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Pubblicazioni recenti - cardiac amyloidosis
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Diagnosis of cardiac amyloid transthyretin (ATTR) amyloidosis by early (soft tissue) phase [Tc]Tc-DPD whole body scan: comparison with late (bone) phase imaging.
Eur Radiol2022 Jan;():. doi: 10.1007/s00330-021-08420-9.
Minutoli Fabio, Russo Massimo, Di Bellaearly Phase Planar Imaging Gianluca, Laudicella Riccardo, Spataro Alessandro, Vento Antonio, Comis Alessio, Gentile Luca, Mazzeo Anna, Vita Giuseppe, Baldari Sergio,
Abstract
OBJECTIVES:
Although expert consensus recommendations suggest 2-3 h as the time interval between bone-seeking radiotracers injection and acquisition, it has been reported that images obtained early after [Tc]Tc-HMDP administration are sufficient to diagnose cardiac amyloidosis. We evaluated the diagnostic performance of [Tc]Tc-DPD early phase whole body scan with respect to late phase imaging.
METHODS:
We qualitatively and semiquantitatively reviewed [Tc]Tc-DPD imaging of 53 patients referred for suspect cardiac amyloidosis. Findings of early and late phase images were compared with SPECT results (considered the standard-of-reference) determining sensitivity and specificity for visual analysis of each phase imaging and for each semiquantitative index.
RESULTS:
SPECT imaging was negative for cardiac accumulation in 25 patients and positive in 28. Visual analysis of early phase whole body scan had an extremely significant capability to predict SPECT results; nevertheless, complete agreement was not reached. Visual analysis of late phase imaging showed slightly better results. Semiquantitative analysis of early phase images, namely heart to mediastinum ratio, performed better than semiquantitative analysis of late phase images.
CONCLUSION:
Visual analysis of [Tc]Tc-DPD early phase whole body scan is promising in diagnosing cardiac amyloidosis; further studies are needed to confirm our results in different clinical scenarios.
KEY POINTS:
? Visual analysis of early phase planar imaging using [Tc]Tc-DPD is accurate to diagnose cardiac amyloidosis and may be satisfactory at least in frail patients with high cardiac burden of amyloid fibrils.
© 2021. The Author(s), under exclusive licence to European Society of Radiology.
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[Diagnosis and treatment of cardiac transthyretin amyloidosis. Innovative therapy with tafamidis.]
Rev Med Liege2022 Jan;77(1):63-68.
Legrand D, Nyssen A, Jackers L, Brogneaux C, Pirotte I, Grayet D, Lacremans D, Magnée M,
Abstract
Cardiac amyloidosis is a rare and severe disease with worse prognosis than classic cardiac insufficiency. Transthyretin amyloïdosis is an underdiagnosed cause of amyloidosis. Technetium scintigraphy allows to confirm diagnosis of transthyretin amyloidosis with great specificity. A new hope is brought by tafamidis, a novel therapeutic option for this specific condition. This medication is the first one that proved a risk reduction in mortality among patients with cardiac amyloidosis in class NYHA I and II. In this article, we review across a clinical case the modalities for the diagnosis and treatment of transthyretin amyloidosis.
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Healthcare resource use associated with the diagnosis of transthyretin amyloidosis cardiomyopathy.
Health Sci Rep2022 Mar;5(1):e466. doi: 10.1002/hsr2.466.
Asher Clint, Guilder Andrew, Finocchiaro Gherardo, Carr-White Gerry, Rodríguez-Guadarrama Yael,
Abstract
Objectives:
Our primary aim was to evaluate the healthcare resource use associated with the diagnosis of transthyretin amyloidosis cardiomyopathy. Second, we aim to assess the effect of the number of diagnostic tests and clinical contact points on the total time and costs between symptom onset and diagnosis defining a quantitative hypothetical optimized diagnostic pathway.
Setting:
Clinical and cost data were collected from patients presenting between 2010 and 2018 in a tertiary referral institution in South London involving two participating hospitals.
Participants:
Thirty-eight adult patients with a definite diagnosis of transthyretin amyloidosis cardiomyopathy were included, mostly male (n = 28, 74%) and of African-Caribbean descent (n = 23, 64%). We excluded patients without a confirmed transthyretin amyloidosis cardiomyopathy or those on inotersen, patisiran, or diflunisal at point of referral.
Primary and secondary outcome measures:
The average time between first presentation and final diagnosis, and the cost per patient per month. By comparing to a more optimal clinical pathway towards diagnosis, we considered what could be the theoretical gain in terms of time to diagnosis and financial savings.
Results:
The average time between first presentation and final diagnosis was 2.74?years. The average cost per patient per month was higher with progressive heart failure symptoms. A hypothetical optimal pathway reduces time to diagnosis of 1.65 to 1.74?years per patient. The potential financial savings are estimated within the range of £3000 to £4800 per patient.
Conclusions:
Patients diagnosed with transthyretin amyloidosis cardiomyopathy have substantial healthcare resource utilization and costs starting from symptom onset. Higher costs were observed with progression in symptoms and appear linked to a delayed diagnosis. The number of additional diagnostic tests and clinical contact points may contribute to this and could represent a path to explore further for important health and cost savings, with more efficient pathways for these patients to be managed.
© 2022 The Authors. Health Science Reports published by Wiley Periodicals LLC.
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Successful management of refractory constipation using Kampo medicine Mashiningan in a patient with wild-type ATTR cardiac amyloidosis.
J Cardiol Cases2022 Jan;25(1):34-36. doi: 10.1016/j.jccase.2021.06.004.
Imamura Teruhiko, Hori Masakazu, Tanaka Shuhei, Kinugawa Koichiro,
Abstract
A wild-type ATTR amyloidosis is a systemic disease with multi-organ dysfunction, involving heart, kidney, skin, and gastrointestinal tract, due to deposition of wild-type transthyretin in each organ. We had a 76-year-old man diagnosed with wild-type ATTR cardiac amyloidosis, whose heart failure symptom improved by anti-heart failure medications but constipation refractory to multiple conventional medications persisted. Following the conversion from lubiprostone to Kampo medicine mashiningan, his average days per one evacuation decreased from around 7.0 days down to 1.6 days. Mashininigan might be an alternative option to improve refractory constipation in patients with cardiac amyloidosis. .
© 2021 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.
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A Guide to the Non-Invasive Imaging Assessment of Cardiac Amyloidosis.
Can J Cardiol2022 Jan;():. doi: S0828-282X(22)00003-4.
Music Sanela, Szilagyi Ariana, Chetrit Michael,
Abstract
Cardiac amyloidosis, thought to be rare, is increasingly recognized and benefits from newer therapeutic options. This article provides a practical approach for the clinical cardiologist to the non-invasive assessment of cardiac amyloidosis, from clinical red flags to the appropriate use of echocardiography, cardiovascular magnetic resonance imaging, and technetium-99m pyrophosphate scintigraphy in the screening, diagnosis, and prognosis of cardiac amyloidosis.
Copyright © 2022. Published by Elsevier Inc.
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Molecular Mechanisms of Cardiac Amyloidosis.
Int J Mol Sci2021 Dec;23(1):. doi: 25.
Saito Yukihiro, Nakamura Kazufumi, Ito Hiroshi,
Abstract
Cardiac involvement has a profound effect on the prognosis of patients with systemic amyloidosis. Therapeutic methods for suppressing the production of causative proteins have been developed for ATTR amyloidosis and AL amyloidosis, which show cardiac involvement, and the prognosis has been improved. However, a method for removing deposited amyloid has not been established. Methods for reducing cytotoxicity caused by amyloid deposition and amyloid precursor protein to protect cardiovascular cells are also needed. In this review, we outline the molecular mechanisms and treatments of cardiac amyloidosis.
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Diagnostic Tools for Cardiac Amyloidosis: A Pragmatic Comparison of Pathology, Imaging and Laboratories.
Curr Probl Cardiol2022 Jan;():101106. doi: S0146-2806(22)00005-6.
Joury Abdulaziz, Faaborg-Andersen Christian, Quintana Raymundo A, daSilva-deAbreu Adrian, Nativi-Nicolau Jose,
Abstract
Cardiac amyloidosis (CA) is a complex disease considered to be the most common underdiagnosed form of restrictive cardiomyopathy. Accumulation of misfolded proteins called amyloid fibrils in the extracellular space results in clinical deterioration and late diagnosis is associated with morbidity and mortality. Both types of this disease, light chain CA and transthyretin-related CA share many cardiac and extracardiac features that compromise multiple organs such as kidneys, musculoskeletal system, autonomic nervous system, and gastrointestinal tract. Early diagnosis and detection of CA are imperative. Clinicians should maintain a high degree of suspicion among patients with unexplained diastolic heart failure to implement different disease-altering therapies at the early stages of the disease. In this article, we provided a comprehensive review of multiple invasive and non-invasive cardiac imaging modalities with their respective degrees of sensitivities and specificity.
Copyright © 2022. Published by Elsevier Inc.
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Atrial function and geometry differences in transthyretin versus immunoglobulin light chain amyloidosis: a cardiac magnetic resonance study.
Sci Rep2022 Jan;12(1):140. doi: 10.1038/s41598-021-03359-9.
Palmer Cassady, Truong Vien T, Slivnick Jeremy A, Wolking Sarah, Coleman Paige, Mazur Wojciech, Zareba Karolina M,
Abstract
To determine the differences in left atrial (LA) function and geometry assessed by cardiac magnetic resonance (CMR) between transthyretin (ATTR) and immunoglobulin light chain (AL) cardiac amyloidosis (CA). We performed a retrospective analysis of 54 consecutive patients (68.5% male, mean age 67?±?11 years) with confirmed CA (24 ATTR, 30 AL) who underwent comprehensive CMR examinations. LA structural and functional assessment including LA volume, LA sphericity index, and LA strain parameters were compared between both subtypes. In addition, 15 age-matched controls were compared to all groups. Patients with ATTR-CA were older (73?±?9 vs. 62?±?10 years, p?0.001) and more likely to be male (83.3% vs. 56.7%, p?=?0.036) when compared to AL-CA. No significant difference existed in LA maximum volume and LA sphericity index between ATTR-CA and AL-CA. LA minimum volumes were larger in ATTR-CA when compared with AL-CA. There was a significant difference in LA function with worse strain values in ATTR vs AL: left atrial reservoir [7.4 (6.3-12.8) in ATTR vs. 13.8 (6.90-24.8) in AL, p?=?0.017] and booster strains [3.6 (2.6-5.5) in ATTR vs. 5.2 (3.6-12.1) in AL, p?=?0.039]. After adjusting for age, LA reservoir remained significantly lower in ATTR-CA compared to AL-CA (p?=?0.03), but not LA booster (p?=?0.16). We demonstrate novel differences in LA function between ATTR-CA and AL-CA despite similar LA geometry. Our findings of more impaired LA function in ATTR may offer insight into higher AF burden in these patients.
© 2022. The Author(s).
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Restrictive spirometry pattern and abnormal cardiopulmonary response to exercise in transthyretin cardiac amyloidosis.
Eur Respir J2022 Jan;():. doi: 2102838.
Banydeen Rishika, Vergaro Giuseppe, Deney Antoine, Monfort Astrid, Emdin Michele, Lairez Olivier, Giguet Anna Gaelle, Inamo Jocelyn, Neviere Remi,
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Cardiac amyloidosis: Description of a series of 143 cases.
Med Clin (Barc)2022 Jan;():. doi: S0025-7753(21)00672-2.
Barge-Caballero Gonzalo, Barge-Caballero Eduardo, López-Pérez Manuel, Bilbao-Quesada Raquel, González-Babarro Eva, Gómez-Otero Inés, López-López Andrea, Gutiérrez-Feijoo Mario, Varela-Román Alfonso, González-Juanatey Carlos, Díaz-Castro Óscar, Crespo-Leiro María G,
Abstract
INTRODUCTION AND OBJECTIVES:
Recently, there have been important advances in the diagnosis and treatment of cardiac amyloidosis (CA). Our aim was to provide an updated description of its 2 most frequent types: the transthyretin CA (ATTR-CA) and the light chain CA (AL-CA).
METHODS:
Prospective registry of patients with CA diagnosed in 7 institutions in Galicia (Spain) between January 1, 2018 and June 30, 2020. Variables related to clinical characteristics, complementary tests, survival and causes of death were collected.
RESULTS:
One hundred and forty-three patients with CA were consecutively included, 128 ATTR-CA (89.5%) and 15 AL-CA (10.5%). Mean age was 79.6±7.7 years and 23.8% were women. Most patients with ATTR-CA were diagnosed non-invasively (87.5%). On physical examination, 35.7, 35 and 7% had Popeye's sign, Dupuytren's contracture and macroglossia, respectively. Twelve-month and 24-month survival was 92.1 and 76.2% in the ATTR-CA group, and 78.6 and 61.1% in the AL-CA group (P=.152). The cause of death was cardiovascular in 80.8% of the cohort.
CONCLUSIONS:
ATTR-CA can be diagnosed non-invasively in most cases and it is the most common type of CA in routine clinical practice. Furthermore, an increase in the short-term survival of CA appears to be observed, which could be due to advances related to its diagnosis and treatment.
Copyright © 2021 Elsevier España, S.L.U. All rights reserved.
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Sex-related differences in clinical presentation and all-cause mortality in patients with cardiac transthyretin amyloidosis and light chain amyloidosis.
Int J Cardiol2022 Jan;():. doi: S0167-5273(21)02051-9.
Zampieri Mattia, Argirò Alessia, Allinovi Marco, Tassetti Luigi, Zocchi Chiara, Gabriele Martina, Andrei Valentina, Fumagalli Carlo, Di Mario Carlo, Tomberli Alessia, Olivotto Iacopo, Perfetto Federico, Cappelli Francesco,
Abstract
We aimed to ascertain whether sex-related differences are relevant to clinical presentation, cardiac phenotype and all-cause mortality in different types of cardiac amyloidosis, a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated. Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL). Women with wtATTR, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men, otherwise, bio-humoral parameters, NYHA class, and ECG characteristics were similar. Comparing females and male with wtATTR, hATTR and AL, no differences in Kaplan-Meier curves for all-cause mortality were observed with regard to sex, p-value >0.05. In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, all-cause mortality was not affected. Nevertheless, women with wtATTR had echocardiographic signs of more advanced disease and higher NAC score at diagnosis suggesting a possible later recognition of disease compared to men.
Copyright © 2021. Published by Elsevier B.V.
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Potential Applications for Targeted Gene Therapy to Protect Against Anthracycline Cardiotoxicity: Primer.
JACC CardioOncol2021 Dec;3(5):650-662. doi: 10.1016/j.jaccao.2021.09.008.
Kok Cindy Y, MacLean Lauren M, Ho Jett C, Lisowski Leszek, Kizana Eddy,
Abstract
Anthracyclines are associated with risk of significant dose-dependent cardiotoxicity. Conventional heart failure therapies have neither ameliorated declining cardiac function nor addressed the underlying cause. Gene therapy may confer long-term cardioprotection by rendering the heart resistant to anthracyclines after 1 treatment, although the optimal therapeutic target remains to be elucidated. Recombinant adeno-associated virus is now clinically approved for the treatment of lipoprotein lipase deficiency, spinal muscular atrophy, and hereditary transthyretin amyloidosis. High-throughput methods allow selection of recombinant adeno-associated virus capsids that facilitate efficient gene delivery to specific target cells. Vector safety is enhanced by incorporating cardiac-specific promoters into vector design and localizing delivery to reduce off-target risk. Any cardioprotective transgene may bear a degree of risk as they may play as yet unknown roles, which require careful assessment using clinically relevant models. The innovative technologies outlined here make gene therapy a promising proof of principle, with potential further application to nonanthracycline chemotherapeutics.
© 2021 The Authors.
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A Case of Early Hereditary Transthyretin Amyloid Cardiomyopathy Recognition With Genetic Screening: A Case Report.
J Prim Care Community Health;13():21501319211062682. doi: 10.1177/21501319211062682.
Juarez Michel, Del Rio-Pertuz Gaspar, Parmar Kanak, Bois Melanie C, Shurmur Scott, Argueta-Sosa Erwin,
Abstract
Transthyretin amyloid cardiomyopathy (ATTR-CM) is one of the most common types of cardiac amyloidosis. Amyloid cardiomyopathy more commonly affects men, elderly, and 3% to 4% of the African-American population. ATTR-CM suspicion and diagnosis is challenging; however, awareness of the disease is increasing, and best practices to identify it are being proposed. The approach to suspected cases of ATTR-CM relies on the presence of heart failure, red flag signs and symptoms, and age >65 or >70 for men and women respectively. Little is known about cases when it presents in early ages. We report a 62-year-old African American male with past medical history of hyperlipidemia, prostate cancer, hypertension, bilateral carpal tunnel surgery that had debuted with a cardiac arrhythmia at age 55 and was diagnosed with heart failure several years later. Restrictive cardiomyopathy was suspected, and genetic screening was sent for ATTRm which confirmed a pathogenic trasnthyretin gene mutation. Endomyocardial biopsy was performed which confirmed cardiac amyloid deposition. ATTR-CM is a rare disease with an increasing prevalence. Cases with out of proportion signs and symptoms of heart failure with preserved ejection fractions should raise the suspicion of ATTR-CM despite age.
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Myocardial Parametric Mapping by Cardiac Magnetic Resonance Imaging in Pediatric Cardiology and Congenital Heart Disease.
Circ Cardiovasc Imaging2022 Jan;15(1):e012242. doi: 10.1161/CIRCIMAGING.120.012242.
Rao Sruti, Tseng Stephanie Y, Pednekar Amol, Siddiqui Saira, Kocaoglu Murat, Fares Munes, Lang Sean M, Kutty Shelby, Christopher Adam B, Olivieri Laura J, Taylor Michael D, Alsaied Tarek,
Abstract
Parametric mapping, that is, a pixel-wise map of magnetic relaxation parameters, expands the diagnostic potential of cardiac magnetic resonance by enabling quantification of myocardial tissue-specific magnetic relaxation on an absolute scale. Parametric mapping includes T mapping (native and postcontrast), T and T* mapping, and extracellular volume measurements. The myocardial composition is altered in various disease states affecting its inherent magnetic properties and thus the myocardial relaxation times that can be directly quantified using parametric mapping. Parametric mapping helps in the diagnosis of nonfocal disease states and allows for longitudinal disease monitoring, evaluating therapeutic response (as in Thalassemia patients with iron overload undergoing chelation), and risk-stratification of certain diseases. In this review article, we describe various mapping techniques and their clinical utility in congenital heart disease. We will also review the available literature on normative values in children, the strengths, and weaknesses of these techniques. This review provides a starting point for pediatric cardiologists to understand and implement parametric mapping in their practice.
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The functional and structural evaluation of small fibers in asymptomatic carriers of p.Val50Met (Val30Met) mutation.
Neuromuscul Disord2021 Dec;():. doi: S0960-8966(21)00718-5.
Bekircan-Kurt Can Ebru, Yilmaz Ezgi, Arslan Doruk, Yildiz Fatma Gokcem, Dikmetas Özlem, Ergul-Ulger Zeynep, Kocabeyoglu Sibel, Irkec Murat, Hekimsoy Vedat, Tokgozoglu Lale, Tan Ersin, Erdem-Ozdamar Sevim,
Abstract
Therapeutic advances in hereditary amyloid transthyretin (ATTRv) amyloidosis with polyneuropathy extended life expectancy and delayed symptom progression especially in patients with early disease. Thus, detection and monitoring of asymptomatic carriers gained importance. However, there is still limited consensus on genetic screening of ATTRv-PN patients' family members and diagnostic tests that must be done in the follow-up. In this study, we followed prospectively five asymptomatic carriers of a family with ATTRV30M (p.Val50Met) mutation by different diagnostic tests for three years. The carriers were followed by neurological examination, nerve conduction studies, sympathetic skin response test, heart rate variability, SFN-SIQ and DN4 questionnaires, quantitative sensory testing (QST), skin biopsy and in vivo corneal confocal microscopy. Nerve conduction studies, sympathetic skin response test and heart rate variability were normal in all for three years. Baseline QST and SFN-SIQ were normal but became abnormal during follow-up of two individuals who developed small fiber neuropathy symptoms. Baseline intraepidermal nerve fiber density was low in three carriers and decreased to below normative values in all during follow-up, while corneal sub-basal nerve density was low in all carriers compared to controls during the entire follow-up. Thus, our study showed that SFN-SIQ and QST are useful diagnostic tools to detect the transition to symptomatic ATTRv-polyneuropathy.
Copyright © 2021 Elsevier B.V. All rights reserved.
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Racial Differences in Val122Ile associated Transthyretin Cardiac Amyloidosis.
J Card Fail2021 Dec;():. doi: S1071-9164(21)00527-3.
Batra Jaya, Rosenblum Hannah, Cappelli Francesco, Zampieri Mattia, Olivotto Iacopo, Griffin Jan M, Saith Sunil E, Teruya Sergio, Santos Jeffeny De Los, Argiro Alessia, Burkhoff Daniel, Perfetto Federico, Maurer Mathew S,
Abstract
BACKGROUND:
The valine-to-isoleucine substitution (Val122Ile) is the most common variant of transthyretin (TTR) amyloidosis in the U.S., primarily affecting individuals of African descent. This variant has recently been identified in a cluster of White individuals in Italy.
METHODS AND RESULTS:
Clinical phenotype and chamber performance of Black and White individuals with Val122Ile TTR cardiac amyloidosis (ATTR-CA) were compared. Compared to White patients (n=17), Black individuals (n=53) had lower systolic blood pressures (110 vs. 131 mmHg, p
CONCLUSION:
Despite presenting at a similar age to White patients, Black individuals with Val122Ile associated ATTR-CA have a greater degree of cardiac chamber dysfunction at the time of diagnosis due to impaired ventricular capacitance. Whether these differences are attributable to amyloidosis or other cardiovascular disease requires further study.
Copyright © 2021 Elsevier Inc. All rights reserved.
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Usefulness of quantitative Tc-pyrophosphate SPECT/CT for predicting the prognosis of patients with wild-type transthyretin cardiac amyloidosis.
Jpn J Radiol2022 Jan;():. doi: 10.1007/s11604-021-01221-6.
Ogasawara Kouji, Shiraishi Shinya, Tsuda Noriko, Sakamoto Fumi, Oda Seitarou, Takashio Seiji, Tsujita Kenichi, Hirai Toshinori,
Abstract
PURPOSE:
Wild-type transthyretin-related amyloidosis cardiomyopathy (ATTRwt-CM) is an increasingly recognized cause of heart failure especially in elderly patients. The purpose of the present study was to determine retrospectively whether the quantitative indices of Tc-pyrophosphate (PYP) SPECT/CT help to predict the prognosis of ATTRwt-CM patients when compared with other clinical parameters.
MATERIALS AND METHODS:
Sixty-eight patients with biopsy-proven ATTRwt-CM who underwent PYP SPECT/CT were enrolled. Baseline clinical characteristics, echocardiographic parameters, and qualitative and/or quantitative indices of planar and SPECT/CT imaging in PYP scintigraphy for each patient were included. For quantitative analysis of SPECT/CT, the accumulation ratio of PYP in the septum, posterior, anterior, lateral, and apex walls to the cavity pool was calculated as the septal wall-to-cavity ratio (Se/C), lateral wall-to-cavity ratio (La/C), anterior wall-to-cavity ratio (An/C), inferior wall-to-cavity ratio (In/C), and apical wall-to-cavity ratio (Ap/C), respectively. Endpoints for prognostic accuracy evaluation were cardiac death or hospitalization due to heart failure. Event-free survival rate was evaluated through Cox proportional hazards regression analysis, providing estimated hazard ratios (HRs) with 95% confidence intervals (CIs) and Kaplan-Meier curves.
RESULTS:
High-sensitivity cardiac troponin T (hs-cTnT), La/C, age, interventricular septal thickness in diastole, and E/e' ratio in the septal wall were significantly associated with event-free survival (P?0.05). For a multivariable Cox proportional hazards analysis, hs-cTnT (HR 1.153; 95% CI 1.034-1.286; P?0.01), La/C (HR 2.091; 95% CI 1.012-4.322; P?=?0.046), and age (HR 1.116; 95% CI 1.007-1.238; P?=?0.037) were significant independent prognostic factors.
CONCLUSION:
This study indicated that the quantitative indices of PYP SPECT/CT can help to predict the prognosis of ATTRwt-CM patients.
© 2021. The Author(s).
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Tissue Characterization in Cardiology: Moving Beyond Function.
Adv Exp Med Biol2021 ;1337():89-97. doi: 10.1007/978-3-030-78771-4_11.
Markousis-Mavrogenis George, Bacopoulou Flora, Vlachakis Dimitrios, Mavrogeni Sophie,
Abstract
Cardiovascular Magnetic Resonance (CMR) offers accurate and highly reproducible tissue characterization, beyond cardiac function. Late gadolinium enhancement (LGE), although represents a noninvasive biopsy for fibrosis quantification, it is unable to detect diffuse myocardial disease. Native T1 mapping and extracellular volume fraction (ECV) are able to provide important information about processes involving both myocardial cells and interstitium that otherwise cannot be identified. Changes in myocardial native T1 mapping reflect cardiac diseases such as acute coronary syndromes, myocardial infarction, myocarditis, diffuse fibrosis, systemic disease such as cardiac amyloidosis, all presented with high T1 and Anderson-Fabry disease and siderosis, presented with low T1 mapping. The ECV, an index generated by native and postcontrast T1 mapping, introduces a new way to measure the cellular and extracellular interstitial matrix (ECM). ECV has a prognostic value equal to Left ventricular ejection fraction (LVEF); however, LVEF underscores the interstitial matrix. This myocyte-ECM dichotomy has important implications for identifying therapeutic targets that are of great value for heart failure (HF) treatment. Furthermore, T2 mapping is superior compared with myocardial T1 and ECM for assessing the activity of myocarditis in recent-onset HF. These indices will affect significantly the clinical decision making. However, there is still lack of multicenter studies and community-wide approach including MRI vendors, clinicians, fundings, softwares, and contrast agent manufacturers.
© 2021. The Author(s), under exclusive license to Springer Nature Switzerland AG.
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Epidemiology of cardiomyopathies and incident heart failure in a population-based cohort study.
Heart2021 Dec;():. doi: heartjnl-2021-320181.
Brownrigg Jack Rw, Leo Vincenzo, Rose Joel, Low Eric, Richards Sarah, Carr-White Gerry, Elliott Perry M,
Abstract
AIMS:
The population prevalence of cardiomyopathies and the natural history of symptomatic heart failure (HF) and arrhythmia across cardiomyopathy phenotypes is poorly understood. Study aims were to estimate the population-diagnosed prevalence of cardiomyopathies and describe the temporal relationship between a diagnosis of cardiomyopathy with HF and arrhythmia.
METHODS:
People with cardiomyopathy (n=4116) were identified from linked electronic health records (~9?million individuals; 2000-2018) and categorised into hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM) and cardiac amyloidosis (CA). Cardiomyopathy point prevalence, rates of symptomatic HF and arrhythmia and timing relative to a diagnosis of cardiomyopathy were determined.
RESULTS:
In 2018, DCM was the most common cardiomyopathy. DCM and HCM were twice as common among men, with the reverse trend for ARVC. Between 2010 and 2018, prevalence increased for ARVC by 180% and HCM by 9%. At diagnosis, more patients with CA (66%), DCM (56%) and RCM (62%) had pre-existing HF compared with ARVC (29%) and HCM (27%). Among those free of HF at diagnosis of cardiomyopathy, annualised HF incidence was greatest in CA and DCM. Diagnoses of all cardiomyopathies clustered around the time of HF onset.
CONCLUSIONS:
The recorded prevalence of all cardiomyopathies increased over the past decade. Recognition of CA is generally preceded by HF, whereas individuals with ARVC or HCM more often developed HF after their cardiomyopathy diagnosis suggesting a more indolent course or better asymptomatic recognition. The clustering of HF and cardiomyopathy diagnoses suggests opportunities for presymptomatic or earlier diagnosis.
© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.
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Overcoming the Diagnostic Pitfalls of Women With Cardiac Amyloidosis.
JACC Heart Fail2022 Jan;10(1):71-72. doi: S2213-1779(21)00535-7.
Itzhaki Ben Zadok Osnat, Porter Avital,
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