Pubblicazioni recenti - cardiac amyloidosis
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Frequency of misdiagnosis in hypertrophic cardiomyopathy.
Eur Heart J Qual Care Clin Outcomes2024 Apr;():. doi: qcae031.
Nielsen Søren K, Rasmussen Torsten B, Hey Thomas M, Zaremba Tomas, Lassen Jens F, Mogensen Jens,
Abstract
BACKGROUND:
Hypertrophic Cardiomyopathy (HCM) is characterized by unexplained left ventricle hypertrophy (LVH) ?15 mm. The condition is often hereditary and family screening is recommended to reduce the risk of adverse disease complications and premature death among relatives. Correct diagnosis of index patients is important to ensure that only relatives at risk of disease development are invited for family screening.
PURPOSE:
To investigate if patients with ICD-10 codes for HCM (DI421) or hypertrophic obstructive cardiomyopathy (DI422) fulfilled recognised diagnostic criteria.
METHODS:
All patients with ICD-10 codes for HCM or HOCM at a Department of Cardiology were identified and had their diagnosis validated by a cardiac investigation or a review of their medical records and previous investigations.
RESULTS:
Two hundred and forty patients had ICD-10 codes for HCM/HOCM, of whom 202 (84%, 202/240) underwent re-examination, while 38 (16%, 38/240) had their hospital notes reviewed. Seventy-six patients (32%, n = 76/240) did not fulfil diagnostic criteria, of whom 39, (51%, n = 39/76) had normal (10 mm) or modest LV wall thickness (11-14 mm). The remaining 37 patients (49%, n = 37/76) had LVH ?15 mm, which was well-explained by uncontrolled hypertension, (32%, n = 24/76), aortic valve stenosis (19%, n = 7/76) or wild-type amyloidosis (16%, 6/76).
CONCLUSION:
One-third of patients with ICD-10 codes for HCM or HOCM did not fulfil recognised diagnostic criteria. Incorrect diagnosis of HCM may cause unnecessary family investigations which may be associated with anxiety, and a waste of health care resources. This highlights the need for specialised cardiomyopathy services to ensure correct diagnosis and management of HCM.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
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When Waldenström macroglobulinemia hits the kidney: Description of a case series and management of a "rare in rare" scenario.
Cancer Rep (Hoboken)2024 Apr;7(4):e2062. doi: e2062.
Danesin Nicolò, Scapinello Greta, Del Prete Dorella, Naso Elena, Berno Tamara, Visentin Andrea, Bonaldi Laura, Martines Annalisa, Bertorelle Roberta, Vianello Fabrizio, Gurrieri Carmela, Zambello Renato, Castellani Chiara, Fedrigo Marny, Rizzo Stefania, Angelini Annalisa, Trentin Livio, Piazza Francesco,
Abstract
BACKGROUND:
Renal injury related to Waldenström macroglobulinemia (WM) occurs in approximately 3% of patients. Kidney biopsy is crucial to discriminate between distinct histopathological entities such as glomerular (amyloidotic and non-amyloidotic), tubulo-interstitial and non-paraprotein mediated renal damage. In this context, disease characterization, management, relationship between renal, and hematological response have been poorly explored. We collected clinical, genetic and laboratory data of seven cases of biopsy-proven renal involvement by WM managed at our academic center and focused on three cases we judged paradigmatic discussing their histopathological patterns, clinical features, and therapeutic options.
CASE:
In this illustrative case series, we confirm that serum creatinine levels and 24?h proteinuria are parameters that when altered should prompt the clinical suspicion of WM-related renal involvement, even if at present there are not precise cut-off levels recommending the execution of a renal biopsy. In our series AL Amyloidosis (n?=?3/7) and tubulo-interstitial infiltration by lymphoma cells (n?=?3/7) were the two more represented entities. BTKi did not seem to improve renal function (Case 1), while bortezomib-based regimens demonstrated a beneficial activity on the hematological and organ response, even when used as second-line therapy after chemoimmunotherapy (Case 3) and also with coexistence of anti-MAG neuropathy (Case 2). In case of poor response to bortezomib, standard chemoimmunotherapy (CIT), such as rituximab-bendamustine, represents an effective option (Case 1, 6, and 7). In our series, CIT generates durable responses more frequently in cases with amyloidogenic renal damage (Case 1, 5, and 7).
CONCLUSION:
In this illustrative case series, we confirm that serum creatinine levels and 24?h proteinuria are parameters that when altered should prompt the clinical suspicion of WM-related renal involvement, even if at present there are not precise cut-off levels recommending the execution of a renal biopsy. Studies with higher numerosity are needed to better clarify the pathological and clinical features of renal involvement during WM and to determine the potential benefit of different therapeutic regimens according to the histopathological subtypes.
© 2024 The Authors. Cancer Reports published by Wiley Periodicals LLC.
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Carpal Tunnel Syndrome and Transthyretin Amyloidosis in the All of Us Research Program.
Mayo Clin Proc2024 Apr;():. doi: S0025-6196(23)00564-5.
Shetty Naman S, Pampana Akhil, Patel Nirav, Maurer Mathew S, Goyal Parag, Li Peng, Arora Garima, Arora Pankaj,
Abstract
OBJECTIVE:
To evaluate the association of carpal tunnel syndrome (CTS) with incident heart failure and incident amyloidosis and to assess the risk of CTS in pathogenic TTR genetic variant carriers.
METHODS:
This prospective cohort study included multiethnic US adults 18 years of age and older without prevalent heart failure and amyloidosis with available genotypic data from the All of Us Research Program. The primary outcomes were incident heart failure and incident amyloidosis. The association of incident heart failure and incident amyloidosis with CTS was assessed using multivariable adjusted Cox models accounting for age, sex, race and ethnicity, obesity, hypertension, diabetes, statin use, and smoking status.
RESULTS:
Of the 166,987 individuals included, the median age was 54 (38 to 66) years; 105,279 (63.0%) were female, and 92,780 (55.6%) were non-Hispanic White individuals; CTS was identified in 12,407 (7.4%) individuals. Compared with individuals without CTS, the adjusted hazard ratio for incident heart failure was 1.13 (95% CI, 1.02 to 1.26) in individuals with CTS. The risk of amyloidosis was ?3-fold higher (adjusted hazard ratio, 2.86; 95% CI, 1.71 to 4.77) in individuals with CTS compared with those without CTS. Individuals carrying a pathogenic TTR variant had an approximately 40% higher risk (adjusted hazard ratio, 1.38; 95% CI, 1.16 to 1.65) for development of CTS compared with noncarriers.
CONCLUSION:
Cardiac amyloidosis screening programs may use CTS as a sentinel event and use genetic testing to identify individuals at a higher risk of TTR amyloidosis.
Copyright © 2023 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
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Concomitant amyloidosis is the primary cause of endothelial and coronary microvascular dysfunction in carpal tunnel syndrome.
Am Heart J Plus2024 May;41():100393. doi: 100393.
?rgi Tu?çe, Baycan Ömer Faruk, Güvenç Tolga Sinan, Özcan Fatma Betül, At?c? Adem, Y?lmaz Yusuf, Çali?kan Mustafa,
Abstract
STUDY OBJECTIVES:
Patients with carpal tunnel syndrome (CTS) show manifestations of arterial abnormalities, including carotid intimal thickening and increased vascular stiffness. As carpal tunnel syndrome is associated with amyloidosis, we hypothesized that previously observed abnormalities can largely be related with concomitant amyloidosis rather than CTS itself.
DESIGN:
Prospective observational study.
SETTING:
Medeniyet University Goztepe Hospital.
PARTICIPANTS:
61 patients with CTS (of whom 32 had biopsy-proven amyloidosis) and 36 healthy controls.
INTERVENTIONS:
Subjects underwent ultrasound examinations for the measurement of coronary flow velocity reserve (CFVR), flow-mediated vasodilatation (FMD) and carotid intimal-media thickness (CIMT).
MAIN OUTCOME MEASURES:
Comparison of CFVR, FMD and CIMT in CTS patients with or without amyloidosis.
RESULTS:
Patients with either CTS or CTS with concomitant amyloidosis (CTS-A) had significantly lower FMD (9.7 % ± 4.0 % in CTS and 10.3 % ± 4.6 % in CTS-A groups,
CONCLUSION:
Most arterial phenomena in CTS patients could be attributable to concomitant amyloidosis, although endothelial dysfunction was present even in patients with CTS without amyloidosis.
© 2024 The Author(s).
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Serum and cerebrospinal fluid neurofilament light chain and glial fibrillary acid protein levels in early and advanced stages of cerebral amyloid Angiopathy.
Alzheimers Res Ther2024 Apr;16(1):86. doi: 10.1186/s13195-024-01457-0.
Rasing Ingeborg, Voigt Sabine, Koemans Emma A, de Kort Anna M, van Harten Thijs W, van Etten Ellis S, van Zwet Erik W, Stoops Erik, Francois Cindy, Kuiperij Bea H, Klijn Catharina J M, Schreuder Floris H B M, van der Weerd Louise, van Osch Matthias J P, van Walderveen Marianne A A, Verbeek Marcel M, Terwindt Gisela M, Wermer Marieke J H,
Abstract
BACKGROUND:
Neurofilament light chain (NFL) is a biomarker for neuroaxonal damage and glial fibrillary acidic protein (GFAP) for reactive astrocytosis. Both processes occur in cerebral amyloid angiopathy (CAA), but studies investigating the potential of NFL and GFAP as markers for CAA are lacking. We aimed to investigate NFL and GFAP as biomarkers for neuroaxonal damage and astrocytosis in CAA.
METHODS:
For this cross-sectional study serum and cerebrospinal fluid (CSF) samples were collected between 2010 and 2020 from controls, (pre)symptomatic Dutch-type hereditary (D-CAA) mutation-carriers and participants with sporadic CAA (sCAA) from two prospective CAA studies at two University hospitals in the Netherlands. NFL and GFAP levels were measured with Simoa-assays. The association between NFL and GFAP levels and age, cognitive performance (MoCA), CAA-related MRI markers (CAA-CSVD-burden) and A?40 and A?42 levels in CSF were assessed with linear regression adjusted for confounders. The control group was divided in age?55 and ?55 years to match the specific groups.
RESULTS:
We included 187 participants: 28 presymptomatic D-CAA mutation-carriers (mean age 40 years), 29 symptomatic D-CAA participants (mean age 58 years), 59 sCAA participants (mean age 72 years), 33 controls?55 years (mean age 42 years) and 38 controls???55 years (mean age 65 years). In presymptomatic D-CAA, only GFAP in CSF (7.7*10pg/mL vs. 4.4*10pg/mL in controls; P<.001 was increased compared to controls. in symptomatic d-caa both serum vs. p="0.008," gfap:130.8pg and csf gfap:11.4 levels were higher than controls pre-symptomatic d-caa. scaa only nfl vs all correlated with age. moca caa-csvd score gfap a>
CONCLUSIONS:
GFAP level in CSF is an early biomarker for CAA and is increased years before symptom onset. NFL and GFAP levels in serum and CSF are biomarkers for advanced CAA.
© 2024. The Author(s).
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Letter to the editor concerning "Cardiac [99mTc]Tc-hydroxydiphosphonate uptake on bone scintigraphy in patients with hereditary transthyretin amyloidosis: an early follow-up marker?".
Eur J Nucl Med Mol Imaging2024 Apr;():. doi: 10.1007/s00259-024-06724-8.
Yu An-Li, Tsai Cheng-Hsuan, Cheng Mei-Fang, Lin Yen-Hung,
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A review regarding the article 'Prevalence of Valvular Heart Disease in Cardiac Amyloidosis and Impact on Survival'.
Curr Probl Cardiol2024 Apr;49(7):102574. doi: 10.1016/j.cpcardiol.2024.102574.
Fan Qiang, Zhang Yuanyuan, Ling Yunfei,
Abstract
Cardiac amyloidosis (CA) is a condition characterized by the accumulation of amyloid fibrils in the heart muscle, resulting in an infiltrative cardiomyopathy. The presence of amyloid protein can impact different parts of the heart, including the valves. Limited data is available on the prevalence and prognostic significance of valvular heart disease (VHD) in CA. However, advancements in imaging technology have allowed for accurate noninvasive diagnosis of CA, eliminating the need for confirmatory endomyocardial biopsy and improving our understanding of this dual pathology. The development of targeted drug therapies for CA and transcatheter valve replacement or repair for VHD has significantly improved the prognosis for patients with both conditions. This review will discuss the findings of this original research and provide an overview of current researches on VHD in CA, as well as the progress in diagnosing and treating CA with VHD.
Copyright © 2024. Published by Elsevier Inc.
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Clonal Hematopoiesis of Indeterminate Potential in Patients with Immunoglobulin Light Chain AL Amyloidosis.
Blood Adv2024 Apr;():. doi: bloodadvances.2024012840.
Lopedote Paolo, Evans Benjamin, Marchetti Alfredo, Chen Tianzeng, Moscvin Maria, Boullt Samuel, Bolli Niccolo, Bianchi Giada,
Abstract
Immunoglobulin light chain (AL) amyloidosis is characterized by the deposition of misfolded monoclonal free light chains, with cardiac complications accounting for patients' mortality. Clonal hematopoiesis of indeterminate potential (CHIP) has been associated with worse cardiovascular outcomes in the general population. Its significance in AL amyloidosis remains unclear. We collected clinical information and outcome data on 76 patients with a diagnosis of AL amyloidosis who underwent deep-targeted sequencing for myeloid neoplasia-associated mutations between April 2018 and August 2023. Variant allele fraction was set at 2% to call CHIP-associated mutations. CHIP mutations were present in AL amyloidosis patients at a higher frequency than age-matched control individuals. Sixteen patients (21%) had at least 1 CHIP mutation. DNMT3A was the most frequent mutation (7/16, 44%). Compared to patients without CHIP, patients with CHIP were enriched for the presence of t(11;14) (69% vs 25%, respectively, p = 0.004) and, for patients with renal involvement, a lower Palladini renal stage (p = 0.001). At a median follow-up of 32.5 months, the presence of CHIP was not associated with worse overall survival or major organ dysfunction progression-free survival. Larger studies and longer follow-up are needed to better define the impact of CHIP in patients with AL amyloidosis.
Copyright © 2024 American Society of Hematology.
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Treatment of transthyretin cardiac amyloidosis.
Curr Opin Cardiol2024 Apr;():. doi: 10.1097/HCO.0000000000001156.
Bampatsias Dimitrios, Wardhere Abdirahman, Maurer Mathew S,
Abstract
PURPOSE OF REVIEW:
Tafamidis is currently the only approved disease-modifying treatment for ATTR-CM. However, there have been important developments in the treatment of ATTR-CM, as the results of two phase 3 trials were published and several other trials are in their final stages. In this review, we summarize current and future therapies for ATTR-CM.
RECENT FINDINGS:
Recently, acoramidis, a TTR stabilizer has been proven to be effective in reducing mortality and morbidity compared to placebo in the ATTRibute-CM trial. Additionally, patisiran, an RNA silencer, preserved functional capacity and quality of life compared to placebo in the APOLLO-B trial. However, the FDA declined to approve patisiran for ATTR-CM. The results of phase 1 trial of ALXN2220, an antiamyloid antibody raise hope for reversal of myocardial damage by amyloid depletion. Phase 3 trials evaluating the efficacy of different RNA silencers, gene editing with CRISPR-Cas9, and other anti-amyloid antibodies are ongoing.
SUMMARY:
Therapies targeting different mechanism in the pathophysiology of ATTR-CM provide new alternatives for treating patients with ATTR-CM. Future research should focus on comparing their effectiveness, the potential of combined treatment with agents from different classes and on identifying the patients who will benefit most from each class of medication.
Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.
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Bilateral Multiple Fractures Obstruct Quantitation of 99mTc-Pyrophosphate Imaging for Cardiac Amyloidosis.
Clin Nucl Med2024 Apr;():. doi: 10.1097/RLU.0000000000005230.
Zhou Qin, Zuo Jiahong, Xiao Liu, Jiang Lisha, Zhao Zhen,
Abstract
A 57-year-old woman who had persistent symptoms of transthyretin cardiac amyloidosis underwent 99mTc-pyrophosphate (99mTc-PYP) scintigraphy. The 99mTc-PYP planar and SPECT/CT fusion image showed diffuse myocardial uptake and multiple fractures of the sternum and ribs. These fractures interfered with semiquantitative scores of 99mTc-PYP uptake, leading to false positive in 99mTc-PYP imaging.
Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.
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[Prioritizing the diagnosis and treatment of cardiac amyloidosis].
Zhonghua Xin Xue Guan Bing Za Zhi2024 Apr;52(4):327-329. doi: 10.3760/cma.j.cn112148-20231224-00523.
Tian Z, Zhang S Y,
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Left atrial strains in cardiac amyloidosis -does its subtype matter?
Int J Cardiol -
Transthyretin amyloid polyneuropathy in France: A cross-sectional study with 413 patients and real-world tafamidis meglumine use (2009-2019).
Rev Neurol (Paris)2024 Apr;():. doi: S0035-3787(24)00489-2.
Adams D, Cintas P, Solé G, Tard C, Labeyrie C, Echaniz-Laguna A, Cauquil C, Pereon Y, Magy L, Morales R Juntas, Antoine J C, Lagrange E, Petiot P, Mallaret M, Francou B, Guiochon-Mantel A, Coste A, Demarcq O, Geffroy C, Famelart V, Rudant J, Bartoli M, Donal E, Lairez O, Eicher J C, Kharoubi M, Oghina S, Trochu J N, Inamo J, Habib G, Roubille F, Hagège A, Morio F, Cariou E, Adda J, Slama M S, Charron P, Algalarrondo V, Damy T, Attarian S,
Abstract
OBJECTIVE:
We aimed to describe characteristics of patients with ATTR variant polyneuropathy (ATTRv-PN) and ATTRv-mixed and assess the real-world use and safety profile of tafamidis meglumine 20mg.
METHODS:
Thirty-eight French hospitals were invited. Patient files were reviewed to identify clinical manifestations, diagnostic methods, and treatment compliance.
RESULTS:
Four hundred and thirteen patients (296 ATTRv-PN, 117 ATTRv-mixed) were analyzed. Patients were predominantly male (68.0%) with a mean age of 57.2±17.2 years. Interval between first symptom(s) and diagnosis was 3.4±4.3 years. First symptoms included sensory complaints (85.9%), dysautonomia (38.5%), motor deficits (26.4%), carpal tunnel syndrome (31.5%), shortness of breath (13.3%), and unexplained weight loss (16.0%). Mini-invasive accessory salivary gland or punch skin and nerve biopsies were most common, with a performance of 78.8-100%. TTR genetic sequencing, performed in all patients, revealed 31 TTR variants. Tafamidis meglumine was initiated in 156/214 (72.9%) ATTRv-PN patients at an early disease stage. Median treatment duration was 6.00 years in ATTRv-PN and 3.42 years in ATTRv-mixed patients. Tafamidis was well tolerated, with 20 adverse events likely related to study drug among the 336 patients.
CONCLUSION:
In France, ATTRv patients are usually identified early thanks to the national network and the help of diagnosis combining genetic testing and mini-invasive biopsies.
Copyright © 2024 Elsevier Masson SAS. All rights reserved.
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Left Ventricular Segmentation, Warping, and Myocardial Registration for Automated Strain Measurement.
J Imaging Inform Med2024 Apr;():. doi: 10.1007/s10278-024-01119-5.
Huang Kuan-Chih, Lin Donna Shu-Han, Jeng Geng-Shi, Lin Ting-Tse, Lin Lian-Yu, Lee Chih-Kuo, Lin Lung-Chun,
Abstract
The left ventricular global longitudinal strain (LVGLS) is a crucial prognostic indicator. However, inconsistencies in measurements due to the speckle tracking algorithm and manual adjustments have hindered its standardization and democratization. To solve this issue, we proposed a fully automated strain measurement by artificial intelligence-assisted LV segmentation contours. The LV segmentation model was trained from echocardiograms of 368 adults (11,125 frames). We compared the registration-like effects of dynamic time warping (DTW) with speckle tracking on a synthetic echocardiographic dataset in experiment-1. In experiment-2, we enrolled 80 patients to compare the DTW method with commercially available software. In experiment-3, we combined the segmentation model and DTW method to create the artificial intelligence (AI)-DTW method, which was then tested on 40 patients with general LV morphology, 20 with dilated cardiomyopathy (DCMP), and 20 with transthyretin-associated cardiac amyloidosis (ATTR-CA), 20 with severe aortic stenosis (AS), and 20 with severe mitral regurgitation (MR). Experiments-1 and -2 revealed that the DTW method is consistent with dedicated software. In experiment-3, the AI-DTW strain method showed comparable results for general LV morphology (bias?-?0.137?±?0.398%), DCMP (-?0.397?±?0.607%), ATTR-CA (0.095?±?0.581%), AS (0.334?±?0.358%), and MR (0.237?±?0.490%). Moreover, the strain curves showed a high correlation in their characteristics, with R-squared values of 0.8879-0.9452 for those LV morphology in experiment-3. Measuring LVGLS through dynamic warping of segmentation contour is a feasible method compared to traditional tracking techniques. This approach has the potential to decrease the need for manual demarcation and make LVGLS measurements more efficient and user-friendly for daily practice.
© 2024. The Author(s).
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Postdevelopment Performance and Validation of the Artificial Intelligence-Enhanced Electrocardiogram for Detection of Cardiac Amyloidosis.
JACC Adv2023 Oct;2(8):. doi: 100612.
Harmon David M, Mangold Kathryn, Suarez Abraham Baez, Scott Christopher G, Murphree Dennis H, Malik Awais, Attia Zachi I, Lopez-Jimenez Francisco, Friedman Paul A, Dispenzieri Angela, Grogan Martha,
Abstract
BACKGROUND:
We have previously applied artificial intelligence (AI) to an electrocardiogram (ECG) to detect cardiac amyloidosis (CA).
OBJECTIVES:
In this validation study, the authors observe the postdevelopment performance of the AI-enhanced ECG to detect CA with respect to multiple potential confounders.
METHODS:
Amyloid patients diagnosed after algorithm development (June 2019-January 2022) with a 12-lead ECG were identified (n = 440) and were required to have CA. A 15:1 age- and sex-matched control group was identified (n = 6,600). Area under the receiver operating characteristic (AUC) was determined for the cohort and subgroups.
RESULTS:
The average age was 70.4 ± 10.3 years, 25.0% were female, and most patients were White (91.3%). In this validation, the AI-ECG for amyloidosis had an AUC of 0.84 (95% CI: 0.82-0.86) for the overall cohort and between amyloid subtypes, which is a slight decrease from the original study (AUC 0.91). White, Black, and patients of "other" races had similar algorithm performance (AUC >0.81) with a decreased performance for Hispanic patients (AUC 0.66). Algorithm performance shift over time was not observed. Low ECG voltage and infarct pattern exhibited high AUC (>0.90), while left ventricular hypertrophy and left bundle branch block demonstrated lesser performance (AUC 0.75 and 0.76, respectively).
CONCLUSIONS:
The AI-ECG for the detection of CA maintained an overall strong performance with respect to patient age, sex, race, and amyloid subtype. Lower performance was noted in left bundle branch block, left ventricular hypertrophy, and ethnically diverse populations emphasizing the need for subgroup-specific validation efforts.
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Development and evolution of nuclear cardiology and cardiac PET in Canada.
J Med Imaging Radiat Sci2024 Apr;():. doi: S1939-8654(24)00102-4.
Ruddy Terrence D, Davies Ross A, Kiess Marla C,
Abstract
Gated radionuclide angiography and myocardial perfusion imaging were developed in the United States and Europe in the 1970's and soon adopted in Canadian centers. Much of the early development of nuclear cardiology in Canada was in Toronto, Ontario and was quickly followed by new programs across the country. Clinical research in Canada contributed to the further development of nuclear cardiology and cardiac PET. The Canadian Nuclear Cardiology Society (CNCS) was formed in 1995 and became the Canadian Society of Cardiovascular Nuclear and CT Imaging (CNCT) in 2014. The CNCS had a major role in education and advocacy for cardiovascular nuclear medicine testing. The CNCS established the Dr Robert Burns Lecture and CNCT named the Canadian Society of Cardiovascular Nuclear and CT Imaging Annual Achievement Award for Dr Michael Freeman in memoriam of these two outstanding Canadian leaders in nuclear cardiology. The future of nuclear cardiology in Canada is exciting with the expanding use of SPECT imaging to include Tc-99m-pyrophosphate for diagnosis of transthyretin cardiac amyloidosis and the ongoing introduction of cardiac PET imaging.
Copyright © 2024. Published by Elsevier Inc.
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(Non)-Exertional Variables of Cardiopulmonary Exercise Testing in Heart Failure with and Without Cardiac Amyloidosis.
Curr Heart Fail Rep2024 Apr;():. doi: 10.1007/s11897-024-00661-1.
Wernhart Simon, Michel Lars, Carpinteiro Alexander, Luedike Peter, Rassaf Tienush,
Abstract
PURPOSE OF REVIEW:
Cardiac amyloidosis (CA) constitutes an important etiology of heart failure with preserved ejection fraction (HFpEF) or heart failure with mildly reduced ejection fraction (HFmrEF). Since patients with CA show early exhaustion, we aimed to investigate whether non-exertional variables of cardiopulmonary exercise testing (CPET) provide additional information in comparison to traditional peak oxygen consumption (VO).
RECENT FINDINGS:
We retrospectively investigated CPET variables of patients with HFpEF and HFmrEF with (n?=?21) and without (n?=?21, HF) CA at comparable age and ejection fraction. Exertional and non-exertional CPET variables as well as laboratory and echocardiographic markers were analyzed. The primary outcome was the difference in CPET variables between groups. The secondary outcome was rehospitalization in patients with CA during a follow-up of 24 months. Correlations between CPET, NTproBNP, and echocardiographic variables were calculated to detect patterns of discrimination between the groups. HF patients with CA were inferior to controls in most exertional and non-exertional CPET variables. Patients with CA were hospitalized more often (p?=?0.002), and rehospitalization was associated with VE/VCO (p?=?0.019), peak oxygen pulse (p?=?0.042), the oxygen equivalent at the first ventilatory threshold (p?=?0.003), circulatory (p?=?0.024), and ventilatory power (p?.001), but not VO (p?=?0.127). Higher performance was correlated with lower E/e' and NTproBNP as well as higher resting heart rate and stroke volume in CA. Patients with CA displayed worse non-exertional CPET performance compared to non-CA HF patients, which was associated with rehospitalization. Differences between correlations of resting echocardiography and CPET variables between groups emphasize different properties of exercise physiology despite comparable ejection fraction.
© 2024. The Author(s).
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Preoperative diagnosis and safe surgical approach in gallbladder amyloidosis: a case report.
Surg Case Rep2024 Apr;10(1):89. doi: 89.
Shinohara Makoto, Hashimoto Masakazu, Kitamura Yoshihito, Nakashima Keigo, Hamaoka Michinori, Miguchi Masashi, Misumi Toshihiro, Fujikuni Nobuaki, Ikeda Satoshi, Matsugu Yasuhiro, Hattori Yui, Nishisaka Takashi, Nakahara Hideki,
Abstract
BACKGROUND:
Preoperative diagnosis of gallbladder amyloidosis is usually difficult. In our case, the patient exhibited gallbladder dyskinesia, which led us to suspect cholecystic amyloidosis. We were able to safely perform surgery before cholecystitis onset.
CASE PRESENTATION:
A 59-year-old male patient with a history of multiple myeloma and cardiac amyloidosis presented to our hospital with a chief complaint of epicardial pain. Abdominal ultrasonography and computed tomography revealed an enlarged gallbladder and biliary sludge without any specific imaging findings of cholecystitis. After percutaneous transhepatic gallbladder aspiration (PTGBA), the patient experienced recurrent bile retention and right upper quadrant pain. Flopropione was effective in relieving these symptoms. Based on his symptoms and laboratory findings, we diagnosed the patient with dyskinesia of the gallbladder. Considering his medical history, we suspected that it was caused by amyloidosis of the gallbladder. A laparoscopic cholecystectomy was performed. The histopathological examination showed amyloid deposits in the gallbladder mucosa, from the intrinsic layer to the submucosa, and in the peripheral nerves of the gallbladder neck. The patient was discharged on postoperative day 5 and has had no recurrence of abdominal pain since then.
CONCLUSION:
In our case, gallbladder dyskinesia symptoms led us to suspect gallbladder amyloidosis. We safely surgically treated the patient before cholecystitis onset.
© 2024. The Author(s).
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Acute Exacerbation of Idiopathic Pulmonary Fibrosis With Concurrent Cardiac Amyloidosis: A Technetium Pyrophosphate Study.
Cureus2024 Mar;16(3):e56358. doi: e56358.
Yanagihara Toyoshi, Hatashima Hikaru, Ogata Hiroaki, Moriuchi Yuki, Ishimatsu Akiko, Otsuka Junji, Taguchi Kazuhito, Moriwaki Atushi, Yoshida Makoto,
Abstract
Amyloidosis presents a diagnostic challenge, particularly when concomitant with severe conditions like acute exacerbations of idiopathic pulmonary fibrosis (IPF). In this report, we detail the case of a 73-year-old patient with acute exacerbation of IPF and simultaneous emergence of cardiac amyloidosis. The patient's clinical journey began with persistent exertional dyspnea, progressing to hypoxemia on admission. Chest CT scans showed extensive ground-glass opacities, consolidations, and pre-existing honeycombing-like cysts and reticular shadows, accompanied by a right-sided pleural effusion. The therapeutic strategy for acute exacerbation of IPF encompassed methylprednisolone pulse therapy, tacrolimus, and nintedanib, augmented with intravenous immunoglobulin and recombinant thrombomodulin. Concurrently, heart failure with preserved ejection fraction was managed with a pharmacological trio: empagliflozin, diuretics, and eplerenone. A hypertrophied heart and low limb voltage prompted an investigation for cardiac amyloidosis, which Technetium pyrophosphate (Tc-PYP) scintigraphy confirmed, yielding a probable diagnosis. Following steroid tapering, the patient was discharged home. This case prompted an investigation into the potential role of amyloidosis in pulmonary pathology. Our retrospective review of 10 patients, including four with cardiac amyloidosis, who underwent Tc-PYP scintigraphy, revealed a nonsignificant yet notable trend of increased pulmonary accumulation in cardiac amyloidosis cases (median (interquartile range): 5.4×10 (5.3-13.1×10) vs. 3.6×10 (2.4-5.1×10), p=0.0667). Notably, the pulmonary counts in this patient exceeded the negative cohort's mean values, hinting at a possible contribution of amyloid deposition to pulmonary pathology. This study, pioneering in evaluating lung field accumulation of Tc-PYP in cardiac amyloidosis, may provide novel insights into the influence of amyloidosis on pulmonary conditions.
Copyright © 2024, Yanagihara et al.
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Thermal evaporation as sample preparation for silver-assisted laser desorption/ionization mass spectrometry imaging of cholesterol in amyloid tissues.
Analyst2024 Apr;():. doi: 10.1039/d4an00181h.
Strnad ?t?pán, Vrkoslav Vladimír, Mengr Anna, Fabián Ond?ej, Rybá?ek Ji?í, Kubánek Milo?, Melenovský Vojt?ch, Maletínská Lenka, Cva?ka Josef,
Abstract
Cholesterol plays an important biological role in the body, and its disruption in homeostasis and synthesis has been implicated in several diseases. Mapping the locations of cholesterol is crucial for gaining a better understanding of these conditions. Silver deposition has proven to be an effective method for analyzing cholesterol using mass spectrometry imaging (MSI). We optimized and evaluated thermal evaporation as an alternative deposition technique to sputtering for silver deposition in MSI of cholesterol. A silver layer with a thickness of 6 nm provided an optimal combination of cholesterol signal intensity and mass resolution. The deposition of an ultrathin nanofilm of silver enabled high-resolution MSI with a pixel size of 10 ?m. We used this optimized method to visualize the distribution of cholesterol in the senile plaques in the brains of APP/PS1 mice, a model that resembles Alzheimer's disease pathology. We found that cholesterol was evenly distributed across the frontal cortex tissue, with no evidence of plaque-like accumulation. Additionally, we investigated the presence and distribution of cholesterol in myocardial sections of a human heart affected by wild-type ATTR amyloidosis. We identified the presence of cholesterol in areas with amyloid deposition, but complete colocalization was not observed.
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