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Hussien M, Bermudez F, Bering PT, Weissman G, Hays AG, Sheikh FH. Cardiac Magnetic Resonance Imaging in the Evaluation and Prognosis of Infiltrative Cardiomyopathies. J Cardiovasc Dev Dis 2025; 12:154. [PMID: 40278213 PMCID: PMC12028063 DOI: 10.3390/jcdd12040154] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/27/2025] [Revised: 03/30/2025] [Accepted: 04/08/2025] [Indexed: 04/26/2025] Open
Abstract
Advancements in cardiac magnetic resonance (CMR) imaging quality and availability have made it an essential tool in the care of individuals living with cardiomyopathies. CMR complements clinical suspicion, electrocardiogram patterns, and echocardiographic findings to help elucidate the etiology of cardiomyopathies and can also be used to prognosticate and follow treatment responses. In this review, we highlight the common CMR findings in cardiac amyloidosis, cardiac sarcoidosis, iron overload cardiomyopathy, and Fabry disease. We also summarize prognostic findings and additional potential roles for CMR in the management of infiltrative cardiomyopathies.
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Affiliation(s)
- Merna Hussien
- Department of Cardiology, MedStar Washington Hospital Center, Washington, DC 20010, USA
- School of Medicine, Georgetown University, Washington, DC 20007, USA
| | | | - Patrick T. Bering
- Department of Cardiology, MedStar Washington Hospital Center, Washington, DC 20010, USA
- School of Medicine, Georgetown University, Washington, DC 20007, USA
| | - Gaby Weissman
- Department of Cardiology, MedStar Washington Hospital Center, Washington, DC 20010, USA
- School of Medicine, Georgetown University, Washington, DC 20007, USA
| | - Allison G. Hays
- Department of Cardiology, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA
| | - Farooq H. Sheikh
- Department of Cardiology, MedStar Washington Hospital Center, Washington, DC 20010, USA
- School of Medicine, Georgetown University, Washington, DC 20007, USA
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Wu Y, Wang X, Gào X, Xu L, Wang B, Cai Z. Prognostic factors in Chinese patients with immunoglobulin light chain amyloidosis: a scoping review and meta-analysis. Ann Med 2024; 56:2386635. [PMID: 39129426 PMCID: PMC11321117 DOI: 10.1080/07853890.2024.2386635] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/28/2023] [Revised: 03/07/2024] [Accepted: 03/15/2024] [Indexed: 08/13/2024] Open
Abstract
OBJECTIVE This scoping review and meta-analysis aimed to map the evidence regarding prognostic factors in Chinese patients with immunoglobulin light chain (AL) amyloidosis and to identify current research gaps. METHODS We searched EMBASE, PubMed, and CNKI databases from their inception to 15 September 2021. All studies investigated the association between any prognostic factor and target outcomes, including overall survival (OS), progression-free survival (PFS), and end-stage renal disease (ESRD) in Chinese patients with AL amyloidosis. RESULTS This scoping review included 52 studies, of which 44 with 6,432 patients contributed to the multivariate prognostic analysis. Multivariate analysis identified a total of 106 factors that correlated with OS, 16 factors with PFS, and 18 factors with ESRD. Five prognostic factors were significantly associated with PFS, and 11 prognostic factors were significantly associated with ESRD. Meta-analysis was only available for prognostic factors without heterogeneous cutoff values, for which hazard ratios (HRs) and their 95% confidence intervals (CIs) were reported. Meta-analysis showed that bone marrow plasma cells (BMCs) (HR: 1.96, 95% CI: 1.21-3.19, p < 0.05) and interventricular septal thickness (IVST) (HR: 1.23, 95% CI: 1.10-1.38, p < 0.05) were independently associated with OS. CONCLUSION The significant prognostic factors associated with OS, PFS, and ESRD in Chinese patients with AL amyloidosis were related to plasma cell tumor load, biological characteristics, cardiac involvement, renal involvement, population characteristics, and treatment. Further studies should explore additional prognostic factors in patients with AL amyloidosis to develop prognostic models.
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Affiliation(s)
- Yu Wu
- West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Xiaohong Wang
- Xi’an Janssen Pharmaceutical Ltd, Beijing, Chaoyang District, China
| | - Xīn Gào
- Xi’an Janssen Pharmaceutical Ltd, Beijing, Chaoyang District, China
| | - Lingjie Xu
- Xi’an Janssen Pharmaceutical Ltd, Beijing, Chaoyang District, China
| | - Bin Wang
- Xi’an Janssen Pharmaceutical Ltd, Beijing, Chaoyang District, China
| | - Zhen Cai
- The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang, China
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Hu M, Shen Y, Yu H, Song Y, Zheng T, Hong D, Gong L. Prognostic value of cardiac magnetic resonance imaging feature tracking technology in patients with light chain amyloidosis. Clin Radiol 2024; 79:e239-e246. [PMID: 37953095 DOI: 10.1016/j.crad.2023.10.016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2022] [Revised: 06/27/2023] [Accepted: 10/12/2023] [Indexed: 11/14/2023]
Abstract
AIM To undertake a meta-analysis of the prognostic value of cardiac magnetic resonance imaging feature tracking (CMR-FT) in patients with light-chain cardiac amyloidosis (LCA). MATERIALS AND METHODS A systematic search was conducted in PubMed, EMBASE, Web of Science, and the Cochrane Library. All analyses were conducted using RevMan 5.3 software. RESULTS Eight studies were included with 663 patients. For the left ventricle, the results showed that CMR-FT was statistically significant in predicting death, with less impaired global circumferential (GCS), radial (GRS) and longitudinal (GLS) strain in survivors of LCA (odds ratio [OR] 1.17, 95% confidence interval [CI] 1.09-1.25; 0.95, 0.93-0.96; 1.12, 1.05-1.20, all p<0.001). For ejection fraction (EF) and mass index, surviving patients had higher EFs and mass index (OR 0.96, 95% CI 0.96-0.97; 1.01, 1.01-1.02). For the right ventricle, the results showed that CMR-FT was statistically significant in predicting death, with less impaired GLS and GRS in survivors of LCA (OR 1.11, 95% CI 1.08-1.15; 0.93, 0.90-0.96, all p<0.001). Surviving patients had higher EFs (OR 0.97, 95% CI 0.96-0.98, p<0.001). Upon removing the studies one by one, there was no significant change in the results of the study. Both analyses showed no apparent publication deviation on funnel plots. CONCLUSION Parameters derived from CMR-FT technology are promising new predictors for LCA, and are easily available and reliable. Patients with poor myocardial deformability are at highest risk of death.
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Affiliation(s)
- M Hu
- Medical Imaging Center, The Second Affiliated Hospital of Nanchang University, No. 1 Min-de Road, Donghu District, Nanchang, 33000, Jiangxi Province, People's Republic of China
| | - Y Shen
- Department of Neurology, The First Affiliated Hospital of Nanchang University, No. 17, Yongwai Zheng Street, Donghu District, Nanchang 330006, Jiangxi Province, People's Republic of China
| | - H Yu
- Department of Radiology, Jiangxi Province Medical Imaging Research Institute, The First Affiliated Hospital of Nanchang University, No. 17, Yongwai Zheng Street, Donghu District, Nanchang 330006, Jiangxi Province, People's Republic of China
| | - Y Song
- Medical Imaging Center, The Second Affiliated Hospital of Nanchang University, No. 1 Min-de Road, Donghu District, Nanchang, 33000, Jiangxi Province, People's Republic of China
| | - T Zheng
- Medical Imaging Center, The Second Affiliated Hospital of Nanchang University, No. 1 Min-de Road, Donghu District, Nanchang, 33000, Jiangxi Province, People's Republic of China
| | - D Hong
- Department of Neurology, The First Affiliated Hospital of Nanchang University, No. 17, Yongwai Zheng Street, Donghu District, Nanchang 330006, Jiangxi Province, People's Republic of China.
| | - L Gong
- Medical Imaging Center, The Second Affiliated Hospital of Nanchang University, No. 1 Min-de Road, Donghu District, Nanchang, 33000, Jiangxi Province, People's Republic of China.
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Vidal-Perez R, Brandão M, Zaher W, Casado-Arroyo R, Bouzas-Mosquera A, Fontes-Carvalho R, Vazquez-Rodriguez JM. Value of cardiac magnetic resonance on the risk stratification of cardiomyopathies. World J Cardiol 2023; 15:487-499. [PMID: 37900906 PMCID: PMC10600791 DOI: 10.4330/wjc.v15.i10.487] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/30/2023] [Revised: 09/18/2023] [Accepted: 09/27/2023] [Indexed: 10/24/2023] Open
Abstract
Cardiomyopathies represent a diverse group of heart muscle diseases with varying etiologies, presenting a diagnostic challenge due to their heterogeneous manifestations. Regular evaluation using cardiac imaging techniques is imperative as symptoms can evolve over time. These imaging approaches are pivotal for accurate diagnosis, treatment planning, and optimizing prognostic outcomes. Among these, cardiovascular magnetic resonance (CMR) stands out for its ability to provide precise anatomical and functional assessments. This manuscript explores the significant contributions of CMR in the diagnosis and management of patients with cardiomyopathies, with special attention to risk stratification. CMR's high spatial resolution and tissue characterization capabilities enable early detection and differentiation of various cardiomyopathy subtypes. Additionally, it offers valuable insights into myocardial fibrosis, tissue viability, and left ventricular function, crucial parameters for risk stratification and predicting adverse cardiac events. By integrating CMR into clinical practice, clinicians can tailor patient-specific treatment plans, implement timely interventions, and optimize long-term prognosis. The non-invasive nature of CMR reduces the need for invasive procedures, minimizing patient discomfort. This review highlights the vital role of CMR in monitoring disease progression, guiding treatment decisions, and identifying potential complications in patients with cardiomyopathies. The utilization of CMR has significantly advanced our understanding and management of these complex cardiac conditions, leading to improved patient outcomes and a more personalized approach to care.
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Affiliation(s)
- Rafael Vidal-Perez
- Servicio de Cardiología, Unidad de Imagen y Función Cardíaca, Complexo Hospitalario Universitario A Coruña Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), A Coruña 15006, Galicia, Spain.
| | - Mariana Brandão
- Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia 4434-502, Portugal
| | - Wael Zaher
- Department of Cardiology, Hôpital Erasme, Université Libre de Bruxelles, Brussels 1070, Brussels, Belgium
| | - Ruben Casado-Arroyo
- Department of Cardiology, Hôpital Erasme, Université Libre de Bruxelles, Brussels 1070, Brussels, Belgium
| | - Alberto Bouzas-Mosquera
- Servicio de Cardiología, Unidad de Imagen y Función Cardíaca, Complexo Hospitalario Universitario A Coruña Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), A Coruña 15006, Galicia, Spain
| | - Ricardo Fontes-Carvalho
- Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia 4434-502, Portugal
- Cardiovascular R&D Centre - UnIC@RISE, Department of Surgery and Physiology, Faculty of Medicine of the University of Porto, Porto 4200-319, Portugal
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Brito D, Albrecht FC, de Arenaza DP, Bart N, Better N, Carvajal-Juarez I, Conceição I, Damy T, Dorbala S, Fidalgo JC, Garcia-Pavia P, Ge J, Gillmore JD, Grzybowski J, Obici L, Piñero D, Rapezzi C, Ueda M, Pinto FJ. World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM). Glob Heart 2023; 18:59. [PMID: 37901600 PMCID: PMC10607607 DOI: 10.5334/gh.1262] [Citation(s) in RCA: 37] [Impact Index Per Article: 18.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/10/2023] [Accepted: 08/12/2023] [Indexed: 10/31/2023] Open
Abstract
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive and fatal condition that requires early diagnosis, management, and specific treatment. The availability of new disease-modifying therapies has made successful treatment a reality. Transthyretin amyloid cardiomyopathy can be either age-related (wild-type form) or caused by mutations in the TTR gene (genetic, hereditary forms). It is a systemic disease, and while the genetic forms may exhibit a variety of symptoms, a predominant cardiac phenotype is often present. This document aims to provide an overview of ATTR-CM amyloidosis focusing on cardiac involvement, which is the most critical factor for prognosis. It will discuss the available tools for early diagnosis and patient management, given that specific treatments are more effective in the early stages of the disease, and will highlight the importance of a multidisciplinary approach and of specialized amyloidosis centres. To accomplish these goals, the World Heart Federation assembled a panel of 18 expert clinicians specialized in TTR amyloidosis from 13 countries, along with a representative from the Amyloidosis Alliance, a patient advocacy group. This document is based on a review of published literature, expert opinions, registries data, patients' perspectives, treatment options, and ongoing developments, as well as the progress made possible via the existence of centres of excellence. From the patients' perspective, increasing disease awareness is crucial to achieving an early and accurate diagnosis. Patients also seek to receive care at specialized amyloidosis centres and be fully informed about their treatment and prognosis.
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Affiliation(s)
- Dulce Brito
- Department of Cardiology, Centro Hospitalar Universitário Lisboa Norte, CAML, CCUL@RISE, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
| | - Fabiano Castro Albrecht
- Dante Pazzanese Institute of Cardiology – Cardiac Amyloidosis Center Dante Pazzanese Institute, São Paulo, Brazil
| | | | - Nicole Bart
- St Vincent’s Hospital, Victor Chang Cardiac Research Institute, University of New South Wales, Sydney, Australia
| | - Nathan Better
- Cabrini Health, Malvern, Royal Melbourne Hospital, Parkville, Monash University and University of Melbourne, Victoria, Australia
| | | | - Isabel Conceição
- Department of Neurosciences and Mental Health, CHULN – Hospital de Santa Maria, Portugal
- Centro de Estudos Egas Moniz Faculdade de Medicina da Universidade de Lisboa Portugal, Portugal
| | - Thibaud Damy
- Department of Cardiology, DHU A-TVB, CHU Henri Mondor, AP-HP, INSERM U955 and UPEC, Créteil, France
- Referral Centre for Cardiac Amyloidosis, GRC Amyloid Research Institute, Reseau amylose, Créteil, France. Filière CARDIOGEN
| | - Sharmila Dorbala
- Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
- Cardiac Amyloidosis Program, Cardiovascular Division, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
- CV imaging program, Cardiovascular Division and Department of Radiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA
| | | | - Pablo Garcia-Pavia
- Hospital Universitario Puerta de Hierro Majadahonda, IDIPHISA, CIBERCV, Madrid, Spain
- Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain
| | - Junbo Ge
- Department of Cardiology, Zhongshan Hospital, Fudan University, Shanghai Institute of Cardiovascular Diseases, Shanghai, China
| | - Julian D. Gillmore
- National Amyloidosis Centre, University College London, Royal Free Campus, United Kingdom
| | - Jacek Grzybowski
- Department of Cardiomyopathy, National Institute of Cardiology, Warsaw, Poland
| | - Laura Obici
- Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
| | | | - Claudio Rapezzi
- Cardiovascular Institute, University of Ferrara, Ferrara, Italy
| | - Mitsuharu Ueda
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan
| | - Fausto J. Pinto
- Department of Cardiology, Centro Hospitalar Universitário Lisboa Norte, CAML, CCUL@RISE, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal
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Hahn RT, Lerakis S, Delgado V, Addetia K, Burkhoff D, Muraru D, Pinney S, Friedberg MK. Multimodality Imaging of Right Heart Function: JACC Scientific Statement. J Am Coll Cardiol 2023; 81:1954-1973. [PMID: 37164529 DOI: 10.1016/j.jacc.2023.03.392] [Citation(s) in RCA: 50] [Impact Index Per Article: 25.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2023] [Revised: 03/01/2023] [Accepted: 03/02/2023] [Indexed: 05/12/2023]
Abstract
Right ventricular (RV) size and function assessed by multimodality imaging are associated with outcomes in a variety of cardiovascular diseases. Understanding RV anatomy and physiology is essential in appreciating the strengths and weaknesses of current imaging methods and gives these measurements greater context. The adaptation of the right ventricle to different types and severity of stress, particularly over time, is specific to the cardiovascular disease process. Multimodality imaging parameters, which determine outcomes, reflect the ability to image the initial and longitudinal RV response to stress. This paper will review the standard and novel imaging methods for assessing RV function and the impact of these parameters on outcomes in specific disease states.
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Affiliation(s)
- Rebecca T Hahn
- Department of Medicine, Columbia University Medical Center/NewYork-Presbyterian Hospital, New York, New York, USA.
| | | | - Victoria Delgado
- Hospital University Germans Trias i Pujol Hospital, Badalona, Barcelona, Spain
| | - Karima Addetia
- Department of Medicine, University of Chicago, Chicago, Illinois, USA
| | | | - Denisa Muraru
- Department of Cardiology, Istituto Auxologico Italiano, IRCCS, Milan, Italy; Department of Medicine and Surgery, University of Milano-Bicocca, Milan, Italy
| | - Sean Pinney
- Department of Medicine, University of Chicago, Chicago, Illinois, USA
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Left ventricular assist device in cardiac amyloidosis: friend or foe? Heart Fail Rev 2023; 28:359-365. [PMID: 36451061 DOI: 10.1007/s10741-022-10288-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/21/2022] [Indexed: 12/04/2022]
Abstract
The prevalence of cardiac amyloidosis has progressively increased over the last years, being recognized as a significant cause of heart failure. In fact, the management of advanced heart failure is a cornerstone treatment of amyloid cardiomyopathy due to the frequent delay in its diagnosis. Left ventricular assist devices (LVADs) have been gaining importance in the scenario of end-stage heart failure, representing an alternative to heart transplant. However, only few studies have investigated the role of LVAD in restrictive cardiomyopathies such as cardiac amyloidosis, since there are several problems to consider. In fact, both anatomical factors and the restrictive physiology of this condition make LVAD implant a relevant challenge in this subset of patients. Furthermore, due to the systemic involvement of amyloidosis, several factors have to be considered after LVAD implant, such as an increased risk of bleeding and right ventricular failure. This review attempts to summarize the current evidence of LVAD in cardiac amyloidosis, especially focusing on the challenges that this cardiomyopathy imposes both to the implant and to its management thereafter.
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Khedraki R, Robinson AA, Jordan T, Grodin JL, Mohan RC. A Review of Current and Evolving Imaging Techniques in Cardiac Amyloidosis. CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE 2023; 25:43-63. [PMID: 38239280 PMCID: PMC10795761 DOI: 10.1007/s11936-023-00976-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 12/30/2022] [Indexed: 03/07/2023]
Abstract
Purpose of review Establishing an early, efficient diagnosis for cardiac amyloid (CA) is critical to avoiding adverse outcomes. We review current imaging tools that can aid early diagnosis, offer prognostic information, and possibly track treatment response in CA. Recent findings There are several current conventional imaging modalities that aid in the diagnosis of CA including electrocardiography, echocardiography, bone scintigraphy, cardiac computed tomography (CT), and cardiac magnetic resonance (CMR) imaging. Advanced imaging techniques including left atrial and right ventricular strain, and CMR T1 and T2 mapping as well as ECV quantification may provide alternative non-invasive means for diagnosis, more granular prognostication, and the ability to track treatment response. Summary Leveraging a multimodal imaging toolbox is integral to the early diagnosis of CA; however, it is important to understand the unique role and limitations posed by each modality. Ongoing studies are needed to help identify imaging markers that will lead to an enhanced ability to diagnose, subtype and manage this condition.
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Affiliation(s)
- Rola Khedraki
- Section of Advanced Heart Failure, Division of Cardiovascular Medicine, Scripps Clinic, Prebys Cardiovascular Institute, 9898 Genesee Ave., AMP-300, La Jolla, San Diego, CA 92037, USA
| | - Austin A. Robinson
- Section of Advanced Heart Failure, Division of Cardiovascular Medicine, Scripps Clinic, Prebys Cardiovascular Institute, 9898 Genesee Ave., AMP-300, La Jolla, San Diego, CA 92037, USA
| | - Timothy Jordan
- Section of Advanced Heart Failure, Division of Cardiovascular Medicine, Scripps Clinic, Prebys Cardiovascular Institute, 9898 Genesee Ave., AMP-300, La Jolla, San Diego, CA 92037, USA
| | - Justin L. Grodin
- Division of Cardiology, Department of Medicine, University of Texas Southwestern Medical Center, Dallas, USA
| | - Rajeev C. Mohan
- Section of Advanced Heart Failure, Division of Cardiovascular Medicine, Scripps Clinic, Prebys Cardiovascular Institute, 9898 Genesee Ave., AMP-300, La Jolla, San Diego, CA 92037, USA
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The Clinical Characteristics of Immunoglobulin Light Chain Amyloidosis in the Chinese Population: A Systematic Scoping Review. HEMATO 2022. [DOI: 10.3390/hemato4010002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Immunoglobulin light chain (AL) amyloidosis is the most common type of systemic amyloidosis in China and is associated with increased morbidity and a poor prognosis. However, the clinical characteristics of Chinese patients with AL amyloidosis have not been systematically investigated. This scoping review aimed to summarize the available literature regarding the clinical characteristics of patients with AL amyloidosis and identify potential knowledge gaps. We searched three electronic databases from inception to 7 February 2021. PICOS (Patient, Intervention, Comparison, Outcome and Study) design structure was used to formulate the data extraction. All statistical calculations and analyses were performed with R (version 3.6.0). Sixty-seven articles with 5022 patients were included. Results suggest Chinese patients were younger (57 years) at the time of diagnosis when compared with other patient populations and were predominantly male (61.2%). The time interval from the onset of symptoms to diagnosis was between 6 and 12 months. It was found that 41.1% of Chinese patients with AL amyloidosis were diagnosed with an advanced stage III disease when diagnosed, and 20.2% had a concurrent disease. The most involved organs were the kidneys (84.3%) and the heart (62.5%). In conclusion, our study shows some similarities and differences with other studies on the clinical characteristics of Chinese patients with AL amyloidosis, including the age at diagnosis, Mayo stage, and organ involvement. However, a nationwide epidemiological investigation is still needed to provide a comprehensive overview of this patient population in China.
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Zhang Y, Zhu Y, Zhang M, Liu J, Wu G, Wang J, Sun X, Wang D, Jiang W, Xu L, Kang L, Song L. Implications of structural right ventricular involvement in patients with hypertrophic cardiomyopathy. EUROPEAN HEART JOURNAL. QUALITY OF CARE & CLINICAL OUTCOMES 2022; 9:34-41. [PMID: 35179204 DOI: 10.1093/ehjqcco/qcac008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/16/2022] [Revised: 02/08/2022] [Accepted: 02/11/2022] [Indexed: 12/15/2022]
Abstract
AIMS In the clinical practice, the right ventricular (RV) manifestations have received less attention in hypertrophic cardiomyopathy (HCM). This paper aimed to evaluate the risk prediction value and genetic characteristics of RV involvement in HCM patients. METHODS AND RESULTS A total of 893 patients with HCM were recruited. RV hypertrophy, RV obstruction, and RV late gadolinium enhancement were evaluated by echocardiography and/or cardiac magnetic resonance. Patients with any of the above structural abnormalities were identified as having RV involvement. All patients were followed with a median follow-up time of 3.0 years. The primary endpoint was cardiovascular death; the secondary endpoints were all-cause death and heart failure (HF)-related death. Survival analyses were conducted to evaluate the associations between RV involvement and the endpoints. Genetic testing was performed on 669 patients. RV involvement was recognized in 114 of 893 patients (12.8%). Survival analyses demonstrated that RV involvement was an independent risk factor for cardiovascular death (P = 0.002), all-cause death (P = 0.011), and HF-related death (P = 0.004). These outcome results were then confirmed by a sensitivity analysis. Genetic testing revealed a higher frequency of genotype-positive in patients with RV involvement (57.0% vs. 31.0%, P < 0.001), and the P/LP variants of MYBPC3 were more frequently identified in patients with RV involvement (30.4% vs. 12.0%, P < 0.001). Logistic analyses indicated the independent correlation between RV involvement and these genetic factors. CONCLUSION RV involvement was an independent risk factor for cardiovascular death, all-cause death and HF-related death in HCM patients. Genetic factors might contribute to RV involvement in HCM.
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Affiliation(s)
- Yu Zhang
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China
| | - Yuming Zhu
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China
| | - Mo Zhang
- Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China
| | - Jie Liu
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China
| | - Guixin Wu
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China
| | - Jizheng Wang
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China
| | - Xiaolu Sun
- Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China
| | - Dong Wang
- Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China
| | - Wen Jiang
- Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China
| | - Lianjun Xu
- Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China
| | - Lianming Kang
- Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China
| | - Lei Song
- State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China.,Cardiomyopathy Ward, State Key Laboratory of Cardiovascular Disease, National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, 167, Beilishilu, Xicheng District, 100037 Beijing, China.,National Clinical Research Center of Cardiovascular Diseases, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongdan Santiao No. 9, 100006 Beijing, China
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Scheel PJ, Mukherjee M, Hays AG, Vaishnav J. Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis. Front Cardiovasc Med 2022; 9:787618. [PMID: 35402557 PMCID: PMC8989413 DOI: 10.3389/fcvm.2022.787618] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2021] [Accepted: 02/14/2022] [Indexed: 11/13/2022] Open
Abstract
Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy resulting from deposition of misfolded immunoglobulin light chains (AL-CA) or transthyretin (ATTR-CA) proteins in the myocardium. Survival varies between the different subtypes of amyloidosis and degree of cardiac involvement, but accurate diagnosis is essential to ensure initiation of therapeutic interventions that may slow or potentially prevent morbidity and mortality in these patients. As there are now effective treatment options for CA, identifying underlying disease pathogenesis is crucial and can be guided by multimodality imaging techniques such as echocardiography, magnetic resonance imaging, and nuclear scanning modalities. However, as use of cardiac imaging is becoming more widespread, understanding optimal applications and potential shortcomings is increasingly important. Additionally, certain imaging modalities can provide prognostic information and may affect treatment planning. In patients whom imaging remains non-diagnostic, tissue biopsy, specifically endomyocardial biopsy, continues to play an essential role and can facilitate accurate and timely diagnosis such that appropriate treatment can be started. In this review, we examine the multimodality imaging approach to the diagnosis of CA with particular emphasis on the prognostic utility and limitations of each imaging modality. We also discuss how imaging can guide the decision to pursue tissue biopsy for timely diagnosis of CA.
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12
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Slivnick J, Zareba KM, Varghese J, Truong V, Wallner AL, Tong MS, Hummel C, Mazur W, Rajpal S. Prevalence and haemodynamic profiles of pulmonary hypertension in cardiac amyloidosis. Open Heart 2022; 9:openhrt-2021-001808. [PMID: 35246499 PMCID: PMC8900043 DOI: 10.1136/openhrt-2021-001808] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2021] [Accepted: 01/09/2022] [Indexed: 11/04/2022] Open
Abstract
OBJECTIVES While cardiac amyloidosis (CA) classically involves the left ventricle (LV), less is known about its impact on the right ventricle (RV) and pulmonary vasculature. We performed a retrospective analysis to identify the prevalence and types of pulmonary hypertension (PH) profiles in CA and to determine haemodynamic and cardiovascular magnetic resonance (CMR) predictors of major adverse cardiovascular events (MACE). METHODS Patients with CA who underwent CMR and right heart catheterisation (RHC) within 1 year between 2010 and 2019 were included. Patients were assigned the following haemodynamic profiles based on RHC: no PH, precapillary PH, isolated postcapillary PH (IPCPH), or combined precapillary and postcapillary PH (CPCPH). The relationship between PH profile and MACE (death, heart failure hospitalisation) was assessed using survival analysis. CMR and RV parameters were correlated with MACE using Cox-regression analysis. RESULTS A total of 52 patients were included (age 69±9 years, 85% men). RHC was performed during biopsy in 44 (85%) and for clinical indications in 8 (15%) patients. Rates of no PH, precapillary PH, IPCPH and CPCPH were 5 (10%), 3 (6%), 29 (55%) and 15 (29%), respectively. Haemodynamic PH profile did not correlate with risk of death (p=0.98) or MACE (p=0.67). Transpulmonary gradient (TPG) (HR 0.88, CI 0.80 to 0.97), RV, (HR 0.95, CI 0.92 to 0.98) and LV ejection fraction (HR 0.95, CI 0.92 to 0.98) were significantly associated with MACE. CONCLUSIONS PH is highly prevalent in CA, even at the time of diagnosis. While IPCPH was most common, CPCPH is not infrequent. TPG and RV ejection fraction (RVEF) are prognostic markers in this population.
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Affiliation(s)
- Jeremy Slivnick
- Cardiovascular Medicine, University of Chicago Medicine, Chicago, Illinois, USA
| | - Karolina M Zareba
- Cardiovascular Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Juliet Varghese
- Cardiovascular Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Vien Truong
- Cardiology, Christ Hospital, Cincinnati, Ohio, USA
| | - Alexander L Wallner
- Cardiovascular Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Matthew S Tong
- Cardiovascular Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | - Christopher Hummel
- Cardiovascular Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA
| | | | - Saurabh Rajpal
- Internal Medicine/Cardiology, Ohio State University Wexner Medical Center, Columbus, Ohio, USA.,Cardiology, Nationwide Children's Hospital Doctors Hospital Pediatric Residency Training Program, Columbus, Ohio, USA
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13
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Santarelli MF, Scipioni M, Genovesi D, Giorgetti A, Marzullo P, Landini L. Imaging Techniques as an Aid in the Early Detection of Cardiac Amyloidosis. Curr Pharm Des 2021; 27:1878-1889. [PMID: 32787756 DOI: 10.2174/1381612826666200813133557] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Accepted: 06/23/2020] [Indexed: 11/22/2022]
Abstract
The idea that performing a proper succession of imaging tests and techniques allows an accurate and early diagnosis of cardiac amyloidosis, avoiding the need to perform the myocardial biopsy, is becoming increasingly popular. Furthermore, being imaging techniques non-invasive, it is possible to perform the follow-up of the pathology through repeated image acquisitions. In the present review, the various innovative imaging methodologies are presented, and it is discussed how they have been applied for early diagnosis of cardiac amyloidosis (CA), also to distinguish the two most frequent subtypes in CA: immunoglobulin light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR); this allows to perform the therapy in a targeted and rapid manner.
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Affiliation(s)
| | - M Scipioni
- CNR Institute of Clinical Physiology, Pisa, Italy
| | - D Genovesi
- Fondazione Toscana "G. Monasterio", Pisa, Italy
| | - A Giorgetti
- Fondazione Toscana "G. Monasterio", Pisa, Italy
| | - P Marzullo
- Fondazione Toscana "G. Monasterio", Pisa, Italy
| | - L Landini
- Fondazione Toscana "G. Monasterio", Pisa, Italy
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14
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Reddy A, Singh V, Karthikeyan B, Jiang L, Kristo S, Kattel S, Amuthan R, Pokharel S, Sharma UC. Biventricular Strain Imaging with Cardiac MRI in Genotyped and Histology Validated Amyloid Cardiomyopathy. CARDIOGENETICS 2021; 11:98-110. [PMID: 34336147 PMCID: PMC8318353 DOI: 10.3390/cardiogenetics11030011] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023] Open
Abstract
Cardiac amyloidosis (CA) is a common and potentially fatal infiltrative cardiomyopathy. Contrast-enhanced cardiac MRI (CMR) is used as a diagnostic tool. However, utility of CMR for the comprehensive analysis of biventricular strains and strain rates is not reported as extensively as echocardiography. In addition, RV strain analysis using CMR has not been described previously. Objectives We sought to study the global and regional indices of biventricular strain and strain rates in endomyocardial biopsy (EMB)-proven, genotyped cases of CA. Methods A database of 80 EMBs was curated from 2012 to 2019 based on histology. A total of 19 EMBs positive for CA were subjected to further tissue-characterization with histology, and compared with four normal biopsy specimens. Samples were genotyped for ATTR- or AL-subtypes. Five patients, with both echocardiography and contrast-enhanced CMR performed 72-h apart, were subjected to comprehensive analysis of biventricular strain and strain-rates. Results Histology confirmed that the selected samples were indeed positive for cardiac amyloid. Echocardiography showed reduced global and regional left-ventricular (LV) longitudinal strain indices. CMR with tissue-characterization of LV showed global reductions in circumferential, radial and longitudinal strains and strain-rates, following a general trend with the echocardiographic findings. The basal right-ventricular (RV) segments had reduced circumferential strains with no changes in longitudinal strain. Conclusions In addition to providing a clinical diagnosis of CA based on contrast clearance-dynamics, CMR can be a potent tool for accurate functional assessment of global and regional changes in strain and strain-rates involving both LV and RV. Further studies are warranted to validate and curate the strain imaging capacity of CMR in CA.
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Affiliation(s)
- Abhinay Reddy
- Department of Medicine, Division of Cardiology, Jacob’s School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA
| | - Vasvi Singh
- Department of Radiology, Division of Nuclear Medicine, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA 02115, USA
| | - Badri Karthikeyan
- Department of Medicine, Division of Cardiology, Jacob’s School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA
| | - Leyi Jiang
- Department of Medicine, Division of Cardiology, Jacob’s School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA
| | - Silva Kristo
- Department of Pathology and Laboratory Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, NY 14203, USA
| | - Sharma Kattel
- Department of Medicine, Division of Cardiology, Jacob’s School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA
- Department of Medicine, Division of Cardiology, Yale School of Medicine, New Haven, CT 06510, USA
| | - Ram Amuthan
- Department of Medicine, Division of Cardiology, Jacob’s School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA
| | - Saraswati Pokharel
- Department of Pathology and Laboratory Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, NY 14203, USA
| | - Umesh C. Sharma
- Department of Medicine, Division of Cardiology, Jacob’s School of Medicine and Biomedical Sciences, Buffalo, NY 14203, USA
- Correspondence:
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15
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Abstract
Purpose of Review The purpose of this review is to summarize the application of cardiac magnetic resonance (CMR) in the diagnostic and prognostic evaluation of patients with heart failure (HF). Recent Findings CMR is an important non-invasive imaging modality in the assessment of ventricular volumes and function and in the analysis of myocardial tissue characteristics. The information derived from CMR provides a comprehensive evaluation of HF. Its unique ability of tissue characterization not only helps to reveal the underlying etiologies of HF but also offers incremental prognostic information. Summary CMR is a useful non-invasive tool for the diagnosis and assessment of prognosis in patients suffering from heart failure.
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Affiliation(s)
- Chuanfen Liu
- Cardiovascular Division, Department of Medicine, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA USA
- Department of Cardiology, Peking University People’s Hospital, Beijing, China
| | - Victor A. Ferrari
- Cardiovascular Division, Department of Medicine, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA USA
| | - Yuchi Han
- Cardiovascular Division, Department of Medicine, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, PA USA
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16
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Vidal-Perez R, Vázquez-García R, Barge-Caballero G, Bouzas-Mosquera A, Soler-Fernandez R, Larrañaga-Moreira JM, Crespo-Leiro MG, Vazquez-Rodriguez JM. Diagnostic and prognostic value of cardiac imaging in amyloidosis. World J Cardiol 2020; 12:599-614. [PMID: 33391613 PMCID: PMC7754383 DOI: 10.4330/wjc.v12.i12.599] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2020] [Revised: 09/28/2020] [Accepted: 11/17/2020] [Indexed: 02/06/2023] Open
Abstract
Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years. Different types of amyloidosis can affect the heart. Transthyretin amyloidosis and light chain amyloidosis are the two most common types of cardiac amyloidosis. These entities have a poor prognosis, so accurate diagnostic techniques are imperative for determining an early therapeutic approach. Recent advances in cardiac imaging and diagnostic strategies show that these tools are safe and can avoid the use of invasive diagnostic techniques to histological confirmation, such as endomyocardial biopsy. We performed a review on the diagnostic and prognostic implications of different cardiac imaging techniques in cardiac amyloidosis. We mainly focus on reviewing echocardiography, cardiac magnetic resonance, computed tomography and nuclear imaging techniques and the different safety measurements that can be done with each of them.
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Affiliation(s)
- Rafael Vidal-Perez
- Servicio de Cardiología, Unidad de Imagen y Función Cardíaca, Complexo Hospitalario Universitario A Coruña (CHUAC), Santiago de Compostela 15706, A Coruña, Spain
| | - Raquel Vázquez-García
- Servicio de Cardiología, Complexo Hospitalario Universitario A Coruña (CHUAC), A Coruña 15006, A Coruña, Spain.
| | - Gonzalo Barge-Caballero
- Servicio de Cardiología, Complexo Hospitalario Universitario A Coruña, Unidad de Insuficiencia Cardiaca Avanzada y Trasplante Cardiaco, Instituto de Investigación Biomédica de A Coruña (INIBIC), Centro de Investigación Biomédica en Red (CIBERCV)-Instituto de Salud Carlos III, A Coruña 15006, A Coruña, Spain
| | - Alberto Bouzas-Mosquera
- Servicio de Cardiología, Unidad de Imagen y Función Cardíaca, Complexo Hospitalario Universitario A Coruña (CHUAC), Santiago de Compostela 15706, A Coruña, Spain
| | - Rafaela Soler-Fernandez
- Servicio de Cardiología, Complexo Hospitalario Universitario A Coruña (CHUAC), A Coruña 15006, A Coruña, Spain
| | | | - Maria Generosa Crespo-Leiro
- Servicio de Cardiología, Complexo Hospitalario Universitario A Coruña, Unidad de Insuficiencia Cardiaca Avanzada y Trasplante Cardiaco, Instituto de Investigación Biomédica de A Coruña (INIBIC), Centro de Investigación Biomédica en Red (CIBERCV)-Instituto de Salud Carlos III, A Coruña 15006, A Coruña, Spain
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17
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Cavigli L, Focardi M, Cameli M, Mandoli GE, Mondillo S, D'Ascenzi F. The right ventricle in “Left-sided” cardiomyopathies: The dark side of the moon. Trends Cardiovasc Med 2020; 31:476-484. [DOI: 10.1016/j.tcm.2020.10.003] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/14/2020] [Revised: 09/26/2020] [Accepted: 10/09/2020] [Indexed: 02/07/2023]
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18
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Wan K, Lin J, Guo X, Song R, Wang J, Xu Y, Li W, Cheng W, Sun J, Zhang Q, Han Y, Chen Y. Prognostic Value of Right Ventricular Dysfunction in Patients With AL Amyloidosis: Comparison of Different Techniques by Cardiac Magnetic Resonance. J Magn Reson Imaging 2020; 52:1441-1448. [PMID: 32691470 DOI: 10.1002/jmri.27200] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2020] [Revised: 04/30/2020] [Accepted: 05/01/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Right ventricular (RV) dysfunction is common in patients with amyloid light-chain (AL) amyloidosis. While cardiac MRI is the reference standard tool for RV assessment, there are a number of measures of RV function that can be evaluated and it is yet unknown which of these results in the highest prognostic performance in AL amyloidosis. PURPOSE To examine the prognostic value of various measures of RV function in a bid to find which best predicts outcome in AL amyloidosis. STUDY TYPE Single-center, prospective. SUBJECTS In all, 129 patients (mean age, 58 ± 11 years; 61.2% men) with biopsy-proven AL amyloidosis. FIELD STRENGTH/SEQUENCE 3.0T / balanced steady-state free-precession cine. ASSESSMENT RV ejection fraction (EF), RV fractional area change (FAC), RV long axis strain (LAS), RV free wall longitudinal strain (FWS), RV global longitudinal strain (GLS), and tricuspid annular plane systolic excursion (TAPSE). STATISTICAL TESTS Mann-Whitney U-tests, Student's t-tests, receiver-operating characteristic curves, Kaplan-Meier curves, Cox proportional hazards regression models, and C-statistics. RESULTS During the median follow-up period of 38.0 months (interquartile range, 18.5-58.0 months), all-cause mortality occurred in 95 patients (73.6%). The RVEF, RVGLS, TAPSE, RVFAC, and RVFWS were significant predictors of outcome in univariate Cox regression (all P < 0.001). After adjusting for New York Heart Association (NYHA) class, Mayo staging 2004, left ventricular (LV) EF, and LV mass index, RVFWS (HR [hazard ratio] =1.074; 95% CI [confidence interval]: 1.041-1.108; P < 0.001) was an independent predictor of all-cause mortality and had a higher C-statistic (0.753) compared to the model including RVEF (C-statistic = 0.724, P = 0.034), the model including RVFAC (C-statistic = 0.723, P = 0.033), and the model including RVGLS (C-statistic =0.733, P = 0.011). DATA CONCLUSION RV dysfunction appears to be an independent determinant of outcome in patients with AL amyloidosis. RVFWS is a better predictor of all-cause mortality than RVEF, RVFAC, or RVGLS. EVIDENCE LEVEL 2 TECHNICAL EFFICACY STAGE: 5.
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Affiliation(s)
- Ke Wan
- Department of Geriatrics and National Clinical Research Center for Geriatrics, West China Hospital, Sichuan University, Chengdu, China
| | - Jiayi Lin
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Xinli Guo
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Rizhen Song
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Jie Wang
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Yuanwei Xu
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Weihao Li
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Wei Cheng
- Department of Radiology, West China Hospital, Sichuan University, Chengdu, China
| | - Jiayu Sun
- Department of Radiology, West China Hospital, Sichuan University, Chengdu, China
| | - Qing Zhang
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China
| | - Yuchi Han
- Department of Medicine (Cardiovascular Division), University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Yucheng Chen
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China.,Center of Rare diseases, West China Hospital, Sichuan University, Chengdu, China
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19
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Li X, Li J, Lin L, Shen K, Tian Z, Sun J, Zhang C, An J, Jin Z, Vliegenthart R, Selvanayagam JB, Wang Y. Left and right ventricular myocardial deformation and late gadolinium enhancement: incremental prognostic value in amyloid light-chain amyloidosis. Cardiovasc Diagn Ther 2020; 10:470-480. [PMID: 32695626 DOI: 10.21037/cdt-20-181] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
Background Previous cardiac magnetic resonance (CMR) studies have shown that both late gadolinium enhancement (LGE) and left ventricular (LV) strain have prognostic value in amyloid light-chain (AL) amyloidosis, but the right ventricular (RV) strain has not yet been studied. We aim to determine the incremental prognostic value of LV and RV LGE and strain in AL amyloidosis. Methods This prospective study recruited 87 patients (age, 56.9±9.1 years; M/F, 56/31) and 20 healthy subjects (age, 52.7±8.1 years; M/F, 11/9) who underwent CMR. The LV LGE was classified into no, patchy and global groups. The RV LGE was classified into negative and positive groups. Myocardial deformation was measured using a dedicated software. Follow-up was performed for all-cause mortality using Cox proportional hazards regression and Kaplan-Meier curves. Results During a median follow-up of 21 months, 34 deaths occurred. Presence of LV LGE [HR 2.44, 95% confidence interval (CI), 1.10-5.45, P=0.029] and global longitudinal strain (GLS) (HR 1.13 per 1% absolute decrease, 95% CI, 1.02-1.25, P=0.025) were independent LV predictors. RV LGE (HR 4.07, 95% CI, 1.09-15.24, P=0.037) and GLS (HR 1.10 per 1% absolute decrease, 95% CI, 1.00-1.21, P=0.047) were independent RV predictors. Complementary to LV LGE, LV GLS impairment or RV LGE further reduced survival (both log rank P<0.001). Conclusions This study confirms the incremental prognostic value of LV GLS and RV LGE in AL amyloidosis, which refines the conventional risk evaluation based on LV LGE. GLS based on non-contrast-enhanced CMR are promising new predictors.
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Affiliation(s)
- Xiao Li
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jian Li
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Lu Lin
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Kaini Shen
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Zhuang Tian
- Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jian Sun
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Congli Zhang
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Jing An
- Siemens Shenzhen Magnetic Resonance Ltd., Siemens MRI Center, Hi-Tech Industrial Park, Shenzhen, China
| | - Zhengyu Jin
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
| | - Rozemarijn Vliegenthart
- Department of Radiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Joseph B Selvanayagam
- Department of Cardiovascular Medicine, Flinders University, Flinders Medical Centre, Adelaide, SA, Australia
| | - Yining Wang
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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20
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Zhang Q, Qiao Y, Yan D, Deng Y, Zhang M, Xu P. Myocardial amyloidosis following multiple myeloma in a 38-year-old female patient: A case report. Open Med (Wars) 2020; 15:396-402. [PMID: 33313403 PMCID: PMC7706130 DOI: 10.1515/med-2020-0125] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2019] [Revised: 03/12/2020] [Accepted: 03/19/2020] [Indexed: 01/08/2023] Open
Abstract
Multiple myeloma (MM) is an immunoglobulin-producing tumor of plasma cells, which occurs commonly in the elderly. The incidence of myocardial amyloidosis with MM is extremely low and early clinical manifestations are nonspecific. The diversity of clinical manifestations and first episode symptoms often cause misdiagnosis in young patients with myocardial amyloidosis following MM. In this study, we analyzed the clinical data of a young woman with MM and impaired cardiac function combined with echocardiography, electrocardiography (ECG), laboratory data, cell Congo Red staining, and other manifestations to diagnose amyloidosis. Considering the rapid progression, short survival, and poor prognosis in most patients, a clear, definitive, and timely diagnosis is essential for the treatment of patients with MM complicated with myocardial amyloidosis.
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Affiliation(s)
- Qisi Zhang
- Department of Clinical Laboratory, Henan Provincial People's Hospital, Department of Clinical Laboratory of Central China Fuwai Hospital, Central China Fuwai Hospital of Zhengzhou University, Zhengzhou, Henan, 450003, China
| | - Yingli Qiao
- Department of Clinical Laboratory, Henan Provincial People's Hospital, Department of Clinical Laboratory of Central China Fuwai Hospital, Central China Fuwai Hospital of Zhengzhou University, Zhengzhou, Henan, 450003, China
| | - Dongmei Yan
- Department of Clinical Laboratory, Affiliated Yancheng Hospital, School of Medicine, Southeast University, Jiangsu, Yancheng, 224001, People's Republic of China
| | - Yuhui Deng
- Department of Clinical Laboratory, Henan Provincial People's Hospital, Department of Clinical Laboratory of Central China Fuwai Hospital, Central China Fuwai Hospital of Zhengzhou University, Zhengzhou, Henan, 450003, China
| | - Mengyang Zhang
- Department of Pathology Laboratory, Henan Province People's Hospital, People's Hospital of Zhengzhou University, Zhengzhou, Henan 450000, China
| | - Poshi Xu
- Department of Clinical Laboratory, Henan Provincial People's Hospital, Department of Clinical Laboratory of Central China Fuwai Hospital, Central China Fuwai Hospital of Zhengzhou University, Zhengzhou, Henan, 450003, China
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21
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Liu H, Fu H, Guo YK, Yang ZG, Xu HY, Shuai X, Xu R, Li ZL, Xia CC, He Y, Zhou XY. The prognostic value of right ventricular deformation derived from cardiac magnetic resonance tissue tracking for all-cause mortality in light-chain amyloidosis patients. Cardiovasc Diagn Ther 2020; 10:161-172. [PMID: 32420096 PMCID: PMC7225432 DOI: 10.21037/cdt.2020.01.03] [Citation(s) in RCA: 15] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2019] [Accepted: 12/17/2019] [Indexed: 02/05/2023]
Abstract
BACKGROUND Early detection of right ventricular (RV) dysfunction is vital for determining the prognosis of light-chain amyloidosis (AL) patients. While few studies focused on RV deformation due to the limitation of research methods. The aim of this study was to determine the prognostic significance of RV myocardial strain in AL patients assessed by cardiac magnetic resonance (CMR) tissue tracking. METHODS Sixty-four AL patients (28 females and 36 males, mean age 58±12.8 years old; range 25-81 years old) were enrolled from 1 October 2014 through 31 March 2017 and compared with 20 age- and sex-matched controls. Fifty-one AL patients met the criteria for cardiac amyloidosis (CA). Deformation parameters of both RV and left ventricle (LV) were measured by the CMR tissue tracking technique including myocardial global radial peak strain (GRPS), global circumferential peak strain (GCPS), and global longitudinal peak strain (GLPS). The follow-up time was 20 months or until the occurrence of death. RESULTS Thirty-two (50%) had preserved RV ejection fraction (RVEF ≥45%). AL patients had significantly lower RV-GRPS (20.3±2.12 vs. 31.31±7.61), GCPS (-2.12±0.88 vs. -13.71±2.53), and GLPS (-5.33±0.64 vs. -14.239±2.99) than controls even RVEF remain preserved (all P<0.001). Compared with controls and patients without CA, RV-GRPS (12.26±1.26 vs. 29.72±3.54, P<0.001) and RV-GLPS (-3.78±2.25 vs. -5.66±2.08, P<0.05) were significantly lower in patients with CA. Cox multivariate analyses demonstrated that RV-GRPS [hazard ratio (HR) =0.93, 95% CI: 0.88-0.98, P=0.007] and Mayo stage were (HR =3.11, 95% CI: 1.30-7.41, P=0.01) predictors of mortality in AL patients. CONCLUSIONS CMR tissue tracking is a feasible and highly reproducible technique for the analysis of RV deformation and could aid in the early diagnosis of RV involvement in AL patients. RV-GRPS of RV strain and Mayo stage provides prognostic information about mortality in AL patients.
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Affiliation(s)
- Hui Liu
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, China
| | - Hang Fu
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, China
| | - Ying-Kun Guo
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, China
| | - Zhi-Gang Yang
- Department of Radiology, National Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu 610041, China
| | - Hua-Yan Xu
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, China
| | - Xiao Shuai
- Department of Hematology, West China Hospital, Sichuan University, Chengdu 610041, China
| | - Rong Xu
- Department of Radiology, Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610041, China
| | - Zhen-Lin Li
- Department of Radiology, National Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu 610041, China
| | - Chun-Chao Xia
- Department of Radiology, National Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu 610041, China
| | - Yong He
- Department of Cardiology, West China Hospital, Sichuan University, Chengdu 610041, China
| | - Xiao-Yue Zhou
- MR Collaboration, Siemens Healthcare Ltd., Shanghai 201318, China
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Fine NM, White JA, Jimenez-Zepeda V, Howlett JG. Determinants and Prognostic Significance of Serial Right Heart Function Changes in Patients With Cardiac Amyloidosis. Can J Cardiol 2020; 36:432-440. [DOI: 10.1016/j.cjca.2020.01.020] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2019] [Revised: 01/21/2020] [Accepted: 01/23/2020] [Indexed: 12/19/2022] Open
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Han Y, Chen Y, Ferrari VA. Contemporary Application of Cardiovascular Magnetic Resonance Imaging. Annu Rev Med 2020; 71:221-234. [PMID: 31986088 DOI: 10.1146/annurev-med-041818-015923] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Cardiovascular magnetic resonance imaging (CMR) is a comprehensive and versatile diagnostic and prognostic imaging modality that plays an increasingly important role in management of patients with cardiovascular disease. In this review, we discuss CMR applications in nonischemic cardiomyopathy, ischemic heart disease, arrhythmias, right ventricular diseases, and valvular heart disease. We emphasize the quantitative nature of CMR in current practice, from volumes, function, myocardial strain analysis, and late gadolinium enhancement to parametric mapping, including T1, T2, and T2* relaxation times and extracellular volume fraction assessment.
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Affiliation(s)
- Yuchi Han
- Departments of Medicine (Cardiovascular Division) and Radiology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
| | - Yucheng Chen
- Departments of Cardiology and Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China
| | - Victor A. Ferrari
- Departments of Medicine (Cardiovascular Division) and Radiology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
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24
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Mavrogeni SI, Vartela V, Ntalianis A, Vretou R, Ikonomidis I, Tselegkidou M, Paraskevaidis I, Markousis-Mavrogenis G, Noutsias M, Rigopoulos A, Kolovou G, Kastritis E. Cardiac amyloidosis: in search of the ideal diagnostic tool. Herz 2019; 46:9-14. [PMID: 31796976 DOI: 10.1007/s00059-019-04871-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2018] [Revised: 10/26/2018] [Accepted: 11/12/2019] [Indexed: 11/28/2022]
Abstract
BACKGROUND Cardiac amyloidosis (CA) is due to amyloid deposition in the myocardium. Transthyretin (ATTR) and light-chain (AL) amyloidosis are the main types of CA. Here, we present the clinical and imaging findings in patients with CA and discuss the controversies with the aim of finding the ideal diagnostic tool. METHODS Ten patients suspected of having CA on the basis of electrocardiographic (ECG) and echocardiographic findings were evaluated via cardiovascular magnetic resonance imaging (CMR; 1.5 T) using cine, late gadolinium enhancement (LGE), T1, T2 mapping, and extracellular volume fraction. N‑terminal pro-B-type natriuretic peptide (NT-proBNP) levels were also assessed in all patients. RESULTS All ten patients had an echocardiogram suggestive of CA. The CMR study documented ventricular hypertrophy leading to small ventricular volumes, as assessed by echocardiography. Diffuse subendocardial LGE, supporting the diagnosis of CA, was identified in all except one patient, who had subepicardial LGE due to myocarditis that was verified by endomyocardial biopsy (EMB). Right ventricular (RV) involvement was identified in four of the ten patients, whose condition deteriorated rapidly over the next 6 months. The NT-proBNP levels were >332 pg/ml in all except two patients. Light-chain amyloidosis was identified via fat tissue biopsy in two patients and through renal biopsy in one patient. In two patients with positive technetium-99m, EMB confirmed the diagnosis of ATTR. CONCLUSION NT-proBNP may be a sensitive but nonspecific biomarker for assessing CA. However, CMR is the only imaging modality that can assess the pathophysiologic background of cardiac hypertrophy and the severity of CA, irrespective of NT-proBNP level.
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Affiliation(s)
- Sophie I Mavrogeni
- Onassis Cardiac Surgery Center, 50 Esperou Street, 175-61, P. Faliro, Athens, Greece.
| | - Vasiliki Vartela
- Onassis Cardiac Surgery Center, 50 Esperou Street, 175-61, P. Faliro, Athens, Greece
| | | | | | | | | | | | | | - Michel Noutsias
- Mid-German Heart Center, Department of Internal Medicine III (KIM-III), Division of Cardiology, Angiology and Intensive Medical Care, University Hospital Halle, Martin-Luther-University Halle, Ernst-Grube-Straße 40, 06120, Halle (Saale), Germany
| | - Angelos Rigopoulos
- Mid-German Heart Center, Department of Internal Medicine III (KIM-III), Division of Cardiology, Angiology and Intensive Medical Care, University Hospital Halle, Martin-Luther-University Halle, Ernst-Grube-Straße 40, 06120, Halle (Saale), Germany
| | - Genovefa Kolovou
- Onassis Cardiac Surgery Center, 50 Esperou Street, 175-61, P. Faliro, Athens, Greece
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Nagano N, Yano T, Fujita Y, Kouzu H, Koyama M, Ikeda H, Yasui K, Muranaka A, Nishikawa R, Takahashi R, Kishiue N, Yuda S, Miura T. Assessment of prognosis in immunoglobulin light chain amyloidosis patients with severe heart failure: a predictive value of right ventricular function. Heart Vessels 2019; 35:521-530. [PMID: 31559459 DOI: 10.1007/s00380-019-01513-y] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/07/2019] [Accepted: 09/20/2019] [Indexed: 12/26/2022]
Abstract
Although the benefit of updated therapeutic regimens, including bortezomib, on the survival of immunoglobulin light chain (AL) amyloidosis patients with heart failure (HF) has been reported, predictors of mortality in the patients treated with the updated therapy remain unclear. We retrospectively enrolled AL amyloidosis patients who had severe HF at the time of diagnosis and received the updated therapy, including bortezomib (n = 19, 61 ± 6 years old, 68% male). Severe HF was defined as the presence of both NYHA functional class III or IV and BNP > 200 pg/ml or NT-pro-BNP > 900 pg/ml. One-year mortality rate during follow-up after commencement of the treatment was 37%. Left ventricular morphological parameters and indexes of left ventricular diastolic function on admission were similar in the non-survivors and survivors. However, non-survivors had higher incidences of atrial fibrillation and ventricular tachycardia, higher serum total bilirubin levels (1.34 ± 0.55 vs. 0.61 ± 0.29 mg/dl), higher right atrial volume index (RAVI 49.7 ± 29.9 vs. 27.3 ± 6.8 ml/m2), lower tricuspid annular peak velocities during systole (RVs' 8.0 ± 1.8 vs. 11.6 ± 3.7 cm/sec) and late diastole (RVa' 3.4 ± 0.9 vs. 11.4 ± 5.3 cm/sec), and larger inferior vena cava dimension (22.7 ± 6.4 vs. 16.3 ± 4.9 mm) than those in survivors. Kaplan-Meier curve analyses showed that larger RAVI and lower RVs' and RVa', but not left ventricular systolic/diastolic dysfunction, predicted higher mortality during 1-year follow-up. The present results suggest that the presence of right-sided heart abnormality on admission is associated with high 1-year mortality in AL amyloidosis patients with severe HF under the updated therapeutic regimens.
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Affiliation(s)
- Nobutaka Nagano
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Toshiyuki Yano
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan.
| | - Yugo Fujita
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Hidemichi Kouzu
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Masayuki Koyama
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
- Department of Public Health, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Hiroshi Ikeda
- Department of Hematology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Kenji Yasui
- Division of Laboratory Diagnosis, Sapporo Medical University Hospital, Sapporo, Japan
| | - Atsuko Muranaka
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Ryo Nishikawa
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Ryo Takahashi
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Naohiro Kishiue
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
| | - Satoshi Yuda
- Division of Cardiology, Cardiovascular Center, Teine Keijinkai Hospital, Sapporo, Japan
| | - Tetsuji Miura
- Department of Cardiovascular, Renal and Metabolic Medicine, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan
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Banypersad SM. The Evolving Role of Cardiovascular Magnetic Resonance Imaging in the Evaluation of Systemic Amyloidosis. MAGNETIC RESONANCE INSIGHTS 2019; 12:1178623X19843519. [PMID: 31068754 PMCID: PMC6495435 DOI: 10.1177/1178623x19843519] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/19/2019] [Accepted: 03/19/2019] [Indexed: 12/28/2022]
Abstract
Systemic amyloidosis is a serious multiorgan disease with reduced life expectancy, irrespective of type. The impact of magnetic resonance imaging (MRI) in managing this condition has been immense. The last decade in particular has seen a surge of interest in the assessment and evaluation of the heart in patients with systemic amyloidosis by cardiovascular magnetic resonance imaging (CMR), with approximately 85% of all publications on this subject arising in the last 10 years. This has been largely driven by the creation of new sequences and their subsequent modernisation and technical development, thereby rendering previously prohibitive methods clinically more relevant and applicable. In turn, this has led to an increased awareness and recognition of the disease. This review demonstrates how MRI has become a pivotal diagnostic tool in the assessment of cardiac amyloidosis over the last 2 decades, with the ability to track disease and predict mortality. Several different pathognomonic patterns of late gadolinium enhancement (LGE) are now recognised and are able to prognosticate. T1 mapping and extracellular volume (ECV) techniques have resulted in even earlier disease detection before LGE is even visible and along with T2 mapping, provide new insights into biology. As newer therapies also evolve and become available, the need for accurate tracking of cardiac disease response to treatment carries increasing importance. All these are examined in this review, mainly focussing on light-chain (AL) and transthyretin (ATTR) amyloidosis.
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Mishra T, Pahuja M, Abidov A. Increasingly Recognized Role of Right Ventricle Assessment in Cardiac Amyloidosis. JACC. HEART FAILURE 2019; 7:277-278. [PMID: 30819388 DOI: 10.1016/j.jchf.2018.11.014] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 11/23/2018] [Accepted: 11/24/2018] [Indexed: 05/25/2023]
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Amsallem M, Witteles R, Haddad F. Reply: Increasingly Recognized Role of Right Ventricle Assessment in Cardiac Amyloidosis. JACC. HEART FAILURE 2019; 7:279-280. [PMID: 30819389 DOI: 10.1016/j.jchf.2018.12.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/12/2018] [Accepted: 12/12/2018] [Indexed: 06/09/2023]
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Uzan C, Lairez O, Raud-Raynier P, Garcia R, Degand B, Christiaens LP, Rehman MB. Right ventricular longitudinal strain: a tool for diagnosis and prognosis in light-chain amyloidosis. Amyloid 2018; 25:18-25. [PMID: 29260587 DOI: 10.1080/13506129.2017.1417121] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
OBJECTIVES Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. METHODS We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN. The prognostic value of these parameters was analysed in the AL amyloidosis group. RESULTS Free-wall right ventricular longitudinal strain (FWRVLS) worse than -21.2% discriminates AL amyloidosis [area under the curve (AUC) = 0.744)] from patients with IWT due to other aetiologies. In AL amyloidosis, FWRVLS is the strongest echocardiographic prognostic marker with AUC =0.722 and -16.5% as the optimal cut-off value, beyond which overall mortality increases significantly. It is also the only independent echocardiographic predictor of overall mortality (HR =1.113; 95%CI 1.029-1.204; p = .007), even when adjusted to the Mayo stage and global left ventricular longitudinal strain. CONCLUSIONS FWRVLS should be considered in the diagnostic and prognostic workup in light-chain amyloidosis.
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Affiliation(s)
- Charles Uzan
- a Cardiology Department , CHU de Poitiers , Poitiers , France
| | - Olivier Lairez
- b Imaging Center, University Hospital of Rangueil , Toulouse , France
| | | | - Rodrigue Garcia
- a Cardiology Department , CHU de Poitiers , Poitiers , France
| | - Bruno Degand
- a Cardiology Department , CHU de Poitiers , Poitiers , France
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