUV (M1), 0.964; SUV (M2), 0.969; AmyDV (M1), 0.875; AmyDV (M2), 0.974; and TAU, 0.974. The AmyDV (M2) had higher diagnostic ability than AmyDV (M1). Thus, TAU was calculated as AmyDV (M2)?×?SUV (M2). In the ROC curve, SUV, AmyDV, and TAU had almost the same diagnostic ability as H/CL in distinguishing ATTR from non-ATTRs.
CONCLUSIONS:
We propose two novel 3D-based quantitative parameters (AmyDV and TAU) that have almost equal ability to discriminate ATTR from non-ATTR.
© 2022. The Author(s) under exclusive licence to Japan Radiological Society.
Guarda su PubMed
-
Identification of Transthyretin Cardiac Amyloidosis Among Patients Previously Diagnosed With Hypertrophic Cardiomyopathy.
Circ Cardiovasc Imaging2022 Nov;():e014938. doi: 10.1161/CIRCIMAGING.122.014938.
Rowin Ethan J, Ruberg Frederick L, Das Gaurav, Higgins Daniel, Lipe Willard C, Bokhari Nadia, Dehn Monica, Maron Barry J, Maron Martin S,
Guarda su PubMed
-
Automated Measurement of Native T1 and Extracellular Volume Fraction in Cardiac Magnetic Resonance Imaging Using a Commercially Available Deep Learning Algorithm.
Korean J Radiol2022 Dec;23(12):1251-1259. doi: 10.3348/kjr.2022.0496.
Chang Suyon, Han Kyunghwa, Lee Suji, Yang Young Joong, Kim Pan Ki, Choi Byoung Wook, Suh Young Joo,
Abstract
OBJECTIVE:
T1 mapping provides valuable information regarding cardiomyopathies. Manual drawing is time consuming and prone to subjective errors. Therefore, this study aimed to test a DL algorithm for the automated measurement of native T1 and extracellular volume (ECV) fractions in cardiac magnetic resonance (CMR) imaging with a temporally separated dataset.
MATERIALS AND METHODS:
CMR images obtained for 95 participants (mean age ± standard deviation, 54.5 ± 15.2 years), including 36 left ventricular hypertrophy (12 hypertrophic cardiomyopathy, 12 Fabry disease, and 12 amyloidosis), 32 dilated cardiomyopathy, and 27 healthy volunteers, were included. A commercial deep learning (DL) algorithm based on 2D U-net (Myomics-T1 software, version 1.0.0) was used for the automated analysis of T1 maps. Four radiologists, as study readers, performed manual analysis. The reference standard was the consensus result of the manual analysis by two additional expert readers. The segmentation performance of the DL algorithm and the correlation and agreement between the automated measurement and the reference standard were assessed. Interobserver agreement among the four radiologists was analyzed.
RESULTS:
DL successfully segmented the myocardium in 99.3% of slices in the native T1 map and 89.8% of slices in the post-T1 map with Dice similarity coefficients of 0.86 ± 0.05 and 0.74 ± 0.17, respectively. Native T1 and ECV showed strong correlation and agreement between DL and the reference: for T1, = 0.967 (95% confidence interval [CI], 0.951-0.978) and bias of 9.5 msec (95% limits of agreement [LOA], -23.6-42.6 msec); for ECV, = 0.987 (95% CI, 0.980-0.991) and bias of 0.7% (95% LOA, -2.8%-4.2%) on per-subject basis. Agreements between DL and each of the four radiologists were excellent (intraclass correlation coefficient [ICC] of 0.98-0.99 for both native T1 and ECV), comparable to the pairwise agreement between the radiologists (ICC of 0.97-1.00 and 0.99-1.00 for native T1 and ECV, respectively).
CONCLUSION:
The DL algorithm allowed automated T1 and ECV measurements comparable to those of radiologists.
Copyright © 2022 The Korean Society of Radiology.
Guarda su PubMed
-
AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: State-of-the-Art Review.
JACC CardioOncol2022 Nov;4(4):427-441. doi: 10.1016/j.jaccao.2022.08.009.
Wechalekar Ashutosh D, Fontana Marianna, Quarta C Cristina, Liedtke Michaela,
Abstract
Amyloid light chain (AL) amyloidosis is a rare, debilitating, often fatal disease. Symptoms of cardiomyopathy are common presenting features, and patients often are referred to cardiologists. Cardiac amyloid infiltration is the leading predictor of death. However, the variable presentation and perceived rarity of the disease frequently lead to delay in suspecting amyloidosis as a cause of heart failure, leading to misdiagnoses and a marked delay in diagnosis, with devastating consequences for the patient. A median time from symptom onset to correct diagnosis of about 2 years is often too long when median survival from diagnosis for patients with AL amyloidosis and cardiomyopathy is 4 months to 2 years. The authors highlight the challenges to diagnosis, identify gaps in the current knowledge, and summarize novel treatments on the horizon to raise awareness about the critical need for early recognition of symptoms and diagnosis of AL amyloidosis aimed at accelerating treatment and improving outcomes for patients.
© 2022 The Authors.
Guarda su PubMed
-
Role of Daratumumab in Cardiac AL Amyloidosis.
JACC CardioOncol -
Outcomes by Cardiac Stage in Patients With Newly Diagnosed AL Amyloidosis: Phase 3 ANDROMEDA Trial.
JACC CardioOncol2022 Nov;4(4):474-487. doi: 10.1016/j.jaccao.2022.08.011.
Minnema Monique C, Dispenzieri Angela, Merlini Giampaolo, Comenzo Raymond L, Kastritis Efstathios, Wechalekar Ashutosh D, Grogan Martha, Witteles Ronald, Ruberg Frederick L, Maurer Mathew S, Tran NamPhuong, Qin Xiang, Vasey Sandra Y, Weiss Brendan M, Vermeulen Jessica, Jaccard Arnaud,
Abstract
BACKGROUND:
Patients with amyloid light chain amyloidosis and severe cardiac dysfunction have a poor prognosis. Treatment options that induce rapid and deep hematologic and organ responses, irrespective of cardiac involvement, are needed.
OBJECTIVES:
The aim of this study was to evaluate the impact of baseline cardiac stage on efficacy and safety outcomes in the phase 3 ANDROMEDA trial.
METHODS:
Rates of overall complete hematologic response and cardiac and renal response at 6 months and median major organ deterioration-progression-free survival and major organ deterioration-event-free survival were compared across cardiac stages (I, II, or IIIA) and treatments (daratumumab, bortezomib, cyclophosphamide, and dexamethasone [D-VCd] or bortezomib, cyclophosphamide, and dexamethasone [VCd]). Rates of adverse events (AEs) were summarized for patients with and without baseline cardiac involvement and by cardiac stage.
RESULTS:
Median follow-up duration was 15.7 months. The proportions of stage I, II, and IIIA patients were 23.2%, 40.2%, and 36.6%. Across cardiac stages, hematologic and organ response rates were higher and major organ deterioration-progression-free survival and major organ deterioration-event-free survival were longer with D-VCd than VCd. AE rates were similar between treatments and by cardiac stage; serious AE rates were higher in patients with cardiac involvement and increased with increasing cardiac stage. The incidence of cardiac events was numerically greater with D-VCd vs VCd, but the rate of grade 3 or 4 events was similar. The exposure-adjusted incidence rate for cardiac events was lower with D-VCd than VCd (median exposure 13.4 and 5.3 months, respectively).
CONCLUSIONS:
These findings demonstrate the efficacy of D-VCd over VCd in patients with newly diagnosed amyloid light chain amyloidosis across cardiac stages, thus supporting its use in patients with cardiac involvement. (NCT03201965).
© 2022 The Authors.
Guarda su PubMed
-
Prognosis of Transthyretin Cardiac Amyloidosis Without Heart Failure Symptoms.
JACC CardioOncol2022 Nov;4(4):442-454. doi: 10.1016/j.jaccao.2022.07.007.
Gonzalez-Lopez Esther, Escobar-Lopez Luis, Obici Laura, Saturi Giulia, Bezard Mélanie, Saith Sunil E, AbouEzzeddine Omar F, Mussinelli Roberta, Gagliardi Christian, Kharoubi Mounira, Griffin Jan M, Dispenzieri Angela, Vilches Silvia, Perlini Stefano, Longhi Simone, Oghina Silvia, Rivas Adrian, Grogan Martha, Maurer Mathew S, Damy Thibaud, Palladini Giovanni, Rapezzi Claudio, Garcia-Pavia Pablo,
Abstract
BACKGROUND:
Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly recognized as a treatable cause of heart failure (HF). Advances in diagnosis and therapy have increased the number of patients diagnosed at early stages, but prognostic data on patients without HF symptoms are lacking. Moreover, it is unknown whether asymptomatic patients benefit from early initiation of transthyretin (TTR) stabilizers.
OBJECTIVES:
The aim of this study was to describe the natural history and prognosis of ATTR-CM in patients without HF symptoms.
METHODS:
Clinical characteristics and outcomes of patients with ATTR-CM without HF symptoms were retrospectively collected at 6 international amyloidosis centers.
RESULTS:
A total of 118 patients (78.8% men, median age 66 years [IQR: 53.8-75 years], 68 [57.6%] with variant transthyretin amyloidosis, mean left ventricular ejection fraction 60.5% ± 9.9%, mean left ventricular wall thickness 15.4 ± 3.1 mm, and 53 [45%] treated with TTR stabilizers at baseline or during follow-up) were included. During a median follow-up period of 3.7 years (IQR: 1-6 years), 38 patients developed HF symptoms (23 New York Heart Association functional class II and 14 functional class III or IV), 32 died, and 2 required cardiac transplantation. Additionally, 20 patients received pacemakers, 13 developed AF, and 1 had a stroke. Overall survival was 96.5% (95% CI: 91%-99%), 90.4% (95% CI: 82%-95%), and 82% (95% CI: 71%-89%) at 1, 3, and 5 years, respectively. Treatment with TTR stabilizers was associated with improved survival (HR: 0.31; 95% CI: 0.12-0.82; = 0.019) and remained significant after adjusting for sex, age, ATTR-CM type, and estimated glomerular filtration rate (HR: 0.18; 95% CI: 0.06-0.55; = 0.002).
CONCLUSIONS:
After a median follow-up period of 3.7 years, 1 in 3 patients with asymptomatic ATTR-CM developed HF symptoms, and nearly as many died or required cardiac transplantation. Treatment with TTR stabilizers was associated with improved prognosis.
© 2022 The Authors.
Guarda su PubMed
