Pubblicazioni recenti - cardiac amyloidosis

• The Clinical and Economic Burden of Newly Diagnosed Hereditary Transthyretin (ATTRv) Amyloidosis: A Retrospective Analysis of Claims Data.
  Neurol Ther

  2020 May;():. doi: 10.1007/s40120-020-00194-4.
  
  Reddy Sheila R, Chang Eunice, Tarbox Marian H, Broder Michael S, Tieu Ryan S, Guthrie Spencer, Vera-Llonch Montserrat, Pollock Michael R,
  
  Abstract
  
  INTRODUCTION:
  Little is known about the burden of hereditary transthyretin (ATTRv) amyloidosis, a genetic, progressive, and fatal disease caused by extracellular deposition of transthyretin amyloid fibrils. The study's aim was to estimate costs and disease burden associated with ATTRv amyloidosis in a real-world setting.
  
  METHODS:
  Using IBM MarketScan Commercial and Medicare Supplemental data, we identified patients at least 18 years of age with newly diagnosed ATTRv amyloidosis. Diagnosis required at least one medical claim with relevant diagnosis code (International Classification of Diseases, 9th Revision, Clinical Modification [ICD-9-CM] 277.30-.31, 277.39; ICD-10-CM E85.0-.4, E85.89, E85.9) between January 1, 2014 and December 31, 2016, and at least one additional criterion occurring during study period (2013-2017): at least 15 days diflunisal use without more than a 30-day gap; liver transplant; or claim with codes E85.1 or E85.2. First diagnosis date was study index. Continuous enrollment 1-year pre-index (baseline) and post-index (follow-up) was required. Patients with baseline amyloidosis diagnosis were excluded. Outcomes of interest were comorbidities and 1-year follow-up healthcare utilization and costs (also reported quarterly).
  
  RESULTS:
  Among 185 qualifying patients, mean age was 59.2 years (standard deviation 15.2), 54.1% were female, and baseline Charlson comorbidity index was 2.2 (2.5). Neuropathy (30.3%), diabetes (27.0%), and cardiovascular-related comorbidities, including dyspnea (25.9%) and congestive heart failure (21.6%), were common during follow-up. Nearly a quarter of patients (24.9%) were hospitalized during follow-up. Most hospitalizations and emergency department visits occurred in the first quarter post-diagnosis (18.9%, 17.8%, respectively) and dropped in subsequent quarters. The annual mean total cost was $64,066, with inpatient services contributing the majority of the expenses ($34,461), followed by outpatient ($23,853), and then pharmacy ($5752). As with utilization, costs were highest in the first quarter post-diagnosis and dropped in subsequent quarters.
  
  CONCLUSION:
  Patients newly diagnosed with ATTRv amyloidosis have substantial healthcare utilization and costs in the first year, primarily the initial months, post-diagnosis. Further research should examine later costs associated with disease progression and end-of-life care.
  
  Guarda su PubMed

• A potential pitfall in the use of Tc-PYP imaging for diagnosing cardiac ATTR amyloidosis.
  J Nucl Cardiol

  2020 May;():. doi: 10.1007/s12350-020-02199-w.
  
  Murray Christopher S G, Moadel Renee M, Tauras James M, Zamora Edgar, Travin Mark I,
  
  
  
  Guarda su PubMed

• Trends in Diagnostic Imaging of Cardiac Amyloidosis: Emerging Knowledge and Concepts.
  Radiographics

  2020 May;():190069. doi: 10.1148/rg.2020190069.
  
  Oda Seitaro, Kidoh Masafumi, Nagayama Yasunori, Takashio Seiji, Usuku Hiroki, Ueda Mitsuharu, Yamashita Taro, Ando Yukio, Tsujita Kenichi, Yamashita Yasuyuki,
  
  Abstract
  
  Cardiac amyloidosis (CA) has long been recognized as a rare disease. However, recent advances in cardiac imaging have led to increased identification of hidden CA in patients diagnosed with heart failure. This shift suggests that the actual incidence of CA is underestimated. The prognosis of CA is generally poor, especially in patients with advanced heart failure. However, recent developments in therapeutic interventions have improved the survival of patients with CA. An early diagnosis and interventions involving effective therapies are essential contributors to improved prognoses. Recent noninvasive diagnostic imaging modalities such as echocardiography, cardiac MRI, and nuclear imaging have facilitated the precise and early diagnosis of CA and enabled the initiation of appropriate management. The authors present an updated review of the clinical features of CA, including a discussion of current trends in noninvasive diagnostic imaging. RSNA, 2020.
  
  Guarda su PubMed

• Cardiac sympathetic denervation in wild-type transthyretin amyloidosis.
  Amyloid

  2020 May;():1-7. doi: 10.1080/13506129.2020.1769059.
  
  Gimelli Alessia, Aimo Alberto, Vergaro Giuseppe, Genovesi Dario, Santonato Valeria, Kusch Annette, Emdin Michele, Marzullo Paolo,
  
  Abstract
  
  Tissue accumulation of misfolded transthyretin (TTR) may occur because of gene mutations (variant amyloid TTR amyloidosis, ATTRv), or as an age-related phenomenon (wild-type ATTR, ATTRwt). Cardiac sympathetic denervation has been reported in ATTRv, but has never been investigated in ATTRwt. Fifteen consecutive patients with ATTRwt cardiomyopathy (81% men, median age 82?years, no one with prior myocardial infarction) underwent Cadmium Zinc Telluride tomographic imaging for amyloid burden (Tc-hydroxymethylene diphosphonate - Tc-HMDP), innervation (I-metaiodobenzylguanidine - I-MIBG and perfusion (Tc-tetrofosmin). Median summed Tc-HMDP score was 60 (58-62), denoting a severe and diffuse amyloid burden. Planar I-MIBG examination showed decreased early and late H/M ratios (late H/M ratio: 1.5 [1.3-1.6], range 1.2-1.9, reference value ?2.0). Summed I-MIBG score was 12 (6-22), with the most prominent denervation in the infero-septal, inferior, and infero-lateral regions; summed rest score was 7 (5-11), with lowest degrees of myocardial perfusion in the inferior and infero-septal regions. The correlation between amyloid burden (as relative Tc-HMDP uptake) and innervation (as relative I-MIBG uptake) did not achieve statistical significance at both segmental (?=?.252) and regional level (?=?.251). Nevertheless, denervation tended to worsen in parallel with the amyloid burden, and I-MIBG scores increased with Tc-HMDP scores. Segments and regions with prominent hypoperfusion also showed a higher degree of denervation (?=?0.500 and 0.591, respectively; both ?
  Guarda su PubMed

• Identification of Wild-Type Transthyretin Cardiac Amyloidosis by Quantifying Myocardial Extracellular Volume Using Cardiac Computed Tomography in Atrial Arrhythmias.
  Circ Cardiovasc Imaging

  2020 Jun;13(6):e010261. doi: 10.1161/CIRCIMAGING.119.010261.
  
  Oda Seitaro, Kidoh Masafumi, Takashio Seiji, Nishi Masato, Kanazawa Hisanori, Nagayama Yasunori, Nakaura Takeshi, Tsujita Kenichi, Ikeda Osamu,
  
  
  
  Guarda su PubMed

• F-florbetaben positron computed tomography detects cardiac involvement in systemic AA amyloidosis.
  Eur J Nucl Med Mol Imaging

  2020 May;():. doi: 10.1007/s00259-020-04861-4.
  
  Papathanasiou Maria, Carpinteiro Alexander, Hagenacker Tim, Herrmann Ken, Rassaf Tienush, Rischpler Christoph, Luedike Peter,
  
  
  
  Guarda su PubMed

• Advances in PET-Based Cardiac Amyloid Radiotracers.
  Curr Cardiol Rep

  2020 May;22(6):40. doi: 10.1007/s11886-020-01284-3.
  
  Gallegos Cesia, Miller Edward J,
  
  Abstract
  
  The gold standard for diagnosis of cardiac amyloidosis (CA) is endomyocardial biopsy showing Congo red staining followed by mass spectroscopy, but the diagnosis can also be made with high certainty by demonstration of typical cardiac imaging features along with amyloid on biopsy of another involved organ. The use of cardiac imaging techniques to detect amyloid deposits may frequently obviate the need for invasive methods in order to ascertain the presence, and potentially the type, of amyloid deposition. PURPOSE OF REVIEW: We aim to review the evidence behind the development of novel positron emission tomography (PET) radiotracers for demonstrating cardiac amyloid deposition and potentially distinguishing between light-chain (AL) or transthyretin (ATTR) cardiac amyloidosis. RECENT FINDINGS: Multiple recent studies have shown that thioflavin-analogue tracers such asF-florbetapir, F-florbetaben, F-flutemetamol, and C-labeled Pittsburg Compound-B (PiB) may be able to fulfill the unmet need of elucidating the presence of amyloid deposition in the heart. Because they bind to the beta-pleated motif of the amyloid fibril due to their similarity to the thioflavin structure, they could potentially be used to image CA (Table 1). The use of PET amyloid radiotracers shows promise; however, further data is needed to define their overall accuracy and additive value to the care of patients with suspected systemic and/or cardiac amyloidosis.
  
  Guarda su PubMed

• Analysis of Cardiac Amyloidosis Progression Using Model-Based Markers.
  Front Physiol

  2020 ;11():324. doi: 10.3389/fphys.2020.00324.
  
  Li Wenguang, Lazarus Alan, Gao Hao, Martinez-Naharro Ana, Fontana Marianna, Hawkins Philip, Biswas Swethajit, Janiczek Robert, Cox Jennifer, Berry Colin, Husmeier Dirk, Luo Xiaoyu,
  
  Abstract
  
  Deposition of amyloid in the heart can lead to cardiac dilation and impair its pumping ability. This ultimately leads to heart failure with worsening symptoms of breathlessness and fatigue due to the progressive loss of elasticity of the myocardium. Biomarkers linked to the clinical deterioration can be crucial in developing effective treatments. However, to date the progression of cardiac amyloidosis is poorly characterized. There is an urgent need to identify key predictors for disease progression and cardiac tissue function. In this proof of concept study, we estimate a group of new markers based on mathematical models of the left ventricle derived from routine clinical magnetic resonance imaging and follow-up scans from the National Amyloidosis Center at the Royal Free in London. Using mechanical modeling and statistical classification, we show that it is possible to predict disease progression. Our predictions agree with clinical assessments in a double-blind test in six out of the seven sample cases studied. Importantly, we find that multiple factors need to be used in the classification, which includes mechanical, geometrical and shape features. No single marker can yield reliable prediction given the complexity of the growth and remodeling process of diseased hearts undergoing high-dimensional shape changes. Our approach is promising in terms of clinical translation but the results presented should be interpreted with caution due to the small sample size.
  
  Copyright © 2020 Li, Lazarus, Gao, Martinez-Naharro, Fontana, Hawkins, Biswas, Janiczek, Cox, Berry, Husmeier and Luo.
  
  Guarda su PubMed

• [Clinical and diagnostic key points of left ventricular hypertrophy in adults: insights from the ANMCO Lombardy experience].
  G Ital Cardiol (Rome)

  2020 Jun;21(6):447-456. doi: 10.1714/3359.33329.
  
  Iacovoni Attilio, De Chiara Benedetta, Sormani Paola, Campana Marco, Agostini Francesco, Faggiano Pompilio, Occhi Lucia, Dadone Viola, Raineri Claudia, Moreo Antonella, Di Tano Giuseppe, ,
  
  Abstract
  
  Left ventricular hypertrophy is a common complication of different diseases. Among these, cardiac involvement of amyloidosis or Anderson-Fabry disease are often unrecognized. Early diagnosis is therefore crucial because new therapies can impact the progression of these diseases. Different specific signs unmasked by clinical, laboratory, and non-invasive diagnostic tests such as echocardiography or cardiac magnetic resonance could guide clinicians towards an appropriate diagnosis. The aim of this review is to underline the major diagnostic clues of different forms of left ventricular hypertrophy in adult patients, guiding clinicians towards a more appropriate diagnosis.
  
  Guarda su PubMed

• Severe diffuse alveolar hemorrhage related to autoimmune disease: a multicenter study.
  Crit Care

  2020 May;24(1):231. doi: 10.1186/s13054-020-02936-0.
  
  Mirouse Adrien, Parrot Antoine, Audigier Vincent, Demoule Alexandre, Mayaux Julien, Géri Guillaume, Mariotte Eric, Bréchot Nicolas, de Prost Nicolas, Vautier Mathieu, Neuville Mathilde, Bigé Naïke, de Montmollin Etienne, Cacoub Patrice, Resche-Rigon Matthieu, Cadranel Jacques, Saadoun David,
  
  Abstract
  
  BACKGROUND:
  Diffuse alveolar hemorrhage (DAH) occurs during the course of autoimmune disease and may be life threatening. The objective was to assess characteristics and prognosis factors of DAH who required intensive care unit (ICU) admission in patients with autoimmune diseases.
  
  METHODS:
  French multicenter retrospective study including patients presenting DAH related to autoimmune diseases requiring ICU admission from 2000 to 2016.
  
  RESULTS:
  One hundred four patients (54% of men) with median age of 56 [32-68] years were included with 79 (76%) systemic vasculitis and 25 (24%) connective tissue disorders. All patients received steroids, and 72 (69%), 12 (11.5%), and 57 (55%) patients had cyclophosphamide, rituximab, and plasma exchanges, respectively. During ICU stay, 52 (50%), 36 (35%), and 55 (53%) patients required mechanical ventilation, vasopressor use, and renal replacement therapy, respectively. Factors associated with mechanical ventilation weaning were age (HR [95%CI] 0.97 [0.96-0.99] per 10?years, p?
  CONCLUSION:
  DAH in autoimmune diseases is a life-threatening complication which requires mechanical ventilation in half of the cases admitted to ICU.
  
  Guarda su PubMed

• The prognostic value of right ventricular deformation derived from cardiac magnetic resonance tissue tracking for all-cause mortality in light-chain amyloidosis patients.
  Cardiovasc Diagn Ther

  2020 Apr;10(2):161-172. doi: 10.21037/cdt.2020.01.03.
  
  Liu Hui, Fu Hang, Guo Ying-Kun, Yang Zhi-Gang, Xu Hua-Yan, Shuai Xiao, Xu Rong, Li Zhen-Lin, Xia Chun-Chao, He Yong, Zhou Xiao-Yue,
  
  Abstract
  
  Background:
  Early detection of right ventricular (RV) dysfunction is vital for determining the prognosis of light-chain amyloidosis (AL) patients. While few studies focused on RV deformation due to the limitation of research methods. The aim of this study was to determine the prognostic significance of RV myocardial strain in AL patients assessed by cardiac magnetic resonance (CMR) tissue tracking.
  
  Methods:
  Sixty-four AL patients (28 females and 36 males, mean age 58±12.8 years old; range 25-81 years old) were enrolled from 1 October 2014 through 31 March 2017 and compared with 20 age- and sex-matched controls. Fifty-one AL patients met the criteria for cardiac amyloidosis (CA). Deformation parameters of both RV and left ventricle (LV) were measured by the CMR tissue tracking technique including myocardial global radial peak strain (GRPS), global circumferential peak strain (GCPS), and global longitudinal peak strain (GLPS). The follow-up time was 20 months or until the occurrence of death.
  
  Results:
  Thirty-two (50%) had preserved RV ejection fraction (RVEF ?45%). AL patients had significantly lower RV-GRPS (20.3±2.12 31.31±7.61), GCPS (-2.12±0.88 -13.71±2.53), and GLPS (-5.33±0.64 -14.239±2.99) than controls even RVEF remain preserved (all P<0.001). Compared with controls and patients without CA, RV-GRPS (12.26±1.26 29.72±3.54, P<0.001) and RV-GLPS (-3.78±2.25 -5.66±2.08, P<0.05) were significantly lower in patients with CA. Cox multivariate analyses demonstrated that RV-GRPS [hazard ratio (HR) =0.93, 95% CI: 0.88-0.98, P=0.007] and Mayo stage were (HR =3.11, 95% CI: 1.30-7.41, P=0.01) predictors of mortality in AL patients.
  
  Conclusions:
  CMR tissue tracking is a feasible and highly reproducible technique for the analysis of RV deformation and could aid in the early diagnosis of RV involvement in AL patients. RV-GRPS of RV strain and Mayo stage provides prognostic information about mortality in AL patients.
  
  2020 Cardiovascular Diagnosis and Therapy. All rights reserved.
  
  Guarda su PubMed

• Improved Quantification of Cardiac Amyloid Burden in Systemic Light Chain Amyloidosis: Redefining Early Disease?
  JACC Cardiovasc Imaging

  2020 May;():. doi: S1936-878X(20)30251-5.
  
  Cuddy Sarah A M, Bravo Paco E, Falk Rodney H, El-Sady Samir, Kijewski Marie Foley, Park Mi-Ae, Ruberg Frederick L, Sanchorawala Vaishali, Landau Heather, Yee Andrew J, Bianchi Giada, Di Carli Marcelo F, Cheng Su-Chun, Jerosch-Herold Michael, Kwong Raymond Y, Liao Ronglih, Dorbala Sharmila,
  
  Abstract
  
  OBJECTIVES:
  The purpose of this study was to determine phenotypes characterizing cardiac involvement in AL amyloidosis by using direct (fluorine-18-labeled florbetapir {[F]florbetapir} positron emission tomography [PET]/computed tomography) and indirect (echocardiography and cardiac magnetic resonance [CMR]) imaging biomarkers of AL amyloidosis.
  
  BACKGROUND:
  Cardiac involvement in systemic light chain amyloidosis (AL) is the main determinant of prognosis and, therefore, guides management. The hypothesis of this study was that myocardial AL deposits and expansion of extracellular volume (ECV) could be identified before increases in N-terminal pro-B-type natriuretic peptide or wall thickness.
  
  METHODS:
  A total of 45 subjects were prospectively enrolled in 3 groups: 25 with active AL amyloidosis with cardiac involvement (active-CA), 10 with active AL amyloidosis without cardiac involvement by conventional criteria (active-non-CA), and 10 with AL amyloidosis with cardiac involvement in remission for at least 1 year (remission-CA). All subjects underwent echocardiography, CMR, and [F]florbetapir PET/CT to evaluate cardiac amyloid burden.
  
  RESULTS:
  The active-CA group demonstrated the largest myocardial AL amyloid burden, quantified by [F]florbetapir retention index (RI) 0.110 (interquartile range [IQR]: 0.078 to 0.139) min, and the lowest cardiac function by global longitudinal strain (GLS), median GLS -11% (IQR: -8% to -13%). The remission-CA group had expanded extracellular volume (ECV) and [F]florbetapir RI of 0.097 (IQR: 0.070, 0.124 min), and abnormal GLS despite hematologic remission for >1 year. The active-non-CA cohort had evidence of cardiac amyloid deposition by advanced imaging metrics in 50% of the subjects; cardiac involvement was identified by late gadolinium enhancement in 20%, elevated ECV in 20%, and elevated [F]florbetapir RI in 50%.
  
  CONCLUSIONS:
  Evidence of cardiac amyloid infiltration was found based on direct and indirect imaging biomarkers in subjects without CA by conventional criteria. The findings from [F]florbetapir PET imaging provided insight into the preclinical disease process and on the basis of interpretation of expanded ECV on CMR and have important implications for future research and clinical management of AL amyloidosis. (Molecular Imaging of Primary Amyloid Cardiomyopathy [MICA]; NCT02641145).
  
  Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
  
  Guarda su PubMed

• Molecular Imaging of Amyloid Deposits for Early Diagnosis of Cardiac Amyloidosis and Monitoring Treatment Response.
  JACC Cardiovasc Imaging

  2020 May;():. doi: S1936-878X(20)30252-7.
  
  Chen Wengen, Dilsizian Vasken,
  
  
  
  Guarda su PubMed

• Diagnostic Accuracy of [C]PIB Positron Emission Tomography for Detection of Cardiac Amyloidosis.
  JACC Cardiovasc Imaging

  2020 May;():. doi: S1936-878X(20)30247-3.
  
  Rosengren Sara, Skibsted Clemmensen Tor, Tolbod Lars, Granstam Sven-Olof, Eiskjær Hans, Wikström Gerhard, Vedin Ola, Kero Tanja, Lubberink Mark, Harms Hendrik J, Flachskampf Frank A, Baron Tomasz, Carlson Kristina, Mikkelsen Fabian, Antoni Gunnar, Frost Andersen Niels, Hvitfeldt Poulsen Steen, Sörensen Jens,
  
  Abstract
  
  OBJECTIVES:
  This dual-site study evaluated the diagnostic accuracy of the method.
  
  BACKGROUND:
  Pittsburgh compound ([C]PIB) positron emission tomography (PIB-PET) has shown promise as a specific and noninvasive method for the diagnosis of cardiac amyloidosis (CA).
  
  METHODS:
  The study had 2 parts. In the initial study, 51 subjects were included, 36 patients with known CA and increased wall thickness (15 immunoglobulin light chain [AL] and 21 transthyretin [ATTR] amyloidosis) and 15 control patients (7 were nonamyloid hypertrophic and 8 healthy volunteers). Subjects underwent PIB-PET and echocardiography. Sensitivity and specificity of PIB-PET were established for 2 simple semiquantitative approaches, standardized uptake value ratio (SUVR) and retention index (RI). The second part of the study included 11 amyloidosis patients (5 AL and 6 hereditary ATTR) without increased wall thickness to which the optimal cutoff values of SUVR (>1.09) and RI (>0.037 min) were applied prospectively.
  
  RESULTS:
  The diagnostic accuracy of visual inspection of [C]PIB uptake was 100% in discriminating CA patients with increased wall thickness from controls. Semiquantitative [C]PIB uptake discriminated CA from controls with a 94% (95% confidence interval [CI]: 80% to 99%) sensitivity for both SUVR and RI and specificity of 93% (95% CI: 66% to 100%) for SUVR and 100% (95% CI: 75% to 100%) for RI. [C]PIB uptake was significantly higher in AL-CA than in ATTR-CA patients (p < 0.001) and discriminated AL-CA from controls with 100% (95% CI: 88% to 100%) accuracy for both the semiquantitative measures. In the prospective group without increased wall thickness, RI was elevated compared to controls (p = 0.001) and 5 of 11 subjects were evaluated as [C]PIB PET positive.
  
  CONCLUSIONS:
  In a dual-center setting, [C]PIB PET was highly accurate in detecting cardiac involvement in the main amyloid subtypes, with 100% accuracy in AL amyloidosis. A proportion of amyloidosis patients without known cardiac involvement were [C]PIB PET positive, indicating that the method may detect early stages of CA.
  
  Copyright © 2020 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
  
  Guarda su PubMed

• Technetium pyrophosphate nuclear scintigraphy for cardiac amyloidosis: Imaging at 1 vs 3 hours and planar vs SPECT/CT.
  J Nucl Cardiol

  2020 May;():. doi: 10.1007/s12350-020-02139-8.
  
  Sperry Brett W, Burgett Eric, Bybee Kevin A, McGhie A Iain, O'Keefe James H, Saeed Ibrahim M, Thompson Randall C, Bateman Timothy M,
  
  Abstract
  
  BACKGROUND:
  Multi-societal consensus recommendations endorse both planar and single photon emission tomographic (SPECT) image acquisitions for the evaluation of cardiac amyloidosis. However, the correlation between planar and SPECT findings and the optimal timing of image acquisitions remain uncertain.
  
  METHODS:
  This is an analysis of 109 consecutive patients who underwent technetium pyrophosphate nuclear scintigraphy for the evaluation of cardiac amyloidosis. Patients were imaged at 1 and 3 hours after radiotracer injection using both planar and SPECT/CT, and the correlations between imaging protocols were compared.
  
  RESULTS:
  In the overall cohort (median age 77 years, 75% male), 33 patients had radiotracer localized to the myocardium on SPECT/CT images. There was strong correlation between 1- and 3-hour planar heart-to-contralateral lung ratios (mean difference 0.07, r?=?0.94). However, there was discordance between planar image interpretation (based upon semiquantitative score and H/CL ratio) and myocardial localization of radiotracer on SPECT/CT in 17 patients (16%). The pattern of SPECT/CT uptake was identical at 1 and 3 hours in all cases (32 diffuse, 1 focal).
  
  CONCLUSION:
  These data support the recommendation that SPECT/CT should be obtained in addition to planar images when performing nuclear scintigraphy for the detection of cardiac amyloidosis. A 1-hour planar and SPECT/CT protocol appears optimal.
  
  Guarda su PubMed

• Phenotyping of Cardiac Amyloidosis: Advancing From Macro to Micro?
  Circ Cardiovasc Imaging

  2020 May;13(5):e010785. doi: 10.1161/CIRCIMAGING.120.010785.
  
  Cuddy Sarah, Jerosch-Herold Michael, Dorbala Sharmila,
  
  
  
  Guarda su PubMed

• Diffusion Tensor Cardiovascular Magnetic Resonance in Cardiac Amyloidosis.
  Circ Cardiovasc Imaging

  2020 May;13(5):e009901. doi: 10.1161/CIRCIMAGING.119.009901.
  
  Khalique Zohya, Ferreira Pedro F, Scott Andrew D, Nielles-Vallespin Sonia, Martinez-Naharro Ana, Fontana Marianna, Hawkins Phillip, Firmin David N, Pennell Dudley J,
  
  Abstract
  
  Background Cardiac amyloidosis (CA) is a disease of interstitial myocardial infiltration, usually by light chains or transthyretin. We used diffusion tensor cardiovascular magnetic resonance (DT-CMR) to noninvasively assess the effects of amyloid infiltration on the cardiac microstructure. Methods DT-CMR was performed at diastole and systole in 20 CA, 11 hypertrophic cardiomyopathy, and 10 control subjects with calculation of mean diffusivity, fractional anisotropy, and sheetlet orientation (secondary eigenvector angle). Results Mean diffusivity was elevated and fractional anisotropy reduced in CA compared with both controls and hypertrophic cardiomyopathy (<0.001). In CA, mean diffusivity was correlated with extracellular volume (=0.68, =0.004), and fractional anisotropy was inversely correlated with circumferential strain (=-0.65, =0.02). In CA, diastolic secondary eigenvector angle was elevated, and secondary eigenvector angle mobility was reduced compared with controls (both <0.001). Diastolic secondary eigenvector angle was correlated with amyloid burden measured by extracellular volume in transthyretin, but not light chain amyloidosis. Conclusions DT-CMR can characterize the microstructural effects of amyloid infiltration and is a contrast-free method to identify the location and extent of the expanded disorganized myocardium. The diffusion biomarkers mean diffusivity and fractional anisotropy effectively discriminate CA from hypertrophic cardiomyopathy. DT-CMR demonstrated that failure of sheetlet relaxation in diastole correlated with extracellular volume in transthyretin, but not light chain amyloidosis. This indicates that different mechanisms may be responsible for impaired contractility in CA, with an amyloid burden effect in transthyretin, but an idiosyncratic effect in light chain amyloidosis. Consequently, DT-CMR offers a contrast-free tool to identify novel pathophysiology, improve diagnostics, and monitor disease through noninvasive microstructural assessment.
  
  Guarda su PubMed

• Rapid response to single agent daratumumab is associated with improved progression-free survival in relapsed/refractory AL amyloidosis.
  Amyloid

  2020 May;():1-6. doi: 10.1080/13506129.2020.1765768.
  
  Cohen Oliver C, Brodermann Maximillian H, Blakeney Iona J, Mahmood Shameem, Sachchithanantham Sajitha, Ravichandran Sriram, Law Steven, Lachmann Helen J, Whelan Carol J, Popat Rakesh, Rabin Neil, Yong Kwee, Kyriakou Charalampia, Shah Raakhee, Cheesman Simon, Worthington Sarah, Hawkins Philip, Gillmore Julian D, Wechalekar Ashutosh D,
  
  Abstract
  
  Daratumumab is a monoclonal antibody, which targets CD38; an antigen expressed on malignant plasma cells in AL amyloidosis thus providing a rationale for its use. Patients treated with daratumumab monotherapy (2016-2019) for relapsed/refractory systemic AL amyloidosis were identified from the database at the UK National Amyloidosis Centre. Of 50 evaluable patients, haematological responses at 3?months were: CR - 19 (38%), VGPR - 14 (28%), PR - 9 (18%) and no response - 8 (16%). Median time to response was 1 (1-6) month. Of assessable patients, cardiac, renal and hepatic responses were seen in 43.8%, 25.0% and 0% of patients whilst progression occurred in 25.0%, 12.5% and 37.5% respectively. Patients achieving a CR had longer median OS (not reached vs. 22.7?months [95% CI 17.0-28.4?months]) (?=?.036). Furthermore, patients achieving a rapid response (at 1?month) had a longer median PFS (not reached vs. 9?months [95% CI 5.8-12.2?months]) (?=?.013). Daratumumab monotherapy is effective in multiply-relapsed systemic AL amyloidosis and should be considered, if available, in patients who have not received prior daratumumab therapy. Responses are achieved rapidly and overall response rate was 84%. CR predicts overall survival whilst speed of response is predictive of a longer PFS.
  
  Guarda su PubMed

• Cardiac Care of Patients with Cardiac Amyloidosis.
  Acta Haematol

  2020 May;():1-9. doi: 10.1159/000506919.
  
  Itzhaki Ben Zadok Osnat, Kornowski Ran,
  
  Abstract
  
  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, emphasizing the importance of multidisciplinary care.
  
  © 2020 S. Karger AG, Basel.
  
  Guarda su PubMed

• Quantification of cardiac amyloid with [F]Flutemetamol in patients with V30M hereditary transthyretin amyloidosis.
  Amyloid

  2020 May;():1-9. doi: 10.1080/13506129.2020.1760237.
  
  Möckelind Sofia, Axelsson Jan, Pilebro Björn, Lindqvist Per, Suhr Ole B, Sundström Torbjörn,
  
  Abstract
  
  Hereditary transthyretin amyloid (ATTRv) is a systemic amyloidosis with mainly neurological and cardiac symptoms. The aim of this study was to evaluate the outcome of [F]Flutemetamol PET/CT-scan of the heart in long-term survivors with ATTRV30M amyloidosis. Twenty-one patients with ATTRV30M amyloidosis and predominantly neurological symptoms, mainly negative on cardiac technetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD)-scintigraphy, were examined with a dynamic [F]Flutemetamol PET/CT-scan. Five patients suffering from Alzheimer's disease and one healthy individual served as controls. Volumes of interests were drawn over the intraventricular septum, lateral wall of the left ventricle and free wall of the right ventricle. Clinical records were reviewed for data from previous completed DPD-scintigraphy of the heart and echocardiography. Patients with ATTRv amyloidosis had a higher cardiac uptake than the control-group in all analysed regions of the heart and could be identified with high accuracy (sensitivity 88%, specificity 100%) in static image acquisition at 30 or 60?min. We found no correlation between cardiac [F]Flutemetamol uptake and clinical variables. In this small study of selected patients, cardiac [F]Flutemetamol PET/CT could differentiate between healthy individuals and patients with ATTRV30M. [F]Flutemetamol PET/CT imaging of amyloidosis in patients with a negative DPD-scintigraphy has a potential as a diagnostic method.
  
  Guarda su PubMed

Guarda l'elenco completo su PubMed