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Pubblicazioni recenti - cardiac amyloidosis
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Diagnosis of transthyretin cardiac amyloidosis with cadmium zinc telluride cameras: Is it feasible?
J Nucl Cardiol -
Autonomic dysfunction in cardiac amyloidosis assessed by heart rate variability and heart rate turbulence.
Ann Noninvasive Electrocardiol2020 Feb;():e12749. doi: 10.1111/anec.12749.
Yamada Shinya, Yoshihisa Akiomi, Hijioka Naoko, Kamioka Masashi, Kaneshiro Takashi, Yokokawa Tetsuro, Misaka Tomofumi, Ishida Takafumi, Takeishi Yasuchika,
Abstract
BACKGROUND:
Cardiac amyloidosis (CA) is characterized by left ventricular hypertrophy (LVH) and autonomic nervous imbalance due to amyloid infiltration. However, autonomic dysfunction is often seen in heart failure (HF) with LVH from other etiologies. We aimed to characterize autonomic dysfunction in CA from other etiologies of LVH.
METHODS:
Fifty-five HF patients with LVH (35 males, mean age 65 ± 16 years) were enrolled. LVH was defined as left ventricular mass index measured by echocardiography >95 g/m in women and 115 g/m in men. The etiology was as follows: amyloid light chain (AL)-CA, n = 14; hypertrophic cardiomyopathy, n = 21; and aortic stenosis (AS), n = 20. With the patient in a clinically stable condition, heart rate variability (HRV) and heart rate turbulence (HRT), which reflect autonomic dysfunction, were measured using Holter monitoring and compared among the three groups.
RESULTS:
Brain natriuretic peptide levels, LVH severity, left ventricular ejection fraction, and tissue Doppler index E/e' did not differ among the three groups. However, severe abnormalities of HRV and HRT were obtained in AL-CA. In the ROC analysis to identify AL-CA in HF with LVH, the best cutoff value for standard deviation of all R-R intervals, standard deviation of the 5-min mean R-R intervals, turbulence onset, and turbulence slope were 68.5 ms (AUC: 0.865), 58.5 ms (AUC: 0.834), 0.25% (AUC: 0.813), and 1.00 ms/RR (AUC 0.736), respectively.
CONCLUSION:
Autonomic dysfunction is a hallmark of AL-CA, and its noninvasive assessment by Holter monitoring may be a useful tool for differential diagnosis of HF with LVH.
© 2020 The Authors. Annals of Noninvasive Electrocardiology published by Wiley Periodicals, Inc.
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Diphosphonate single-photon emission computed tomography in cardiac transthyretin amyloidosis.
Int J Cardiol2020 Feb;():. doi: S0167-5273(19)35840-1.
Grigoratos Chrysanthos, Aimo Alberto, Rapezzi Claudio, Genovesi Dario, Barison Andrea, Aquaro Giovanni Donato, Vergaro Giuseppe, Pucci Angela, Passino Claudio, Marzullo Paolo, Gimelli Alessia, Emdin Michele,
Abstract
BACKGROUND:
Planar diphosphonate scintigraphy is an established diagnostic tool for amyloid transthyretin (ATTR) cardiomyopathy. Characterization of the amyloid burden up to the segmental level by single photon emission computed tomography (SPECT) has not been evaluated so far.
METHODS:
Data from consecutive patients undergoing cardiac Tc-hydroxymethylene diphosphonate (Tc-HMDP) SPECT and diagnosed with ATTR cardiomyopathy at a tertiary referral center from June 2016 to April 2019 were collected.
RESULTS:
Thirty-eight patients were included (median age 81 years, 79% men, 92% with wild-type ATTR). In patients with Perugini score 1, the most intense diphosphonate regional uptake was found in septal segments, particularly in infero-septal segments. Among patients scoring 2, the amyloid burden in the septum became more significant, and extended to inferior and apical segments. Finally, patients scoring 3 displayed an intense and widespread tracer uptake. All patients with Perugini score 1 had LGE in at least one antero-septal, one infero-septal, and one infero-lateral segment. All patients with score 2 displayed LGE in infero-septal, inferior, and infero-lateral segments. LGE became extensive in patients scoring 3, with all patients having at least one LGE-positive segment in each region.
CONCLUSIONS:
When assimilating different Perugini grades to evolutive stages of the disease, amyloid deposition seem to progress from the septum to the inferior wall and then to the other regions and from the basis to the apex. The potential of segmental analysis might be particularly relevant in patients with very limited cardiac uptake at planar scintigraphy (Perugini score 1).
Copyright © 2020. Published by Elsevier B.V.
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A case report on transient global ventricular wall thickening secondary to acute myocarditis: Focus on the unique role of cardiac MRI.
Medicine (Baltimore)2020 Feb;99(8):e19223. doi: 10.1097/MD.0000000000019223.
Xu Tiantian, Hu Hongjie,
Abstract
INTRODUCTION:
Transient left ventricular wall thickening is known to develop in the acute phase of myocarditis, with several reports documenting this unusual mode of myocarditis. Diagnosing myocarditis can be challenging because symptoms, clinical exam findings, electrocardiogram results, biomarkers, and echocardiogram results are often non-specific. Therefore, cardiac magnetic resonance imaging has become the primary non-invasive imaging tool in patients with suspected myocarditis.
PATIENT CONCERNS AND DIAGNOSIS:
A 51-year-old male was referred to our hospital with a 20-day history of fever. Initial echocardiogram demonstrated diffuse concentric left ventricular hypertrophy with depressed left ventricular diastolic function, previously misdiagnosed as restrictive cardiomyopathy. Cardiac magnetic resonance imaging (MRI) showed global ventricular wall thickening, and the negative delayed enhancement made hypertrophic cardiomyopathy and myocardial amyloidosis less likely. This information, along with laboratory analyses, led to a diagnosis of acute myocarditis.
INTERVENTIONS AND OUTCOMES:
The patient underwent a treatment regimen, including a prescription of levofloxacin and other supporting treatments. During the period following, the patient experienced a few minor episodes of atypical chest pain with spontaneous remission. The patient was discharged after 8 days of hospitalization. A cardiac MRI evaluation was repeated after 17 months, this time showing that the wall thickness had returned to normal; the myocarditis resolved without sequela.
CONCLUSIONS:
In summary, we report on a case of transient global ventricular wall thickening secondary to acute myocarditis, which rarely has been described previously. Our study demonstrates that transient ventricular wall thickening related to myocardial interstitial edema also can involve the right ventricular wall, a fact that is important in diagnosis and differential diagnosis. Cardiovascular magnetic resonance currently is considered the most comprehensive and accurate diagnostic tool in patients with suspected myocarditis.
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Cost-Effectiveness of Tafamidis Therapy for Transthyretin Amyloid Cardiomyopathy.
Circulation2020 Feb;():. doi: 10.1161/CIRCULATIONAHA.119.045093.
Kazi Dhruv S, Bellows Brandon K, Baron Suzanne J, Shen Changyu, Cohen David J, Spertus John A, Yeh Robert W, Arnold Suzanne V, Sperry Brett W, Maurer Mathew S, Shah Sanjiv J,
Abstract
In patients with transthyretin amyloid cardiomyopathy (ATTR-CM), tafamidis reduces all-cause mortality and cardiovascular hospitalizations, and slows decline in quality-oflife compared with placebo. In May 2019, tafamidis received expedited approval from the US FDA as a breakthrough drug for a rare disease. However, at $225,000 per year, it is the most expensive cardiovascular drug ever launched in the US, and its long-term cost-effectiveness and budget impact are uncertain. We therefore sought to estimate the cost-effectiveness of tafamidis and its potential effect on US health care spending. We developed a Markov model of patients with wild-type or variant ATTR-CM and heart failure (mean age 74.5 years) using inputs from the Transthyretin Amyloidosis Cardiomyopathy Clinical Trial (ATTR-ACT), published literature, US Food and Drug Administration review documents, healthcare claims, and national survey data. We compared no disease-specific treatment ("usual care") with tafamidis therapy. The model reproduced 30-month survival, quality-of-life, and cardiovascular hospitalization rates observed in ATTR-ACT; future projections used a parametric survival model in the control arm, with constant hazards reduction in the tafamidis arm. We discounted future costs and quality-adjusted life years (QALYs) by 3% annually, and examined key parameter uncertainty using deterministic and probabilistic sensitivity analyses. The main outcomes were lifetime incremental costeffectiveness ratio (ICER) and annual budget impact, assessed from the US healthcare sector perspective. This study was independent of the ATTR-ACT trial sponsor. Compared with usual care, tafamidis was projected to add 1.29 (95% uncertainty interval, 0.47-1.75) QALYs at an incremental cost of $1,135,000 (872,000-1,377,000), resulting in an ICER of $880,000 (697,000-1,564,000) per QALY gained. Assuming a threshold of $100,000 per QALY gained and current drug price, tafamidis was cost-effective in 0% of 10,000 probabilistic simulations. A 92.6% price reduction from $225,000 to $16,563 would be necessary to make tafamidis cost-effective at $100,000/QALY. Results were sensitive to assumptions related to long-term effectiveness of tafamidis. Treating all eligible patients with ATTR-CM in the US with tafamidis (n = 120,000) was estimated to increase annual healthcare spending by $32.3 billion. Treatment with tafamidis is projected to produce substantial clinical benefit but would greatly exceed conventional cost-effectiveness thresholds at the current US list price. Based on recent US experience with high-cost cardiovascular medications, access to and uptake of this effective therapy may be limited unless there is a large reduction in drug costs.
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Light-chain and transthyretin cardiac amyloidosis in severe aortic stenosis: prevalence, screening possibilities, and outcome.
Eur J Heart Fail2020 Feb;():. doi: 10.1002/ejhf.1756.
Nitsche Christian, Aschauer Stefan, Kammerlander Andreas A, Schneider Matthias, Poschner Thomas, Duca Franz, Binder Christina, Koschutnik Matthias, Stiftinger Julian, Goliasch Georg, Siller-Matula Jolanta, Winter Max-Paul, Anvari-Pirsch Anahit, Andreas Martin, Geppert Alexander, Beitzke Dietrich, Loewe Christian, Hacker Marcus, Agis Hermine, Kain Renate, Lang Irene, Bonderman Diana, Hengstenberg Christian, Mascherbauer Julia,
Abstract
AIMS:
Concomitant cardiac amyloidosis (CA) in severe aortic stenosis (AS) is difficult to recognize, since both conditions are associated with concentric left ventricular thickening. We aimed to assess type, frequency, screening parameters, and prognostic implications of CA in AS.
METHODS AND RESULTS:
A total of 191 consecutive AS patients (81.2?±?7.4?years; 50.3% female) scheduled for transcatheter aortic valve replacement (TAVR) were prospectively enrolled. Overall, 81.7% underwent complete assessment including echocardiography with strain analysis, electrocardiography (ECG), cardiac magnetic resonance imaging (CMR), Tc-DPD scintigraphy, serum and urine free light chain measurement, and myocardial biopsy in immunoglobulin light chain (AL)-CA. Voltage/mass ratio (VMR; Sokolow-Lyon index on ECG/left ventricular mass index) and stroke volume index (SVi) were tested as screening parameters. Receiver operating characteristic curve, binary logistic regression, and Kaplan-Meier curve analyses were performed. CA was found in 8.4% of patients (n = 16); 15 had transthyretin (TTR)-CA and one AL-CA. While global longitudinal strain by echo did not reliably differentiate AS from CA-AS [area under the curve (AUC) 0.643], VMR as well as SVi showed good discriminative power (AUC 0.770 and 0.773, respectively), which was comparable to extracellular volume by CMR (AUC 0.756). Also, VMR and SVi were independently associated with CA by multivariate logistic regression analysis (P = 0.016 and P = 0.027, respectively). CA did not significantly affect survival 15.3?±?7.9 months after TAVR (P = 0.972).
CONCLUSION:
Both TTR- and AL-CA can accompany severe AS. Parameters solely based on ECG and echocardiography allow for the identification of the majority of CA-AS. In the present cohort, CA did not significantly worsen prognosis 15.3 months after TAVR.
© 2020 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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Hot hearts on bone scintigraphy are not all amyloidosis: hydroxychloroquine-induced restrictive cardiomyopathy.
Eur Heart J2020 Feb;():. doi: ehaa091.
Layoun Michael E, Desmarais Julianna, Heitner Stephen B, Masri Ahmad,
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Refining the role of carpal tunnel syndrome in cardiac amyloidosis.
Eur J Heart Fail -
Diagnostic amyloid proteomics: experience of the UK National Amyloidosis Centre.
Clin Chem Lab Med2020 Feb;():. doi: 10.1515/cclm-2019-1007.
Canetti Diana, Rendell Nigel B, Gilbertson Janet A, Botcher Nicola, Nocerino Paola, Blanco Angel, Di Vagno Lucia, Rowczenio Dorota, Verona Guglielmo, Mangione P Patrizia, Bellotti Vittorio, Hawkins Philip N, Gillmore Julian D, Taylor Graham W,
Abstract
Systemic amyloidosis is a serious disease which is caused when normal circulating proteins misfold and aggregate extracellularly as insoluble fibrillary deposits throughout the body. This commonly results in cardiac, renal and neurological damage. The tissue target, progression and outcome of the disease depends on the type of protein forming the fibril deposit, and its correct identification is central to determining therapy. Proteomics is now used routinely in our centre to type amyloid; over the past 7 years we have examined over 2000 clinical samples. Proteomics results are linked directly to our patient database using a simple algorithm to automatically highlight the most likely amyloidogenic protein. Whilst the approach has proved very successful, we have encountered a number of challenges, including poor sample recovery, limited enzymatic digestion, the presence of multiple amyloidogenic proteins and the identification of pathogenic variants. Our proteomics procedures and approaches to resolving difficult issues are outlined.
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Efficacy of Chemotherapies and Stem Cell Transplantation for Systemic AL Amyloidosis: A Network Meta-Analysis.
Front Pharmacol2019 ;10():1601. doi: 10.3389/fphar.2019.01601.
Cai Yuwen, Xu Shizhang, Li Na, Li Song, Xu Gaosi,
Abstract
Background/Aims:
The present Bayesian network meta-analysis (NMA) was to compare the efficacy of different chemotherapies and autologous stem cell transplantation (ASCT) in immunoglobulin light-chain (AL) amyloidosis.
Methods:
We systematically searched PubMed, Embase, Web of Science, and the Cochrane Central Register of Controlled Trials (CENTRAL) for studies compared the rates of hematological response (HR), complete response (CR), renal response, and cardiac response in AL amyloidosis patients.
Results:
There were three randomized controlled trials (RCTs) and thirteen observational controlled trials (OCTs) comprising 3,402 participants enrolled for the comparisons of seven treatments: melphalan + dexamethasone (MDex), high-dose melphalan followed by ASCT, bortezomib + melphalan + dexamethasone (BMDex), thalidomide + cyclophosphamide + dexamethasone (CTD), bortezomib + dexamethasone (BDex), bortezomib + cyclophosphamide + dexamethasone (CyBorD), cyclophosphamide + lenalidomide + dexamethasone (CLD). BMDex was ranked first in the aspect of both HR and CR, CTD induced the highest rate of renal response, and BDex was possibly the best treatment for the cardiac response.
Conclusion:
Although more data about safety and cost are needed, BMDex was recommended as the most efficient treatment for AL amyloidosis patients for enhancing the response rate for HR and CR.
Copyright © 2020 Cai, Xu, Li, Li and Xu.
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Efficient 1-Hour Technetium-99 m Pyrophosphate Imaging Protocol for the Diagnosis of Transthyretin Cardiac Amyloidosis.
Circ Cardiovasc Imaging2020 Feb;13(2):e010249. doi: 10.1161/CIRCIMAGING.119.010249.
Masri Ahmad, Bukhari Syed, Ahmad Shahzad, Nieves Ricardo, Eisele Yvonne S, Follansbee William, Brownell Amy, Wong Timothy C, Schelbert Erik, Soman Prem,
Abstract
BACKGROUND:
Technetium-99 m pyrophosphate protocols for transthyretin cardiac amyloidosis diagnosis have variably used 1- and 3-hour imaging time points. We investigated whether imaging at 1 hour with superior efficiency had comparable diagnostic accuracy as 3-hour imaging.
METHODS:
This is a registry analysis of patients with suspected transthyretin cardiac amyloidosis referred for technetium-99 m pyrophosphate at a single tertiary center from June 2015 through January 2019. Patients underwent planar and single-photon emission computed tomography (SPECT) imaging at 1 and 3 hours. A positive Tc-99m pyrophosphate study was defined by the presence of diffuse myocardial tracer uptake on SPECT. For planar imaging, visual semiquantitative (grades 0-3, ?2 considered positive) and quantitative heart to contralateral ratios (?1.5 considered positive) were used.
RESULTS:
Two hundred thirty-three patients (69% men; median age, 77 [69-83] years) underwent the study protocol. There were 60 (25.8%) patients with diffuse myocardial uptake, 1 (0.4%) with regional uptake, and 172 (73.8%) with no myocardial uptake. Results of SPECT were identical at 1 and 3 hours. Planar imaging at 1 hour had 98% sensitivity and 96% specificity. Planar grade 0 uptake or heart to contralateral ratio ?1.2 and planar grade 3 uptake or heart to contralateral ratio ?2.0 were always associated with negative and positive SPECT, respectively. For planar grades 1 and 2 uptake and heart to contralateral ratio 1.3 to 1.9, SPECT was needed to make a diagnosis. No patient with light-chain cardiac amyloidosis had positive SPECT.
CONCLUSIONS:
An efficient 1-hour technetium-99 m pyrophosphate protocol had comparable diagnostic performance to a 3-hour protocol.
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Phase 3 Multicenter Study of Revusiran in Patients with Hereditary Transthyretin-Mediated (hATTR) Amyloidosis with Cardiomyopathy (ENDEAVOUR).
Cardiovasc Drugs Ther2020 Feb;():. doi: 10.1007/s10557-019-06919-4.
Judge Daniel P, Kristen Arnt V, Grogan Martha, Maurer Mathew S, Falk Rodney H, Hanna Mazen, Gillmore Julian, Garg Pushkal, Vaishnaw Akshay K, Harrop Jamie, Powell Christine, Karsten Verena, Zhang Xiaoping, Sweetser Marianne T, Vest John, Hawkins Philip N,
Abstract
PURPOSE:
The Phase 3 ENDEAVOUR study evaluated revusiran, an investigational RNA interference therapeutic targeting hepatic transthyretin (TTR) production, for treating cardiomyopathy caused by hereditary transthyretin-mediated (hATTR) amyloidosis.
METHODS:
Patients with hATTR amyloidosis with cardiomyopathy were randomized 2:1 to receive subcutaneous daily revusiran 500 mg (n?=?140) or placebo (n?=?66) for 5 days over a week followed by weekly doses. Co-primary endpoints were 6-min walk test distance and serum TTR reduction.
RESULTS:
Revusiran treatment was stopped after a median of 6.71 months; the study Sponsor prematurely discontinued dosing due to an observed mortality imbalance between treatment arms. Eighteen (12.9%) patients on revusiran and 2 (3.0%) on placebo died during the on-treatment period. Most deaths in both treatment arms were adjudicated as cardiovascular due to heart failure (HF), consistent with the natural history of the disease. A post hoc safety investigation of patients treated with revusiran found that, at baseline, a greater proportion of those who died were???75 years and showed clinical evidence of more advanced HF compared with those who were alive throughout treatment. Revusiran pharmacokinetic exposures and TTR lowering did not show meaningful differences between patients who died and who were alive. Revusiran did not deleteriously affect echocardiographic parameters, cardiac biomarkers, or frequency of cardiovascular and HF hospitalization events.
CONCLUSIONS:
Causes for the observed mortality imbalance associated with revusiran were thoroughly investigated and no clear causative mechanism could be identified. Although the results suggest similar progression of cardiac parameters in both treatment arms, a role for revusiran cannot be excluded.
CLINICAL TRIAL REGISTRATION:
NCT02319005.
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Prognostic significance of the frontal QRS-T angle in patients with AL cardiac amyloidosis.
J Electrocardiol2020 Feb;59():122-125. doi: S0022-0736(19)30903-3.
Medvedovsky Anna Turyan, Pollak Arthur, Shuvy Mony, Gotsman Israel,
Abstract
INTRODUCTION:
Cardiac involvement is a leading cause of morbidity and mortality in primary light chain (AL) amyloidosis. The electrocardiographic spatial QRS-T angle reflects changes in the direction of the repolarization sequence and is a powerful predictor of outcome in patients with heart failure. We examined the significance of the frontal QRS-T angle in predicting the clinical outcome in patients with AL cardiac amyloidosis.
METHODS:
Forty-three consecutive patients with cardiac involvement of AL amyloidosis were studied. Patients were followed for survival.
RESULTS:
Patient median age was 62 years, 56% were males. After a median follow up of 56 months, 16 out of 43 patients had died (37%). The median QRS-T angle was 102 (interquartile range 35-148). QRS-T angle>102° was associated with increased prevalence of lambda free light chain disease and the presence of a pleural effusion. It was also associated with increased interventricular septum thickness, smaller left ventricle end-diastolic diameter, echocardiographic myocardial sparkling texture, pericardial effusion, elevated NT-Pro-BNP and increased restrictive physiology evident by increased E/A and E/e`. A QRS-T angle>102 was a significant predictor of increased mortality by Kaplan-Meier survival analysis (71.6 ± 11.1% vs. 45.7 ± 11.1%, P = .02). A QRS-T angle>102° was an independent predictor of mortality by Cox regression analysis (HR 3.00, 95% CI 1.01-8.89, P < .05).
CONCLUSIONS:
The QRS-T angle is associated with indices of advanced amyloid disease and is an independent predictor of survival.
Copyright © 2020 Elsevier Inc. All rights reserved.
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Cardiac Amyloidosis: Mimics, Multimodality Imaging Diagnosis, and Treatment.
JACC Cardiovasc Imaging -
Left Ventricular Pressure-Strain-Derived Myocardial Work at Rest and during Exercise in Patients with Cardiac Amyloidosis.
J Am Soc Echocardiogr2020 Feb;():. doi: S0894-7317(19)31207-6.
Clemmensen Tor Skibsted, Eiskjær Hans, Mikkelsen Fabian, Granstam Sven-Olof, Flachskampf Frank A, Sørensen Jens, Poulsen Steen Hvitfeldt,
Abstract
BACKGROUND:
Left ventricular pressure-strain-derived myocardial work index (LVMWI) is a novel, noninvasive method for left ventricular (LV) function evaluation in relation to LV pressure dynamics. LV global longitudinal strain (LVGLS) has proven benefit for diagnosis and risk stratification in patients with cardiac amyloidosis (CA), but LVGLS does not adjust for loading conditions. The aim of the present study was to characterize LVMWI at rest and during exercise in patients with CA.
METHODS:
A total of 155 subjects were retrospectively included. These subjects comprised 100 patients with CA and 55 healthy control subjects. All patients had previously undergone comprehensive two-dimensional echocardiographic examinations at rest. Furthermore, a subgroup 27 patients with CA and 41 control subjects was examined using semisupine exercise stress echocardiography.
RESULTS:
Patients with CA had significantly lower LVGLS, LVMWI, and LV myocardial work efficiency (LVMWE) than control subjects (P < .0001 for all). The reduction in LV myocardial performance was more pronounced in the basal segments, which led to significant alterations in the average apical-to-basal segmental ratios between patients with CA and control subjects (LVGLS, 2.6 [1.9 to 4.1] vs 1.3 [1.2 to 1.5]; LVMWI, 2.6 [1.7 to 3.8] vs 1.3 [1.1 to 1.5]; LVMWE, 1.1 [1.0 to 1.3] vs 1.0 [1.0 to 1.1]; P < .0001 for all). The average increase in LVMWI from rest to peak exercise was 1,974 mm Hg% (95% CI, 1,699 to 2,250 mm Hg%; P < .0001) in control subjects and 496 mm Hg% (95% CI, 156 to 835 mm Hg%; P < .01) in patients with CA. The absolute numeric LVGLS increase was 5.6% (95% CI, 3.9% to 7.3%; P < .0001) in control subjects and only 1.2% (95% CI, -0.9% to 3.3%; P = .26) in patients with CA (between groups, P < .0001) from rest to peak exercise. The LVMWI increase in patients with CA was mediated by improvement in the apical segments (P < .0001), whereas there was no significant LVMWI alterations in the midventricular or basal segments. LVMWE remained stable during exercise in control subjects (? -0.6%; 95% CI, -2.5% to 1.2%; P = .50) but decreased significantly in patients with CA (? -2.5%; 95% CI, -4.8% to -0.2%; P < .05).
CONCLUSIONS:
Patients with CA have significantly reduced magnitude of LVMWI compared with healthy control subjects. With exercise, the differences are even more pronounced. Even though LVMWI increased with exercise, LVMWE decreased, suggesting inefficient myocardial energy exploitation in patients with CA.
Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.
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Geographic variation in public interest about amyloidosis in the United States and English speaking countries.
Amyloid2020 Feb;():1-3. doi: 10.1080/13506129.2020.1724941.
Vuong Jacqueline, Singh Avinainder, Falk Rodney H, Merchant Raina, Dorbala Sharmila,
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A real-life cohort study of immunoglobulin light-chain (AL) amyloidosis patients ineligible for autologous stem cell transplantation due to severe cardiac involvement or advanced disease.
Amyloid2020 Feb;():1-9. doi: 10.1080/13506129.2020.1714581.
Brunger Anne F, Nienhuis Hans L A, Bijzet Johan, Roeloffzen Wilfried W H, Vellenga Edo, Hazenberg Bouke P C,
Abstract
To study the outcome of patients with AL amyloidosis who were ineligible for high dose melphalan (HDM) and autologous stem cell transplantation (ASCT). A real-life retrospective observational cohort study of Dutch patients with AL amyloidosis ineligible for HDM and ASCT was performed at the University Medical Center Groningen from January 2001 until April 2017. Primary outcome measure was overall survival (OS). Secondary outcome measures were hematological response (HR), organ responses, and treatment toxicity. Eighty-four patients were included. Ineligibility was due to NYHA class III/IV (?=?58), otherwise advanced disease (?=?11), advanced age (?=?14), or treatment refusal (?=?1). Early death (<3?months) rate was high (44%). Median OS improved from 4?months in period 2001-2009 (?=?36) to 8?months in period 2009-2017 (?=?48, ?=?.02). HR was seen in 29%, and 42% of the patients, respectively. Median OS was 36?months after induction treatment with bortezomib (?=?32) and 18?months with immunomodulatory imide drug (IMID) (?=?16), both higher than median OS (7?months) with other regimens (?=?27). Incidence of toxicity was high (51%). OS improved in this high-risk group over the years, especially after introduction of new treatment modalities. However, early death rate remains high, illustrating the need for more effective treatment.
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Regression of cardiac amyloid load documented by cardiovascular magnetic resonance in a patient with hereditary amyloidosis.
Clin Res Cardiol2020 Feb;():. doi: 10.1007/s00392-020-01611-2.
Florian Anca, Bietenbeck Michael, Chatzantonis Grigorios, Hüsing-Kabar Anna, Schmidt Hartmut, Yilmaz Ali,
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An antibody against pre-amyloid oligomers: a potential clinical tool or merely an intriguing observation?
Eur Heart J -
An Urgent Need for Data to Drive Decision Making: Rationale for the Canadian Registry for Amyloidosis Research.
Can J Cardiol2019 Dec;():. doi: S0828-282X(19)31511-9.
Davis Margot K, Fine Nowell M,
Abstract
Recent developments in cardiac amyloidosis have raised awareness of the disease and have advanced diagnostic and treatment strategies. Novel therapies may vastly improve the prognosis of the disease but will be associated with significant costs. Data are needed to inform clinical decisions and to drive resource allocation within the health care system. Many aspects of disease management are unlikely to be addressed by clinical trials and are better suited to nonrandomized cohort studies, but the limited numbers of patients in any single centre present barriers to high-quality observational research. Disease registries offer opportunities to assemble large numbers of patients from multiple institutions for adequately powered observational studies, to recruit patients for randomized clinical trials; to provide real-world effectiveness and safety data, to study cost-effectiveness of novel therapies, and to engage patients in the collection of patient-reported outcome data. Existing amyloidosis registries have limitations. Canada is an ideal setting for a national amyloidosis registry, with ethnic diversity, relatively few academic centres, access to advanced diagnostic and therapeutic options, and a track record of collaboration among institutions. The Canadian Registry for Amyloidosis Research aims to capitalize on this opportunity and provide high-quality data to inform clinical practice and health care policy in Canada and beyond.
Copyright © 2020 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
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