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Mohseni A, Zandieh G, Porter K, Pozzessere C, Wagle A, Borhani A, Kamel IR, Vaishnav J, Rowe S, Umair M, Zimmerman SL. Cardiac Amyloidosis: A Comprehensive Review of Imaging Findings. Acad Radiol 2025:S1076-6332(25)00064-9. [PMID: 39893144 DOI: 10.1016/j.acra.2025.01.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2024] [Revised: 01/16/2025] [Accepted: 01/17/2025] [Indexed: 02/04/2025]
Abstract
Cardiac amyloidosis is an infiltrative cardiomyopathy caused by the deposition of insoluble amyloid fibrils in the myocardium, leading to abnormal cardiac function and heart failure. Diagnosis is often challenging due to its diverse symptoms and related comorbidities. Although endomyocardial biopsy is the gold standard for diagnosis, a complete diagnostic approach often includes non-invasive methods such as Cardiac Magnetic Resonance (CMR) and nuclear medicine techniques. Nuclear medicine bone-seeking agents are useful for diagnosing the transthyretin (ATTR) amyloidosis subtype. CMR is especially useful when echocardiography results are inconclusive or for differentiating cardiac amyloidosis from other conditions like hypertrophic cardiomyopathy. CMR provides a comprehensive evaluation of morphological and functional abnormalities, inversion time, late gadolinium enhancement, and can include advanced techniques such as T1 mapping and extracellular volume quantification for diagnostic and prognostic purposes. This review outlines how CMR and nuclear medicine are integral to the diagnostic process for cardiac amyloidosis, emphasizing their crucial roles and the synergy between various diagnostic techniques in assessing suspected cases. ESSENTIALS: REQUIRED SUMMARY STATEMENT: Nuclear medicine PYP scans and CMR play a pivotal role in the non-invasive diagnosis of cardiac amyloidosis, alongside other essential diagnostic modalities.
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Affiliation(s)
- Alireza Mohseni
- Russell H. Morgan Department of Radiology and Radiological Sciences, School of Medicine, Johns Hopkins University, Baltimore, Maryland (A.M., G.Z., A.B., I.R.K., M.U., S.L.Z.)
| | - Ghazal Zandieh
- Russell H. Morgan Department of Radiology and Radiological Sciences, School of Medicine, Johns Hopkins University, Baltimore, Maryland (A.M., G.Z., A.B., I.R.K., M.U., S.L.Z.)
| | | | - Chiara Pozzessere
- Department of Diagnostic and Interventional Radiology, Lausanne University Hospital (CHUV), Lausanne, Switzerland (C.P.)
| | - Anjali Wagle
- Department of Medicine, Johns Hopkins Division of Cardiology, Baltimore, Maryland (A.W., J.V.)
| | - Ali Borhani
- Russell H. Morgan Department of Radiology and Radiological Sciences, School of Medicine, Johns Hopkins University, Baltimore, Maryland (A.M., G.Z., A.B., I.R.K., M.U., S.L.Z.)
| | - Ihab R Kamel
- Russell H. Morgan Department of Radiology and Radiological Sciences, School of Medicine, Johns Hopkins University, Baltimore, Maryland (A.M., G.Z., A.B., I.R.K., M.U., S.L.Z.)
| | - Joban Vaishnav
- Department of Medicine, Johns Hopkins Division of Cardiology, Baltimore, Maryland (A.W., J.V.)
| | - Steven Rowe
- Department of Radiology, University of North Carolina, Chapel Hill, North Carolina (S.R.)
| | - Muhammad Umair
- Russell H. Morgan Department of Radiology and Radiological Sciences, School of Medicine, Johns Hopkins University, Baltimore, Maryland (A.M., G.Z., A.B., I.R.K., M.U., S.L.Z.)
| | - Stefan L Zimmerman
- Russell H. Morgan Department of Radiology and Radiological Sciences, School of Medicine, Johns Hopkins University, Baltimore, Maryland (A.M., G.Z., A.B., I.R.K., M.U., S.L.Z.).
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Parlati ALM, Nardi E, Marzano F, Madaudo C, Di Santo M, Cotticelli C, Agizza S, Abbellito GM, Perrone Filardi F, Del Giudice M, Annunziata FR, Martone I, Prastaro M, Paolillo S, Perrone Filardi P, Gargiulo P. Advancing Cardiovascular Diagnostics: The Expanding Role of CMR in Heart Failure and Cardiomyopathies. J Clin Med 2025; 14:865. [PMID: 39941536 PMCID: PMC11818251 DOI: 10.3390/jcm14030865] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2025] [Revised: 01/25/2025] [Accepted: 01/26/2025] [Indexed: 02/16/2025] Open
Abstract
Cardiovascular magnetic resonance (CMR) imaging has become a cornerstone in the diagnosis, risk stratification, and management of cardiovascular disease (CVD), particularly heart failure (HF) and cardiomyopathies. Renowned as the gold standard for non-invasive quantification of ventricular volumes and ejection fraction, CMR delivers superior spatial and temporal resolution with excellent tissue-blood contrast. Recent advancements, including T1, T2, and T2* mapping, extracellular volume quantification, and late gadolinium enhancement, enable precise tissue characterization, allowing early detection of myocardial changes such as fibrosis, edema, and infiltration. These features provide critical insights into the pathophysiological mechanisms underlying HF phenotypes and diverse cardiomyopathies, enhancing diagnostic accuracy and guiding therapeutic decisions. This review explores the expanding role of CMR in CV disease, highlighting its diagnostic value in HF and in several cardiomyopathies, as well as its contribution to improving patient outcomes through detailed tissue characterization and prognosis.
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Affiliation(s)
| | - Ermanno Nardi
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Federica Marzano
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Cristina Madaudo
- Cardiology Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University Hospital P. Giaccone, University of Palermo, 90127 Palermo, Italy
| | - Mariafrancesca Di Santo
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Ciro Cotticelli
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Simone Agizza
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Giuseppe Maria Abbellito
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Fabrizio Perrone Filardi
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Mario Del Giudice
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | | | - Isabel Martone
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Maria Prastaro
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Stefania Paolillo
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Pasquale Perrone Filardi
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
| | - Paola Gargiulo
- Department of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, Italy
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Gravina M, Casavecchia G, Mangini F, Mautone F, Ruggeri D, Guglielmi G, Macarini L, Brunetti ND. Magnetic resonance mapping for the assessment of cardiomyopathies and myocardial disease. Int J Cardiol 2024; 415:132440. [PMID: 39153509 DOI: 10.1016/j.ijcard.2024.132440] [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: 04/03/2024] [Revised: 07/02/2024] [Accepted: 08/09/2024] [Indexed: 08/19/2024]
Abstract
In recent years, the use of cardiac magnetic resonance (CMR) has grown exponentially in clinical practice. The keys for this success are represented by the possibility of tissue characterization, cardiac volumes and myocardial perfusion assessment, biventricular function evaluation, with no use of ionizing radiations and with an extremely interesting profile of reproducibility. The use of late gadolinium enhancement (LGE) nearly compares a non-invasive biopsy for cardiac fibrosis quantification. LGE, however, is partly unable to detect diffuse myocardial disease. These limits are overcome by new acquisition techniques, mainly T1 and T2 mapping, which allow the diagnosis and characterization of various cardiomyopathies, both ischemic and non-ischemic, such as amyloidosis (high T1), Fabry's disease (low T1), hemochromatosis (low T1), dilated and hypertrophic cardiomyopathy and myocarditis. In this review we detail and summarize principal evidence on the use of T1 and T2 mapping for the study and clinical management of cardiomyopathies.
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Affiliation(s)
- Matteo Gravina
- Radiology Unit, Department of Medical and Surgical Sciences, University of Foggia, Italy.
| | - Grazia Casavecchia
- Cardiology Unit, Department of Medical and Surgical Sciences, University of Foggia, Italy.
| | - Francesco Mangini
- Cardiac Magnetic Resonance Unit, "Di Summa-Perrino" Hospital, Brindisi, Italy
| | - Francesco Mautone
- Cardiology Unit, Department of Medical and Surgical Sciences, University of Foggia, Italy
| | - Debora Ruggeri
- Cardiology Unit, Department of Medical and Surgical Sciences, University of Foggia, Italy
| | - Giuseppe Guglielmi
- Radiology Unit, Department of Medical and Surgical Sciences, University of Foggia, Italy.
| | - Luca Macarini
- Radiology Unit, Department of Medical and Surgical Sciences, University of Foggia, Italy.
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Dicorato MM, Basile P, Muscogiuri G, Carella MC, Naccarati ML, Dentamaro I, Guglielmo M, Baggiano A, Mushtaq S, Fusini L, Pontone G, Forleo C, Ciccone MM, Guaricci AI. Novel Insights into Non-Invasive Diagnostic Techniques for Cardiac Amyloidosis: A Critical Review. Diagnostics (Basel) 2024; 14:2249. [PMID: 39410653 PMCID: PMC11475987 DOI: 10.3390/diagnostics14192249] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2024] [Revised: 10/03/2024] [Accepted: 10/07/2024] [Indexed: 10/20/2024] Open
Abstract
Cardiac amyloidosis (CA) is a cardiac storage disease caused by the progressive extracellular deposition of misfolded proteins in the myocardium. Despite the increasing interest in this pathology, it remains an underdiagnosed condition. Non-invasive diagnostic techniques play a central role in the suspicion and detection of CA, also thanks to the continuous scientific and technological advances in these tools. The 12-lead electrocardiography is an inexpensive and reproducible test with a diagnostic accuracy that, in some cases, exceeds that of imaging techniques, as recent studies have shown. Echocardiography is the first-line imaging modality, although none of its parameters are pathognomonic. According to the 2023 ESC Guidelines, a left ventricular wall thickness ≥ 12 mm is mandatory for the suspicion of CA, making this technique crucial. Cardiac magnetic resonance provides high-resolution images associated with tissue characterization. The use of contrast and non-contrast sequences enhances the diagnostic power of this imaging modality. Nuclear imaging techniques, including bone scintigraphy and positron emission tomography, allow the detection of amyloid deposition in the heart, and their role is also central in assessing the prognosis and response to therapy. The role of computed tomography was recently evaluated by several studies, above in population affected by aortic stenosis undergoing transcatheter aortic valve replacement, with promising results. Finally, machine learning and artificial intelligence-derived algorithms are gaining ground in this scenario and provide the basis for future research. Understanding the new insights into non-invasive diagnostic techniques is critical to better diagnose and manage patients with CA and improve their survival.
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Affiliation(s)
- Marco Maria Dicorato
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Paolo Basile
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Giuseppe Muscogiuri
- Department of Radiology, IRCCS Istituto Auxologico Italiano, San Luca Hospital, 20149 Milan, Italy
| | - Maria Cristina Carella
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Maria Ludovica Naccarati
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Ilaria Dentamaro
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Marco Guglielmo
- Department of Cardiology, Division of Heart and Lungs, Utrecht University, Utrecht University Medical Center, 3584 Utrecht, The Netherlands;
- Department of Cardiology, Haga Teaching Hospital, 2545 The Hague, The Netherlands
| | - Andrea Baggiano
- Department of Perioperative Cardiology and Cardiovascular Imaging, Centro Cardiologico Monzino, IRCCS, 20138 Milan, Italy; (A.B.); (S.M.); (L.F.); (G.P.)
| | - Saima Mushtaq
- Department of Perioperative Cardiology and Cardiovascular Imaging, Centro Cardiologico Monzino, IRCCS, 20138 Milan, Italy; (A.B.); (S.M.); (L.F.); (G.P.)
| | - Laura Fusini
- Department of Perioperative Cardiology and Cardiovascular Imaging, Centro Cardiologico Monzino, IRCCS, 20138 Milan, Italy; (A.B.); (S.M.); (L.F.); (G.P.)
| | - Gianluca Pontone
- Department of Perioperative Cardiology and Cardiovascular Imaging, Centro Cardiologico Monzino, IRCCS, 20138 Milan, Italy; (A.B.); (S.M.); (L.F.); (G.P.)
- Department of Biomedical, Surgical and Dental Sciences, University of Milan, 20122 Milan, Italy
| | - Cinzia Forleo
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Marco Matteo Ciccone
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
| | - Andrea Igoren Guaricci
- Interdisciplinary Department of Medicine, University of Bari “Aldo Moro”, Polyclinic University Hospital, 70124 Bari, Italy; (M.M.D.); (P.B.); (M.C.C.); (M.L.N.); (I.D.); (C.F.); (M.M.C.); (A.I.G.)
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aus dem Siepen F, Hansen T. Diagnosing AL and ATTR Amyloid Cardiomyopathy: A Multidisciplinary Approach. J Clin Med 2024; 13:5873. [PMID: 39407933 PMCID: PMC11477302 DOI: 10.3390/jcm13195873] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/24/2024] [Revised: 09/22/2024] [Accepted: 09/30/2024] [Indexed: 10/20/2024] Open
Abstract
Amyloidosis with cardiac involvement is a fatal disease leading to progressive heart failure. The most common forms of amyloidosis with cardiac involvement are light chain (AL) and transthyretin (ATTR) amyloidosis. To allow effective specific treatment for both forms, precise and early diagnosis is important. This review focuses on diagnostic approaches for AL and ATTR amyloidosis with cardiac involvement, including different strategies, the role of imaging and biomarkers and possible pitfalls.
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Affiliation(s)
- Fabian aus dem Siepen
- Department of Cardiology, University Hospital Heidelberg, Im Neuenheimer Feld 410, 69120 Heidelberg, Germany
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Kida K, Kurosaki T, Fukui R, Matsuura R, Goto S. Native myocardial T 1 mapping using inversion recovery T 1-weighted turbo field echo sequence. Radiol Phys Technol 2024; 17:425-432. [PMID: 38532208 DOI: 10.1007/s12194-024-00795-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2023] [Revised: 02/20/2024] [Accepted: 03/11/2024] [Indexed: 03/28/2024]
Abstract
This study proposes the use of the inversion recovery T1-weighted turbo field echo (IR-T1TFE) sequence for myocardial T1 mapping and compares the results obtained with those of the modified Look-Locker inversion recovery (MOLLI) method for accuracy, precision, and reproducibility. A phantom containing seven vials with different T1 values was imaged, thereby comparing the T1 measurements between the inversion recovery spin-echo (IR-SE) technique, MOLLI, and the IR-T1TFE. The accuracy, precision, and reproducibility of the T1-mapping sequences were analyzed in a phantom study. Fifteen healthy subjects were recruited for the in vivo comparison of native myocardial T1 mapping using MOLLI and IR-T1TFE sequences. After myocardium segmentation, the T1 value of the entire myocardium was calculated. In the phantom study, excellent accuracy was achieved using IR-T1TFE for all T1 ranges. MOLLI displayed lower accuracy than IR-T1TFE (p =0.016), substantially underestimating T1 at large T1 values (> 1000 ms). In the in vivo study, the first mean myocardial T1 values ± SD using MOLLI and IR-T1TFE were 1306 ± 70 ms and 1484 ± 28 ms, respectively, and the second were 1297 ± 68 ms and 1474 ± 43 ms, respectively. The native myocardial T1 obtained with MOLLI was lower than that of IR-T1TFE (p < 0.001). The reproducibility of native myocardial T1 mapping within the same sequence was not statistically significant (p = 0.11). This study demonstrates the utility and validity of myocardial T1 mapping using IR-T1TFE, which is a common sequence. This method was found to have high accuracy and reproducibility.
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Affiliation(s)
- Katsuhiro Kida
- Department of Radiological Technology, Faculty of Health Sciences, Okayama University, 2-5-1 Shikata-Cho, Kita-Ku, Okayama-Shi, Okayama, 700-8558, Japan.
| | - Takamasa Kurosaki
- Department of Central Radiology, Japanese Red Cross Okayama Hospital, 2-1-1 Aoe, Kita-Ku, Okayama-Shi, Okayama, 700-0941, Japan
| | - Ryohei Fukui
- Department of Radiological Technology, Faculty of Health Sciences, Okayama University, 2-5-1 Shikata-Cho, Kita-Ku, Okayama-Shi, Okayama, 700-8558, Japan
| | - Ryutaro Matsuura
- Department of Radiological Technology, Faculty of Health Sciences, Okayama University, 2-5-1 Shikata-Cho, Kita-Ku, Okayama-Shi, Okayama, 700-8558, Japan
| | - Sachiko Goto
- Department of Radiological Technology, Faculty of Health Sciences, Okayama University, 2-5-1 Shikata-Cho, Kita-Ku, Okayama-Shi, Okayama, 700-8558, Japan
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Cui Q, Yu J, Ge X, Gao G, Liu Y, He Q, Shen W. Diagnostic value of LGE and T1 mapping in multiple myeloma patients'heart. BMC Cardiovasc Disord 2024; 24:230. [PMID: 38678215 PMCID: PMC11055279 DOI: 10.1186/s12872-024-03895-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2023] [Accepted: 04/19/2024] [Indexed: 04/29/2024] Open
Abstract
BACKGROUND Unidentified heart failure occurs in patients with multiple myeloma when their heart was involved. CMR with late gadolinium enhancement (LGE) and T1 mapping can identify myocardial amyloid infiltrations. PURPOSE To explore the role of CMR with late gadolinium enhancement (LGE) and T1 mapping for detection of multiple myeloma patients'heart. MATERIAL AND METHODS A total of 16 MM patients with above underwent CMR (3.0-T) with T1 mapping (pre-contrast and post-contrast) and LGE imaging. In addition, 26 patients with non-obstructive hypertrophic cardiomyopathy and 26 healthy volunteers were compared to age- and sex-matched healthy controls without a history of cardiac disease, diabetes mellitus, or normal in CMR. All statistical analyses were performed using the statistical software GraphPad Prism. The measurement data were represented by median (X) and single sample T test was adopted. Enumeration data were represented by examples and Chi-tested was adopted. All tests were two-sided, and P values < 0.05 were considered statistically significant. RESULTS In MM group, LVEF was lower than healthy controls and higher than that of non-obstructive hypertrophic cardiomyopathy group, but without statistically significant difference (%: 49.1 ± 17.5 vs. 55.6 ± 10.3, 40.4 ± 15.6, all P > 0.05). Pre-contrast T1 values of MM group were obviously higher than those of healthy controls and non-obstructive hypertrophic cardiomyopathy group (ms:1462.0 ± 71.3vs. 1269.3 ± 42.3, 1324.0 ± 45.1, all P < 0.05). 16 cases (100%) in MM group all had LGE. CONCLUSION LGE joint T1 mapping wider clinical use techniques and follow-up the patients'disease severity.
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Affiliation(s)
- Qian Cui
- The First Central Clinical School, Tianjin Medical University, Tianjin, China
- Department of Radiology, Tianjin First Central Hospital, School of Medicine, Nankai University, No.24 Fukang Road, Tianjin, 300192, China
| | - Jing Yu
- Department of Radiology, Tianjin First Central Hospital, School of Medicine, Nankai University, No.24 Fukang Road, Tianjin, 300192, China
| | - Xihong Ge
- Department of Radiology, Tianjin First Central Hospital, School of Medicine, Nankai University, No.24 Fukang Road, Tianjin, 300192, China
| | - Guangfeng Gao
- Department of Radiology, Tianjin First Central Hospital, School of Medicine, Nankai University, No.24 Fukang Road, Tianjin, 300192, China
| | - Yang Liu
- Department of Radiology, Tianjin First Central Hospital, School of Medicine, Nankai University, No.24 Fukang Road, Tianjin, 300192, China
| | - Qiang He
- Department of Cardiology, Tianjin First Central Hospital, School of Medicine, Nankai University, Tianjin, China
| | - Wen Shen
- The First Central Clinical School, Tianjin Medical University, Tianjin, China.
- Department of Radiology, Tianjin First Central Hospital, School of Medicine, Nankai University, No.24 Fukang Road, Tianjin, 300192, China.
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Khachatoorian Y, Fuisz A, Frishman WH, Aronow WS, Ranjan P. The Significance of Parametric Mapping in Advanced Cardiac Imaging. Cardiol Rev 2024:00045415-990000000-00243. [PMID: 38595125 DOI: 10.1097/crd.0000000000000695] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 04/11/2024]
Abstract
Cardiac magnetic resonance imaging has witnessed a transformative shift with the integration of parametric mapping techniques, such as T1 and T2 mapping and extracellular volume fraction. These techniques play a crucial role in advancing our understanding of cardiac function and structure, providing unique insights into myocardial tissue properties. Native T1 mapping is particularly valuable, correlating with histopathological fibrosis and serving as a marker for various cardiac pathologies. Extracellular volume fraction, an early indicator of myocardial remodeling, predicts adverse outcomes in heart failure. Elevated T2 relaxation time in cardiac MRI indicates myocardial edema, enabling noninvasive and early detection in conditions like myocarditis. These techniques offer precise insights into myocardial properties, enhancing the accuracy of diagnosis and prognosis across a spectrum of cardiac conditions, including myocardial infarction, autoimmune diseases, myocarditis, and sarcoidosis. Emphasizing the significance of these techniques in myocardial tissue analysis, the review provides a comprehensive overview of their applications and contributions to our understanding of cardiac diseases.
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Affiliation(s)
- Yeraz Khachatoorian
- From the Departments of Cardiology and Medicine, Westchester Medical Center and New York Medical College, Valhalla, NY
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Kottam A, Hanneman K, Schenone A, Daubert MA, Sidhu GD, Gropler RJ, Garcia MJ. State-of-the-Art Imaging of Infiltrative Cardiomyopathies: A Scientific Statement From the American Heart Association. Circ Cardiovasc Imaging 2023; 16:e000081. [PMID: 37916407 DOI: 10.1161/hci.0000000000000081] [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] [Indexed: 11/03/2023]
Abstract
Infiltrative cardiomyopathies comprise a broad spectrum of inherited or acquired conditions caused by deposition of abnormal substances within the myocardium. Increased wall thickness, inflammation, microvascular dysfunction, and fibrosis are the common pathological processes that lead to abnormal myocardial filling, chamber dilation, and disruption of conduction system. Advanced disease presents as heart failure and cardiac arrhythmias conferring poor prognosis. Infiltrative cardiomyopathies are often diagnosed late or misclassified as other more common conditions, such as hypertrophic cardiomyopathy, hypertensive heart disease, ischemic or other forms of nonischemic cardiomyopathies. Accurate diagnosis is also critical because clinical features, testing methodologies, and approach to treatment vary significantly even within the different types of infiltrative cardiomyopathies on the basis of the type of substance deposited. Substantial advances in noninvasive cardiac imaging have enabled accurate and early diagnosis. thereby eliminating the need for endomyocardial biopsy in most cases. This scientific statement discusses the role of contemporary multimodality imaging of infiltrative cardiomyopathies, including echocardiography, nuclear and cardiac magnetic resonance imaging in the diagnosis, prognostication, and assessment of response to treatment.
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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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11
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Mazzucato S, Bandini A, Micera S, Vergaro G, Dalmiani S, Emdin M, Passino C, Moccia S. Classification of patients with cardiac amyloidosis using machine learning models on Italian electronic clinical health records. ANNUAL INTERNATIONAL CONFERENCE OF THE IEEE ENGINEERING IN MEDICINE AND BIOLOGY SOCIETY. IEEE ENGINEERING IN MEDICINE AND BIOLOGY SOCIETY. ANNUAL INTERNATIONAL CONFERENCE 2023; 2023:1-4. [PMID: 38083260 DOI: 10.1109/embc40787.2023.10340074] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/18/2023]
Abstract
Amyloidosis refers to a range of medical conditions in which misshapen proteins accumulate in various organs and tissues, forming insoluble fibrils. Cardiac amyloidosis is frequently linked to the buildup of misfolded transthyretin (TTR) or immunoglobulin light chains (AL). Delayed diagnosis, due to lack of disease awareness, results in a poor prognosis, especially in patients with AL amyloidosis. Early identification is therefore a key factor to improve patient outcomes. This study investigates the use of supervised machine-learning algorithms to support clinicians in classifying amyloidosis and control subjects. The aim of this work is to foster model interpretability reporting the most important risk factors in predicting the presence of cardiac amyloidosis. We analyzed electronic health records (EHRs) of 418 participants acquired in a time window of 12 years as part of a case-control study conducted in Fondazione Toscana Gabriele Monasterio (Italy) clinical practice. This work paves the way for the creation of digital health solutions that can aid in amyloidosis screening. The effective handling, analysis, and interpretation of these solutions can have a transformative effect on modern healthcare, offering new opportunities for improved patient care.
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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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Tanaka H. Illustrative review of cardiac amyloidosis by multimodality imaging. Heart Fail Rev 2023; 28:113-122. [PMID: 35474404 DOI: 10.1007/s10741-022-10245-7] [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] [Accepted: 04/20/2022] [Indexed: 02/07/2023]
Abstract
Cardiac involvement in amyloidosis is characterized by the extracellular deposition of misfolded proteins in the heart with the pathognomonic histological property of green birefringence when viewed under cross-polarized light after staining with Congo red. Although considered a rare disease, recent data suggest that cardiac amyloidosis is underappreciated as a cause of common cardiac diseases or syndromes. The prognosis for transthyretin (TTR) amyloidosis (ATTR) amyloidosis is better than that for amyloid light-chain amyloidosis; however, it is not as good as for other etiologies heart failure. Although there is no proven therapy for patients with ATTR cardiomyopathy (ATTR-CM), tafamidis meglumine, a TTR stabilizer, a study in 2018 found it was associated with reductions in all-cause mortality and cardiovascular-related hospitalizations, as well as with a reduction in the decline in functional capacity and quality of life compared with a placebo for patients with ATTR-CM. As a result of these findings, tafamidis meglumine is currently the only drug approved for patients with both wild-type and variant ATTR-CM, and should be considered for patients whose survival can be reasonably expected. In addition, recent advances in cardiac imaging, diagnostic strategies, and therapies have improved so that interest has been growing in the diagnosis of ATTR-CM by means of non-invasive imaging modalities as a potential means for better management of patients with ATTR-CM. This article reviews the efficacy of non-invasive imaging, especially echocardiography, cardiac magnetic imaging, and 99mTc-pyrophosphate scintigraphy for diagnosis of cardiac amyloidosis.
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Affiliation(s)
- Hidekazu Tanaka
- Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, 7-5-2, Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
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Yue X, Yang L, Wang R, Chan Q, Yang Y, Wu X, Ruan X, Zhang Z, Wei Y, Wang F. The diagnostic value of multiparameter cardiovascular magnetic resonance for early detection of light-chain amyloidosis from hypertrophic cardiomyopathy patients. Front Cardiovasc Med 2022; 9:1017097. [PMID: 36330005 PMCID: PMC9623184 DOI: 10.3389/fcvm.2022.1017097] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2022] [Accepted: 09/26/2022] [Indexed: 11/17/2022] Open
Abstract
Background Early-stage amyloidosis of the heart is prone to be underdiagnosed or misdiagnosed, increasing the risk of early heart failure and even death of the patient. To ensure timely intervention for cardiac light-chain amyloidosis (AL CA), it is vital to develop an effective tool for early identification of the disease. Recently, multiparameter cardiovascular magnetic resonance (CMR) has been used as a comprehensive tool to assess myocardial tissue characterization. We aimed to investigate the difference in left ventricular (LV) strain, native T1, extracellular volume (ECV), and late gadolinium enhancement (LGE) between AL CA patients, hypertrophic cardiomyopathy patients (HCM), and healthy control subjects (HA). Moreover, we explored the value of multiparameter CMR for differential diagnosis of the early-stage AL CA patients from HCM patients, who shared similar imaging characteristics under LGE imaging. Methods A total of 38 AL CA patients, 16 HCM patients, and 17 HA people were prospectively recruited. All subjects underwent LGE imaging, Cine images, and T1 mapping on a 3T scanner. The LV LGE pattern was recorded as none, patchy or global. LV strain, native T1, and ECV were measured semi-automatically using dedicated CMR software. According to clinical and biochemical markers, all patients were classified as Mayo stage I/II and Mayo stage IIIa/IIIb. Univariable and multivariable logistic regression models were utilized to identify independent predictors of early-stage AL CA from HCM patients. Receiver operator characteristic (ROC) curve analysis and Youden’s test were done to determine the accuracy of multiparameter CMR in diagnosing Mayo stage I/II AL CA and establish a cut-off value. Results For Mayo stage I/II AL CA patients, the global longitudinal strain (GLS) absolute value (11.9 ± 3.0 vs. 9.5 ± 1.8, P < 0.001) and the global circumferential strain (GCS) absolute value (19.0 ± 3.6 vs. 9.5 ± 1.8, P < 0.001) were significantly higher than in HCM patients. The native T1 (1334.9 ± 49.9 vs. 1318.2 ± 32.4 ms, P < 0.0001) and ECV values (37.8 ± 5.7 vs. 31.3 ± 2.5%, P < 0.0001) were higher than that of HCM patients. In multiparameter CMR models, GCS (2.097, 95% CI: 1.292–3.403, P = 0.003), GLS (1.468, 95% CI: 1.078–1.998, P = 0.015), and ECV (0.727, 95% CI: 0.569–0.929, P = 0.011) were the significant variables for the discrimination of the early-stage AL CA patients from HCM patients. ROC curve analysis and Youden’s test were used on GCS, GLS, ECV, and pairwise parameters for differentiating between Mayo stage I/II AL CA and HCM patients, respectively. The combination of GLS, GCS, and ECV mapping could distinguish Mayo stage I/II AL amyloidosis patients from hypertrophic cardiomyopathy with excellent performance (AUC = 0.969, Youden index = 0.813). Conclusion In early-stage AL CA patients with atypical LGE, who had similar imaging features as HCM patients, ECV mapping, GCS, and GLS were correlated with the clinical classification of the patients. The combination of GCS, GLS, and ECV could differentiate early-stage AL CA from HCM patients. Multiparameter CMR has the potential to provide an effective and quantitative tool for the early diagnosis of myocardial amyloidosis.
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Affiliation(s)
| | - Lili Yang
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Rui Wang
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Queenie Chan
- Philips Healthcare, Hong Kong, Hong Kong SAR, China
| | - Yanbing Yang
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Xiaohong Wu
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Xiaowei Ruan
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Zhen Zhang
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Yuping Wei
- Department of Hematology, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
| | - Fang Wang
- Medical Imaging Center, People’s Hospital of Ningxia Hui Autonomous Region (The North University of Nationalities Teaching Hospital), Yinchuan, China
- *Correspondence: Fang Wang,
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Dendy JM, Hughes SG, Soslow JH, Clark DE, Paschal CB, Gore JC. Myocardial Tissue Oxygenation and Microvascular Blood Volume Measurement Using a Contrast Blood Oxygenation Level-Dependent Imaging Model. Invest Radiol 2022; 57:561-566. [PMID: 35438656 PMCID: PMC9355912 DOI: 10.1097/rli.0000000000000871] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
OBJECTIVES We propose a method of quantitatively measuring drug-induced microvascular volume changes, as well as drug-induced changes in blood oxygenation using calibrated blood oxygen level-dependent magnetic resonance imaging (MRI). We postulate that for MRI signals there is a contribution to R2* relaxation rates from static susceptibility effects of the intravascular blood that scales with the blood volume/magnetic field and depends on the oxygenation state of the blood. These may be compared with the effects of an intravascular contrast agent. With 4 R2* measurements, microvascular blood volume (MBV) and tissue oxygenation changes can be quantified with the administration of a vasoactive drug. MATERIALS AND METHODS The protocol examined 12 healthy rats in a prospective observational study. R2* maps were acquired with and without infusion of adenosine, which increases microvascular blood flow, or dobutamine, which increases myocardial oxygen consumption. In addition, R2* maps were acquired after the intravenous administration of a monocrystalline iron oxide nanoparticle, with and without adenosine or dobutamine. RESULTS Total microvascular volume was shown to increase by 10.8% with adenosine and by 25.6% with dobutamine ( P < 0.05). When comparing endocardium versus epicardium, both adenosine and dobutamine demonstrated significant differences between endocardial and epicardial MBV changes ( P < 0.05). Total myocardial oxygenation saturation increased by 6.59% with adenosine and by 1.64% with dobutamine ( P = 0.27). The difference between epicardial and endocardial oxygenation changes were significant with each drug (adenosine P < 0.05, dobutamine P < 0.05). CONCLUSIONS Our results demonstrate the ability to quantify microvascular volume and oxygenation changes using calibrated blood oxygen level-dependent MRI, and we demonstrate different responses of adenosine and dobutamine. This method has clinical potential in examining microvascular disease in various disease states without the administration of radiopharmaceuticals or gadolinium-based contrast agents.
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Affiliation(s)
- Jeffrey M Dendy
- From the Division of Cardiovascular Medicine, Department of Internal Medicine, Vanderbilt University Medical Center
| | - Sean G Hughes
- From the Division of Cardiovascular Medicine, Department of Internal Medicine, Vanderbilt University Medical Center
| | - Jonathan H Soslow
- Thomas P. Graham Division of Pediatric Cardiology, Department of Pediatrics, Monroe Carell Jr. Children's Hospital at Vanderbilt
| | - Daniel E Clark
- From the Division of Cardiovascular Medicine, Department of Internal Medicine, Vanderbilt University Medical Center
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Minutoli F, Russo M, Di Bellaearly Phase Planar Imaging G, Laudicella R, Spataro A, Vento A, Comis A, Gentile L, Mazzeo A, Vita G, Baldari S. Diagnosis of cardiac amyloid transthyretin (ATTR) amyloidosis by early (soft tissue) phase [ 99mTc]Tc-DPD whole body scan: comparison with late (bone) phase imaging. Eur Radiol 2022; 32:3035-3044. [PMID: 35031838 DOI: 10.1007/s00330-021-08420-9] [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: 07/02/2021] [Revised: 09/29/2021] [Accepted: 10/17/2021] [Indexed: 11/04/2022]
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 [99mTc]Tc-HMDP administration are sufficient to diagnose cardiac amyloidosis. We evaluated the diagnostic performance of [99mTc]Tc-DPD early phase whole body scan with respect to late phase imaging. METHODS We qualitatively and semiquantitatively reviewed [99mTc]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 [99mTc]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 [99mTc]Tc-DPD is accurate to diagnose cardiac amyloidosis and may be satisfactory at least in frail patients with high cardiac burden of amyloid fibrils.
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Affiliation(s)
- Fabio Minutoli
- Department of Biomedical and Dental Sciences and Morpho-Functional Imaging, University of Messina, Messina, Italy
| | - Massimo Russo
- Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
| | | | - Riccardo Laudicella
- Department of Biomedical and Dental Sciences and Morpho-Functional Imaging, University of Messina, Messina, Italy. .,Nuclear Medicine Unit, University Hospital "Policlinico G.Martino", via Consolare Valeria n.1, 98125, Messina, Italy.
| | - Alessandro Spataro
- Department of Biomedical and Dental Sciences and Morpho-Functional Imaging, University of Messina, Messina, Italy
| | - Antonio Vento
- Department of Biomedical and Dental Sciences and Morpho-Functional Imaging, University of Messina, Messina, Italy
| | - Alessio Comis
- Department of Biomedical and Dental Sciences and Morpho-Functional Imaging, University of Messina, Messina, Italy
| | - Luca Gentile
- Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
| | - Anna Mazzeo
- Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
| | - Giuseppe Vita
- Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
| | - Sergio Baldari
- Department of Biomedical and Dental Sciences and Morpho-Functional Imaging, University of Messina, Messina, Italy
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Joury A, Faaborg-Andersen C, Quintana RA, daSilva-deAbreu A, Nativi-Nicolau J. Diagnostic Tools for Cardiac Amyloidosis: A Pragmatic Comparison of Pathology, Imaging and Laboratories. Curr Probl Cardiol 2022; 48:101106. [PMID: 35007639 DOI: 10.1016/j.cpcardiol.2022.101106] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2021] [Accepted: 01/03/2022] [Indexed: 01/01/2023]
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.
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Affiliation(s)
- Abdulaziz Joury
- John Ochsner Heart and Vascular Institute, Ochsner Medical Center, New Orleans, LA; King Salman Heart Center, King Fahd Medical City, Riyadh, Saudi Arabia
| | | | - Raymundo A Quintana
- Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, GA
| | - Adrian daSilva-deAbreu
- Section of Cardiovascular Medicine, Yale University School of Medicine, New Haven, CT; Heart and Vascular Center, Yale-New Haven Hospital, New Haven, CT
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Hayashi H, Oda S, Kidoh M, Nakaura T, Morita K, Nagayama Y, Yoneda T, Takashio S, Misumi Y, Ueda M, Tsujita K, Hirai T. Can myocardial susceptibility quantification be an imaging biomarker for cardiac amyloidosis? Jpn J Radiol 2021; 40:500-507. [PMID: 34841460 PMCID: PMC9068634 DOI: 10.1007/s11604-021-01228-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2021] [Accepted: 11/22/2021] [Indexed: 11/17/2022]
Abstract
Purpose This study aimed to evaluate whether quantification of myocardial susceptibility by cardiac magnetic resonance imaging (CMR) can be an imaging biomarker for cardiac amyloidosis (CA). Materials and methods Twenty-six patients with CA underwent CMR, including magnetic phase imaging with a 3.0-T magnetic resonance imaging scanner. Myocardial susceptibility was quantified as a phase shift slope value by magnetic phase analysis. Those values from patients with CA were compared with corresponding values from 18 controls and 15 healthy volunteers. A univariate logistic regression analysis was conducted to identify significant parameters related to CA. Results The phase shift slope, a quantitative parameter of myocardial susceptibility, was significantly lower in the CA group compared with the control group and compared with healthy volunteers (p < 0.01). From a total of 17 tested variables, 6 were considered to be significant predictors of CA (p ≤ 0.05) during the univariate analysis. The phase shift slope yielded the best AUC of 0.89 (95% CI = 0.79–0.98) for the prediction of CA (p < 0.01). The phase shift slope was significantly correlated with the end-diastolic thickness of the interventricular septum (r = − 0.39, p < 0.01) and posterior wall of the left ventricle (r = − 0.35, p = 0.02). Conclusion Myocardial susceptibility analysis by CMR helps in the diagnosis of patients with CA and can be a new quantitative imaging biomarker for CA.
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Affiliation(s)
- Hidetaka Hayashi
- Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto, 860-8556, Japan
| | - Seitaro Oda
- Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto, 860-8556, Japan.
| | - Masafumi Kidoh
- Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto, 860-8556, Japan
| | - Takeshi Nakaura
- Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto, 860-8556, Japan
| | - Kosuke Morita
- Department of Central Radiology, Kumamoto University Hospital, Kumamoto, Japan
| | - Yasunori Nagayama
- Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto, 860-8556, Japan
| | - Tetsuya Yoneda
- Department of Medical Physics in Advanced Biomedical Sciences, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Seiji Takashio
- Department of Cardiovascular Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Yohei Misumi
- Department of Neurology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Mitsuharu Ueda
- Department of Neurology, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Kenichi Tsujita
- Department of Cardiovascular Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Toshinori Hirai
- Department of Diagnostic Radiology, Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto, 860-8556, Japan
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Abulizi M, Sifaoui I, Wuliya-Gariepy M, Kharoubi M, Israël JM, Emsen B, Bodez D, Monnet A, Didierlaurent D, Tacher V, Luciani A, Damy T, Deux JF, Itti E. 18F-sodium fluoride PET/MRI myocardial imaging in patients with suspected cardiac amyloidosis. J Nucl Cardiol 2021; 28:1586-1595. [PMID: 31512197 DOI: 10.1007/s12350-019-01885-8] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2019] [Accepted: 07/31/2019] [Indexed: 10/26/2022]
Abstract
BACKGROUND We evaluated the diagnostic performance of 18F-NaF PET/MRI in patients with suspected cardiac amyloidosis (CA). METHODS Twenty-seven consecutive patients underwent myocardial PET 1 hour after injection of 4 MBq/kg 18F-NaF with simultaneous MRI including cine-MRI, T1 and T2 mapping, first-pass and late gadolinium enhancement (LGE). 18F-NaF uptake was measured visually and semi-quantitatively by calculating myocardium-to-blood pool (M/B) ratios. CA was confirmed histologically. RESULTS Transthyretin (TTR)-CA was diagnosed in 16 patients, light-chain (AL)-CA in 7, and no-CA in 4. Visual interpretation of 18F-NaF images revealed a relative increase in myocardial uptake in only 3 patients, all with TTR CA, and a relative decrease in 13, including 7 AL CA, 3 no-CA, and 3 TTR CA. M/B ratios were significantly higher in TTR CA (1.00 ± 0.12) than in AL CA (0.81 ± 0.06, P = 0.001) or in no-CA (0.73 ± 0.16, P = 0.006). The optimal M/B cut-off to distinguish TTR CA from AL CA was ≥ 0.90 (Fischer, P = 0.0005). By comparison, classification of patients using 99mTc-HMDP heart-to-mediastinum ratios with the previously published cut-off ≥ 1.21 reached higher significance (P < 0.0001). Among MRI parameters, myocardial T1, LGE score, and extracellular volume were higher in CA than in no-CA patients, 1409 ± 76 vs 1278 ± 35 ms (P = 0.004), 10.35 ± 5.30 vs 3.50 ± 3.42 (P = 0.03), and 46 ± 10 vs 33 ± 8 % (P = 0.01), respectively. CONCLUSION 18F-NaF PET/MRI shows good diagnostic performance when semi-quantification is used. However, contrast is low and visual interpretation may be challenging in routine. PET/MRI could constitute a one-stop-shop evaluation of amyloid load and cardiac function in patients needing rapid work-up.
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Affiliation(s)
- Mukedaisi Abulizi
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
| | - Islem Sifaoui
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
| | - Mijiti Wuliya-Gariepy
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
- Department of Cardiology, CHU Henri Mondor, AP-HP/U-PEC, 94010, Créteil, France
| | - Mounira Kharoubi
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
- Department of Cardiology, CHU Henri Mondor, AP-HP/U-PEC, 94010, Créteil, France
| | - Jean-Marc Israël
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
| | - Berivan Emsen
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
| | - Diane Bodez
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
- Department of Cardiology, CHU Henri Mondor, AP-HP/U-PEC, 94010, Créteil, France
| | | | | | - Vania Tacher
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
| | - Alain Luciani
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
| | - Thibaud Damy
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
- Department of Cardiology, CHU Henri Mondor, AP-HP/U-PEC, 94010, Créteil, France
| | - Jean-François Deux
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France
| | - Emmanuel Itti
- SyMPTOm PET/MRI platform, Departments of Nuclear Medicine and Radiology, CHU Henri Mondor Hospital, AP-HP/U-PEC, 51 Ave. du Mal de Lattre de Tassigny, 94010, Créteil, France.
- Amyloid Research Institute, IMRB U955, U-PEC, 94010, Créteil, France.
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20
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Dorbala S, Ando Y, Bokhari S, Dispenzieri A, Falk RH, Ferrari VA, Fontana M, Gheysens O, Gillmore JD, Glaudemans AWJM, Hanna MA, Hazenberg BPC, Kristen AV, Kwong RY, Maurer MS, Merlini G, Miller EJ, Moon JC, Murthy VL, Quarta CC, Rapezzi C, Ruberg FL, Shah SJ, Slart RHJA, Verberne HJ, Bourque JM. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2-Evidence Base and Standardized Methods of Imaging. Circ Cardiovasc Imaging 2021; 14:e000029. [PMID: 34196223 DOI: 10.1161/hci.0000000000000029] [Citation(s) in RCA: 45] [Impact Index Per Article: 11.3] [Reference Citation Analysis] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Affiliation(s)
- Sharmila Dorbala
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA
| | - Yukio Ando
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Japan
| | - Sabahat Bokhari
- Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, NY
| | - Angela Dispenzieri
- Division of Hematology, Division of Cardiovascular Diseases, and Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, MN
| | - Rodney H Falk
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA
| | - Victor A Ferrari
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
| | - Marianna Fontana
- National Amyloidosis Centre, Division of Medicine, University College London, London, United Kingdom
| | - Olivier Gheysens
- Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium
| | - Julian D Gillmore
- National Amyloidosis Centre, Division of Medicine, University College London, London, United Kingdom
| | - Andor W J M Glaudemans
- Medical Imaging Center, Department of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
| | - Mazen A Hanna
- Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH
| | - Bouke P C Hazenberg
- Department of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
| | - Arnt V Kristen
- Department of Cardiology, University of Heidelberg, Heidelberg, Germany
| | - Raymond Y Kwong
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA
| | - Mathew S Maurer
- Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, NY
| | - Giampaolo Merlini
- Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
- Department of Molecular Medicine, University of Pavia, Italy
| | - Edward J Miller
- Cardiovascular Medicine, Yale University School of Medicine, New Haven, CT
| | - James C Moon
- National Amyloidosis Centre, Division of Medicine, University College London, London, United Kingdom
| | | | - C Cristina Quarta
- National Amyloidosis Centre, Division of Medicine, University College London, London, United Kingdom
| | - Claudio Rapezzi
- Cardiology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater-University of Bologna, Bologna, Italy
| | - Frederick L Ruberg
- Amyloidosis Center and Section of Cardiovascular Medicine, Department of Medicine, Boston University School of Medicine, Boston Medical Center, Boston, MA
| | - Sanjiv J Shah
- Feinberg School of Medicine, Northwestern University, Chicago, IL
| | - Riemer H J A Slart
- Medical Imaging Center, Department of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
| | - Hein J Verberne
- Division of Hematology, Division of Cardiovascular Diseases, and Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, MN
- Department of Cardiology, University of Heidelberg, Heidelberg, Germany
| | - Jamieson M Bourque
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
- Department of Molecular Medicine, University of Pavia, Italy
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21
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Koike H, Okumura T, Murohara T, Katsuno M. Multidisciplinary Approaches for Transthyretin Amyloidosis. Cardiol Ther 2021; 10:289-311. [PMID: 34089151 PMCID: PMC8177037 DOI: 10.1007/s40119-021-00222-w] [Citation(s) in RCA: 26] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/10/2021] [Indexed: 12/12/2022] Open
Abstract
Amyloidosis caused by systemic deposition of transthyretin (TTR) is called ATTR amyloidosis and mainly includes hereditary ATTR (ATTRv) amyloidosis and wild-type ATTR (ATTRwt) amyloidosis. Until recently, ATTRv amyloidosis had been considered a disease in the field of neurology because neuropathic symptoms predominated in patients described in early reports, whereas advances in diagnostic techniques and increased recognition of this disease revealed the presence of patients with cardiomyopathy as a predominant feature. In contrast, ATTRwt amyloidosis has been considered a disease in the field of cardiology. However, recent studies have suggested that some of the patients with ATTRwt amyloidosis present tenosynovial tissue complications, particularly carpal tunnel syndrome, as an initial manifestation of amyloidosis, necessitating an awareness of this disease among neurologists and orthopedists. Although histopathological confirmation of amyloid deposits has traditionally been considered mandatory for the diagnosis of ATTR amyloidosis, the development of noninvasive imaging techniques in the field of cardiology, such as echocardiography, magnetic resonance imaging, and nuclear imaging, enabled nonbiopsy diagnosis of this disease. The mechanisms underlying characteristic cardiac imaging findings have been deciphered by histopathological studies. Novel disease-modifying therapies for ATTR amyloidosis, such as TTR stabilizers, short interfering RNA, and antisense oligonucleotides, were initially approved for ATTRv amyloidosis patients with polyneuropathy. However, the indications for the use of these disease-modifying therapies gradually widened to include ATTRv and ATTRwt amyloidosis patients with cardiomyopathy. Since the coronavirus disease 2019 (COVID-19) pandemic, which is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, occurred, the minimization of hospital visits and telemedicine have become increasingly important. As older age and cardiovascular disease are major factors associated with increased disease severity and mortality of COVID-19, many ATTR amyloidosis patients are at increased risk of disease aggravation when they are infected with SARS-CoV-2. From this viewpoint, close interspecialty communication to determine the optimal interval of evaluation is needed for the management of patients with ATTR amyloidosis.
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Affiliation(s)
- Haruki Koike
- Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.
| | - Takahiro Okumura
- Department of Cardiology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Toyoaki Murohara
- Department of Cardiology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Masahisa Katsuno
- Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan
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22
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Peix A, Padrón K. Imagine, believe, and achieve. J Nucl Cardiol 2021; 28:831-834. [PMID: 33963494 DOI: 10.1007/s12350-021-02630-w] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2021] [Accepted: 04/05/2021] [Indexed: 10/21/2022]
Affiliation(s)
- Amalia Peix
- Nuclear Medicine Department, Institute of Cardiology, 17 No. 702, Vedado, La Habana, CP, 10 400, Cuba.
| | - Kenia Padrón
- Nuclear Medicine Department, Institute of Cardiology, 17 No. 702, Vedado, La Habana, CP, 10 400, Cuba
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23
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Grigoratos C, Gueli I, Arendt CT, Leithner D, Meloni A, Nugara C, Barison A, Todiere G, Puntmann VO, Novo G, Pepe A, Emdin M, Nagel E, Aquaro GD. Prevalence and prognostic impact of nonischemic late gadolinium enhancement in stress cardiac magnetic resonance. J Cardiovasc Med (Hagerstown) 2021; 21:980-985. [PMID: 33156590 DOI: 10.2459/jcm.0000000000001016] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
AIM To assess the prevalence and prognostic significance of NI-LGE in patients undergoing stress-CMR. METHODS Stress-CMR with either dipyridamole or adenosine was performed in 283 patients (228 men, 81%) including perfusion imaging, wall motion evaluation and LGE. Follow-up was completed in all enrolled patients (median time: 1850 days; interquartile range: 1225-2705 days). Composite endpoint included cardiac death, ventricular tachycardia, myocardial infarction, stroke, hospitalization for cardiac cause and coronary revascularization performed beyond 90 days from stress-CMR scans. RESULTS One hundred and twelve patients (40%) had negative LGE (no-LGE), 140 patients (49%) I-LGE and 31 patients (11%) NI-LGE. Twenty-five events occurred in the no-LGE group, 68 in I-LGE and 11 in the NI-LGE group. On survival curves, patients with NI-LGE had worse prognosis than patients with no-LGE regardless of the presence of inducible perfusion defects. No significant prognostic differences were found between I-LGE and NI-LGE. CONCLUSION NI-LGE can be detected in 11% of patients during stress-CMR providing a diagnosis of nonischemic cardiac disease. Patients with NI-LGE have worse prognosis than those with no-LGE.
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Affiliation(s)
- Chrysanthos Grigoratos
- Fondazione Gabriele Monasterio CNR/Regione Toscana.,Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy
| | | | - Christophe T Arendt
- Institute for Diagnostic and Interventional Radiology, University Hospital Frankfurt, Frankfurt am Main, Germany
| | - Doris Leithner
- Institute for Diagnostic and Interventional Radiology, University Hospital Frankfurt, Frankfurt am Main, Germany
| | | | - Cinzia Nugara
- Fondazione Gabriele Monasterio CNR/Regione Toscana.,Division of Cardiology and Cardiovascular Rehabilitation, Department of Internal Medicine and Cardiovascular Disease, University Hospital Paolo Giaccone, Palermo, Italy
| | | | | | - Valentina O Puntmann
- DZHK Centre for Cardiovascular Imaging, Institute for Experimental and Translational Cardiovascular Imaging, University Hospital Frankfurt, Frankfurt am Main, Germany
| | - Giuseppina Novo
- Division of Cardiology and Cardiovascular Rehabilitation, Department of Internal Medicine and Cardiovascular Disease, University Hospital Paolo Giaccone, Palermo, Italy
| | - Alessia Pepe
- Fondazione Gabriele Monasterio CNR/Regione Toscana
| | - Michele Emdin
- Fondazione Gabriele Monasterio CNR/Regione Toscana.,Institute of Life Sciences, Scuola Superiore Sant'Anna, Pisa, Italy
| | - Eike Nagel
- DZHK Centre for Cardiovascular Imaging, Institute for Experimental and Translational Cardiovascular Imaging, University Hospital Frankfurt, Frankfurt am Main, Germany
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24
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Rajiah P, Kirsch J, Bolen MA, Batlle JC, Brown RKJ, Francois CJ, Galizia MS, Hanneman K, Inacio JR, Johri AM, Lee DC, Singh SP, Villines TC, Wann S, Zimmerman SL, Abbara S. ACR Appropriateness Criteria® Nonischemic Myocardial Disease with Clinical Manifestations (Ischemic Cardiomyopathy Already Excluded). J Am Coll Radiol 2021; 18:S83-S105. [PMID: 33651982 DOI: 10.1016/j.jacr.2021.01.019] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/27/2021] [Accepted: 01/27/2021] [Indexed: 12/13/2022]
Abstract
Nonischemic cardiomyopathies encompass a broad spectrum of myocardial disorders with mechanical or electrical dysfunction without evidence of ischemia. There are five broad variants of nonischemic cardiomyopathies; hypertrophic cardiomyopathy (Variant 1), restrictive or infiltrative cardiomyopathy (Variant 2), dilated or unclassified cardiomyopathy (Variant 3), arrhythmogenic cardiomyopathy (Variant 4), and inflammatory cardiomyopathy (Variant 5). For variants 1, 3, and 4, resting transthoracic echocardiography, MRI heart function and morphology without and with contrast, and MRI heart function and morphology without contrast are the usually appropriate imaging modalities. For variants 2 and 5, resting transthoracic echocardiography and MRI heart function and morphology without and with contrast are the usually appropriate imaging modalities. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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Affiliation(s)
| | - Jacobo Kirsch
- Panel Chair, Cleveland Clinic Florida, Weston, Florida
| | - Michael A Bolen
- Panel Vice-Chair, Cleveland Clinic, Cleveland, Ohio, Radiology Fellowship Director for Cardiovascular CT/MRI Cleveland Clinic Main Campus
| | - Juan C Batlle
- Miami Cardiac and Vascular Institute and Baptist Health of South Florida, Miami, Florida
| | - Richard K J Brown
- University of Utah, Department of Radiology and Imaging Sciences, Salt Lake City, Utah
| | | | | | - Kate Hanneman
- Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada, Director, Cardiac Imaging Research, Department of Medical Imaging, University of Toronto
| | - Joao R Inacio
- The Ottawa Hospital, University of Ottawa, Ottawa, Ontario, Canada
| | - Amer M Johri
- Queen's University, Kingston, Ontario, Canada, Cardiology expert
| | - Daniel C Lee
- Northwestern University Feinberg School of Medicine Chicago, Illinois, Society for Cardiovascular Magnetic Resonance, Co-Director, Cardiovascular Magnetic Resonance Imaging, Northwestern University Feinberg School of Medicine
| | | | - Todd C Villines
- University of Virginia Health System, Charlottesville, Virginia, Society of Cardiovascular Computed Tomography
| | - Samuel Wann
- Wisconsin Heart Hospital, Milwaukee, Wisconsin, Nuclear cardiology expert
| | | | - Suhny Abbara
- Specialty Chair, UT Southwestern Medical Center, Dallas, Texas
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25
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Di Renzi P, Coniglio A, Abella A, Belligotti E, Rossi P, Pasqualetti P, Simonelli I, Della Longa G. Volumetric histogram-based analysis of cardiac magnetic resonance T1 mapping: A tool to evaluate myocardial diffuse fibrosis. Phys Med 2021; 82:185-191. [PMID: 33662882 DOI: 10.1016/j.ejmp.2021.01.080] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/20/2020] [Revised: 12/09/2020] [Accepted: 01/29/2021] [Indexed: 01/19/2023] Open
Affiliation(s)
- P Di Renzi
- S. Giovanni Calibita Hospital, Fatebenefratelli Hospital, Isola Tiberina, Department of Radiology, Rome, Italy
| | - A Coniglio
- S. Giovanni Calibita, Fatebenefratelli Hospital, Isola Tiberina, Department of Medical Physics, Rome, Italy; ASL Roma 1, PO San Filippo Neri, Department of Medical Physics, Rome, Italy.
| | - A Abella
- S. Giovanni Calibita Hospital, Fatebenefratelli Hospital, Isola Tiberina, Department of Radiology, Rome, Italy
| | - E Belligotti
- Ospedali Riuniti Marche Nord, Department of Medical Physics and High Technologies, Pesaro, Italy
| | - P Rossi
- S. Giovanni Calibita Hospital, Fatebenefratelli Hospital, Isola Tiberina, Arrhythmology Unit, Rome, Italy
| | - P Pasqualetti
- Department of Public Health and Infectious Diseases, Section of Health Statistics and Biometry, Sapienza University of Rome, Italy
| | - I Simonelli
- Fatebenefratelli Foundation for Health Research and Education, AFaR Division, Rome, Italy
| | - G Della Longa
- S. Giovanni Calibita Hospital, Fatebenefratelli Hospital, Isola Tiberina, Department of Radiology, Rome, Italy
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26
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Yilmaz A, Bauersachs J, Bengel F, Büchel R, Kindermann I, Klingel K, Knebel F, Meder B, Morbach C, Nagel E, Schulze-Bahr E, Aus dem Siepen F, Frey N. Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK). Clin Res Cardiol 2021; 110:479-506. [PMID: 33459839 PMCID: PMC8055575 DOI: 10.1007/s00392-020-01799-3] [Citation(s) in RCA: 51] [Impact Index Per Article: 12.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/30/2020] [Accepted: 12/21/2020] [Indexed: 12/15/2022]
Abstract
Systemic forms of amyloidosis affecting the heart are mostly light-chain (AL) and transthyretin (ATTR) amyloidoses. The latter is caused by deposition of misfolded transthyretin, either in wild-type (ATTRwt) or mutant (ATTRv) conformation. For diagnostics, specific serum biomarkers and modern non-invasive imaging techniques, such as cardiovascular magnetic resonance imaging (CMR) and scintigraphic methods, are available today. These imaging techniques do not only complement conventional echocardiography, but also allow for accurate assessment of the extent of cardiac involvement, in addition to diagnosing cardiac amyloidosis. Endomyocardial biopsy still plays a major role in the histopathological diagnosis and subtyping of cardiac amyloidosis. The main objective of the diagnostic algorithm outlined in this position statement is to detect cardiac amyloidosis as reliably and early as possible, to accurately determine its extent, and to reliably identify the underlying subtype of amyloidosis, thereby enabling subsequent targeted treatment.
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Affiliation(s)
- A Yilmaz
- Sektion für Herzbildgebung, Klinik für Kardiologie, Universitätsklinikum Münster, Von-Esmarch-Str. 48, 48149, Münster, Germany.
| | - J Bauersachs
- Klinik für Kardiologie und Angiologie, Medizinische Hochschule Hannover, Hannover, Germany
| | - F Bengel
- Klinik für Nuklearmedizin, Medizinische Hochschule Hannover, Hannover, Germany
| | - R Büchel
- Klinik für Nuklearmedizin, Universitätsspital Zürich, Zurich, Switzerland
| | - I Kindermann
- Klinik für Innere Medizin III (Kardiologie, Angiologie und Internistische Intensivmedizin), Universitätsklinikum des Saarlandes und Medizinische Fakultät der Universität des Saarlandes, Homburg, Germany
| | - K Klingel
- Institut für Pathologie und Neuropathologie, Universität Tübingen, Tübingen, Germany
| | - F Knebel
- Medizinische Klinik m.S. Kardiologie und Angiologie, Charite Universitätsmedizin Berlin Campus Mitte, Berlin, Germany
| | - B Meder
- Klinik für Innere Medizin III, Universitätsklinikum Heidelberg, Heidelberg, Germany
| | - C Morbach
- Klinik für Innere Medizin III, Universitätsklinikum Heidelberg, Heidelberg, Germany
| | - E Nagel
- Interdisziplinäres Amyloidosezentrum Nordbayern, Deutsches Zentrum für Herzinsuffizienz, Medizinische Klinik I der Universität Würzburg, Würzburg, Germany
| | - E Schulze-Bahr
- Institut für Experimentelle und translationale kardiovaskuläre Bildgebung, Universitätsklinikum Frankfurt, Frankfurt, Germany
| | - F Aus dem Siepen
- Institut für Genetik von Herzerkrankungen (IfGH), Universitätsklinikum Münster, Münster, Germany
| | - N Frey
- Klinik für Innere Medizin III, Schwerpunkt Kardiologie und Angiologie, Universitätsklinikum Schleswig-Holstein, Kiel, Germany.,Kommission für Klinische Kardiovaskuläre Medizin, Deutsche Gesellschaft für Kardiologie, Düsseldorf, Germany
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27
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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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28
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Spencer-Bonilla G, Alexander KM, Witteles RM. Advances in the Diagnosis and Management of Transthyretin Amyloid Cardiomyopathy. CURRENT TREATMENT OPTIONS IN CARDIOVASCULAR MEDICINE 2020. [DOI: 10.1007/s11936-020-00844-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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29
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Cicco S, Solimando AG, Buono R, Susca N, Inglese G, Melaccio A, Prete M, Ria R, Racanelli V, Vacca A. Right Heart Changes Impact on Clinical Phenotype of Amyloid Cardiac Involvement: A Single Centre Study. Life (Basel) 2020; 10:life10100247. [PMID: 33081052 PMCID: PMC7603245 DOI: 10.3390/life10100247] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/22/2020] [Revised: 10/12/2020] [Accepted: 10/16/2020] [Indexed: 12/18/2022] Open
Abstract
Amyloidosis is due to deposition of an excessive amount of protein in many parenchymal tissues, including myocardium. The onset of cardiac Amyloidosis (CA) is an inauspicious prognostic factor, which can lead to sudden death. We retrospectively analyzed 135 patients with systemic amyloidosis, admitted to our ward between 1981 and 2019. Among them, 54 patients (46.30% F/53.70% M, aged 63.95 ± 12.82) presented CA at baseline. In 53 patients, it was associated with a multiorgan involvement, while in one there was a primary myocardial deposition. As a control group, we enrolled 81 patients (49.30% F/50.70% M, aged 58.33 ± 15.65) who did not meet the criteria for CA. In 44/54 of patients CA was associated with AL, 5/54 with AA and 3/54 of patients with ATTR, and in 1/54 AL was related to hemodialysis and in 1/54 to Gel-Amyloidosis. The most common AL type was IgG (28/44); less frequent forms were either IgA (7/44) or IgD (2/44), while seven patients had a λ free light chain form. The 32 AL with complete Ig were 31 λ-chain and just one k-chain. CA patients presented normal BP (SBP 118.0 ± 8.4 mmHg; DBP 73.8 ± 4.9 mmHg), while those with nCA had an increased proteinuria (p = 0.02). TnI and NT-proBNP were significantly increased compared to nCA (p = 0.031 and p = 0.047, respectively). In CA patients we found an increased LDH compared to nCA (p = 0.0011). CA patients were also found to have an increased interventricular septum thickness compared to nCA (p = 0.002), a decreased Ejection Fraction % (p = 0.0018) and Doppler velocity E/e’ ratio (p = 0.0095). Moreover, CA patients had an enhanced right atrium area (p = 0.0179), right ventricle basal diameter (p = 0.0112) and wall thickness (p = 0.0471) compared to nCA, and an increased inferior cava vein diameter (p = 0.0495) as well. TAPSE was the method chosen to evaluate systolic function of the right heart. In CA subjects very poor TAPSE levels were found compared to nCA patients (p = 0.0495). Additionally, we found a significant positive correlation between TAPSE and lymphocyte count (r = 0.47; p = 0.031) as well as Gamma globulins (r = 0.43, p = 0.033), Monoclonal components (r = 0.72; p = 0.047) and IgG values (r = 0.62, p = 0.018). Conversely, a significant negative correlation with LDH (r = −0.57, p = 0.005), IVS (r = −0.51, p = 0.008) and diastolic function evaluated as E/e’ (r = −0.60, p = 0.003) were verified. CA patients had very poor survival rates compared to controls (30 vs. 66 months in CA vs. nCA, respectively, p = 0.15). Mean survival of CA individuals was worse also when stratified according to NT-proBNP levels, using 2500 pg/mL as class boundary (174 vs. 5.5 months, for patients with lower vs. higher values than the median, respectively p = 0.013). In much the same way, a decreased right heart systolic function was correlated with a worse prognosis (18.0 months median survival, not reached in subjects with lower values than 18 mm, p = 0.0186). Finally, our data highlight the potential prognostic and predictive value of right heart alterations characterizing amyloidosis, as a novel clinical parameter correlated to increased LDH and immunoglobulins levels. Overall, we confirm the clinical relevance of cardiac involvement suggests that right heart evaluation may be considered as a new marker for clinical risk stratification in patients with amyloidosis.
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Affiliation(s)
- Sebastiano Cicco
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
- Internal Medicine Department, AUO Policlinico Ospedali Riuniti, Viale L. Pinto, I-71122 Foggia, Italy
| | - Antonio Giovanni Solimando
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
- IRCCS Istituto Tumori “Giovanni Paolo II” of Bari, Viale Orazio Flacco 65, I-70124 Bari, Italy
| | - Roberta Buono
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Nicola Susca
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Gianfranco Inglese
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Assunta Melaccio
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Marcella Prete
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Roberto Ria
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Vito Racanelli
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
| | - Angelo Vacca
- Unit of Internal Medicine “Guido Baccelli”, Department of Biomedical Sciences and Human Oncology, University of Bari Aldo Moro Medical School, Piazza Giulio Cesare 11, I-70124 Bari, Italy; (S.C.); (A.G.S.); (R.B.); (N.S.); (G.I.); (A.M.); (M.P.); (R.R.); (V.R.)
- Correspondence:
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Oda S, Kidoh M, Nagayama Y, Takashio S, Usuku H, Ueda M, Yamashita T, Ando Y, Tsujita K, Yamashita Y. Trends in Diagnostic Imaging of Cardiac Amyloidosis: Emerging Knowledge and Concepts. Radiographics 2020; 40:961-981. [DOI: 10.1148/rg.2020190069] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Affiliation(s)
- Seitaro Oda
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Masafumi Kidoh
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Yasunori Nagayama
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Seiji Takashio
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Hiroki Usuku
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Mitsuharu Ueda
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Taro Yamashita
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Yukio Ando
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Kenichi Tsujita
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
| | - Yasuyuki Yamashita
- From the Departments of Diagnostic Radiology (S.O., M.K., Y.N., Y.Y.), Cardiovascular Medicine (S.T., H.U., K.T.), Molecular Laboratory Medicine (H.U.), and Neurology (M.U., T.Y., Y.A.), Faculty of Life Sciences, Kumamoto University, 1-1-1 Honjyo, Chuo-ku, Kumamoto 860-8556, Japan
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Diagnostic value of the novel CMR parameter "myocardial transit-time" (MyoTT) for the assessment of microvascular changes in cardiac amyloidosis and hypertrophic cardiomyopathy. Clin Res Cardiol 2020; 110:136-145. [PMID: 32372287 PMCID: PMC7806531 DOI: 10.1007/s00392-020-01661-6] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/04/2020] [Accepted: 04/29/2020] [Indexed: 01/26/2023]
Abstract
BACKGROUND Coronary microvascular dysfunction (CMD) is present in various non-ischemic cardiomyopathies and in particular in those with left-ventricular hypertrophy. This study evaluated the diagnostic value of the novel cardiovascular magnetic resonance (CMR) parameter "myocardial transit-time" (MyoTT) in distinguishing cardiac amyloidosis from other hypertrophic cardiomyopathies. METHODS N = 20 patients with biopsy-proven cardiac amyloidosis (CA), N = 20 patients with known hypertrophic cardiomyopathy (HCM), and N = 20 control patients without relevant cardiac disease underwent dedicated CMR studies on a 1.5-T MR scanner. The CMR protocol comprised cine and late-gadolinium-enhancement (LGE) imaging as well as first-pass perfusion acquisitions at rest for MyoTT measurement. MyoTT was defined as the blood circulation time from the orifice of the coronary arteries to the pooling in the coronary sinus (CS) reflecting the transit-time of gadolinium in the myocardial microvasculature. RESULTS MyoTT was significantly prolonged in patients with CA compared to both groups: 14.8 ± 4.1 s in CA vs. 12.2 ± 2.5 s in HCM (p = 0.043) vs. 7.2 ± 2.6 s in controls (p < 0.001). Native T1 and extracellular volume (ECV) were significantly higher in CA compared to HCM and controls (p < 0.001). Both parameters were associated with a higher diagnostic accuracy in predicting the presence of CA compared to MyoTT: area under the curve (AUC) for native T1 = 0.93 (95% confidence interval (CI) = 0.83-1.00; p < 0.001) and AUC for ECV = 0.95 (95% CI = 0.88-1.00; p < 0.001)-compared to the AUC for MyoTT = 0.76 (95% CI = 0.60-0.92; p = 0.008). In contrast, MyoTT performed better than all other CMR parameters in differentiating HCM from controls (AUC for MyoTT = 0.93; 95% CI = 0.81-1.00; p = 0.003 vs. AUC for native T1 = 0.69; 95% CI = 0.44-0.93; p = 0.20 vs. AUC for ECV = 0.85; 95% CI = 0.66-1.00; p = 0.017). CONCLUSION The relative severity of CMD (measured by MyoTT) in relationship to extracellular changes (measured by native T1 and/or ECV) is more pronounced in HCM compared to CA-in spite of a higher absolute MyoTT value in CA patients. Hence, MyoTT may improve our understanding of the interplay between extracellular/intracellular and intravasal changes that occur in the myocardium during the disease course of different cardiomyopathies.
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Diagnostic Performance of Abnormal Nulling on Cardiac Magnetic Resonance Imaging Look Locker Inversion Time Sequence in Differentiating Cardiac Amyloidosis Types. J Thorac Imaging 2020; 35:334-339. [DOI: 10.1097/rti.0000000000000493] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023]
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Cuddy S, Dorbala S, Di Carli MF. Imaging of cardiac amyloidosis: Will this become a unique application for dual-isotope imaging? J Nucl Cardiol 2020; 27:38-40. [PMID: 31214938 PMCID: PMC6918001 DOI: 10.1007/s12350-019-01754-4] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2019] [Accepted: 04/16/2019] [Indexed: 01/15/2023]
Affiliation(s)
- Sarah Cuddy
- Cardiovascular Imaging Program, Departments of Medicine and Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
- Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
- Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
| | - Sharmila Dorbala
- Cardiovascular Imaging Program, Departments of Medicine and Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
- Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
- Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA
| | - Marcelo F Di Carli
- Cardiovascular Imaging Program, Departments of Medicine and Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
- Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
- Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
- Brigham and Women's Hospital, ASB-L1 037C, 75 Francis St, Boston, MA, 02115, USA.
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Baggiano A, Boldrini M, Martinez-Naharro A, Kotecha T, Petrie A, Rezk T, Gritti M, Quarta C, Knight DS, Wechalekar AD, Lachmann HJ, Perlini S, Pontone G, Moon JC, Kellman P, Gillmore JD, Hawkins PN, Fontana M. Noncontrast Magnetic Resonance for the Diagnosis of Cardiac Amyloidosis. JACC Cardiovasc Imaging 2020; 13:69-80. [DOI: 10.1016/j.jcmg.2019.03.026] [Citation(s) in RCA: 82] [Impact Index Per Article: 16.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2019] [Accepted: 03/16/2019] [Indexed: 11/16/2022]
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Dorbala S, Ando Y, Bokhari S, Dispenzieri A, Falk RH, Ferrari VA, Fontana M, Gheysens O, Gillmore JD, Glaudemans AWJM, Hanna MA, Hazenberg BPC, Kristen AV, Kwong RY, Maurer MS, Merlini G, Miller EJ, Moon JC, Murthy VL, Quarta CC, Rapezzi C, Ruberg FL, Shah SJ, Slart RHJA, Verberne HJ, Bourque JM. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 1 of 2-evidence base and standardized methods of imaging. J Nucl Cardiol 2019; 26:2065-2123. [PMID: 31468376 DOI: 10.1007/s12350-019-01760-6] [Citation(s) in RCA: 241] [Impact Index Per Article: 40.2] [Reference Citation Analysis] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
- Sharmila Dorbala
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA.
| | - Yukio Ando
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
| | - Sabahat Bokhari
- Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, New York, NY, USA
| | - Angela Dispenzieri
- Division of Hematology, Division of Cardiovascular Diseases, and Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, MN, USA
| | - Rodney H Falk
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA
| | - Victor A Ferrari
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | - Marianna Fontana
- Division of Medicine, National Amyloidosis Centre, University College London, London, UK
| | - Olivier Gheysens
- Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium
| | - Julian D Gillmore
- Division of Medicine, National Amyloidosis Centre, University College London, London, UK
| | - Andor W J M Glaudemans
- Department of Nuclear Medicine and Molecular Imaging, Medical Imaging Center, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Mazen A Hanna
- Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH, USA
| | - Bouke P C Hazenberg
- Department of Rheumatology & Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Arnt V Kristen
- Department of Cardiology, University of Heidelberg, Heidelberg, Germany
| | - Raymond Y Kwong
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, MA, USA
| | - Mathew S Maurer
- Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, New York, NY, USA
| | - Giampaolo Merlini
- Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy
- Department of Molecular Medicine, University of Pavia, Pavia, Italy
| | - Edward J Miller
- Cardiovascular Medicine, Yale University School of Medicine, New Haven, CT, USA
| | - James C Moon
- Division of Medicine, National Amyloidosis Centre, University College London, London, UK
| | | | - C Cristina Quarta
- Division of Medicine, National Amyloidosis Centre, University College London, London, UK
| | - Claudio Rapezzi
- Cardiology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater-University of Bologna, Bologna, Italy
| | - Frederick L Ruberg
- Amyloidosis Center and Section of Cardiovascular Medicine, Department of Medicine, Boston University School of Medicine, Boston Medical Center, Boston, MA, USA
| | - Sanjiv J Shah
- Feinberg School of Medicine, Northwestern University, Chicago, IL, USA
| | - Riemer H J A Slart
- Department of Nuclear Medicine and Molecular Imaging, Medical Imaging Center, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Hein J Verberne
- Department of Radiology and Nuclear Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
| | - Jamieson M Bourque
- Departments of Medicine and Radiology, Cardiovascular Imaging Center, University of Virginia, Charlottesville, VA, USA
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Dorbala S, Ando Y, Bokhari S, Dispenzieri A, Falk RH, Ferrari VA, Fontana M, Gheysens O, Gillmore JD, Glaudemans AWJM, Hanna MA, Hazenberg BPC, Kristen AV, Kwong RY, Maurer MS, Merlini G, Miller EJ, Moon JC, Murthy VL, Quarta CC, Rapezzi C, Ruberg FL, Shah SJ, Slart RHJA, Verberne HJ, Bourque JM. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 1 of 2-Evidence Base and Standardized Methods of Imaging. J Card Fail 2019; 25:e1-e39. [PMID: 31473268 DOI: 10.1016/j.cardfail.2019.08.001] [Citation(s) in RCA: 96] [Impact Index Per Article: 16.0] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Affiliation(s)
- Sharmila Dorbala
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, Massachusetts.
| | - Yukio Ando
- Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
| | - Sabahat Bokhari
- Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, New York, New York
| | - Angela Dispenzieri
- Division of Hematology, Division of Cardiovascular Diseases, and Department of Radiology, Division of Nuclear Medicine, Department of Medicine, Mayo Clinic, Rochester, Minnesota
| | - Rodney H Falk
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, Massachusetts
| | - Victor A Ferrari
- Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania
| | - Marianna Fontana
- Division of Medicine, National Amyloidosis Centre, University College London, London, United Kingdom
| | - Olivier Gheysens
- Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium
| | - Julian D Gillmore
- Division of Medicine, National Amyloidosis Centre, University College London, London, United Kingdom
| | - Andor W J M Glaudemans
- Department of Nuclear Medicine and Molecular Imaging, Medical Imaging Center, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Mazen A Hanna
- Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio
| | - Bouke P C Hazenberg
- Department of Rheumatology & Clinical Immunology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Arnt V Kristen
- Department of Cardiology, University of Heidelberg, Heidelberg, Germany
| | - Raymond Y Kwong
- Cardiac Amyloidosis Program, Cardiovascular Imaging Program, Departments of Radiology and Medicine, Harvard Medical School, Brigham and Women's Hospital, Boston, Massachusetts
| | - Mathew S Maurer
- Columbia University Medical Center/New York Presbyterian Hospital, Columbia University, New York, New York
| | - Giampaolo Merlini
- Amyloidosis Research and Treatment Center, Foundation Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Pavia, Italy
| | - Edward J Miller
- Cardiovascular Medicine, Yale University School of Medicine, New Haven, Connecticut
| | - James C Moon
- Division of Medicine, National Amyloidosis Centre, University College London, London, United Kingdom
| | | | - C Cristina Quarta
- Division of Medicine, National Amyloidosis Centre, University College London, London, United Kingdom
| | - Claudio Rapezzi
- Cardiology Unit, Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater-University of Bologna, Bologna, Italy
| | - Frederick L Ruberg
- Amyloidosis Center and Section of Cardiovascular Medicine, Department of Medicine, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts
| | - Sanjiv J Shah
- Feinberg School of Medicine, Northwestern University, Chicago, Illinois
| | - Riemer H J A Slart
- Department of Nuclear Medicine and Molecular Imaging, Medical Imaging Center, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
| | - Hein J Verberne
- Department of Radiology and Nuclear Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands
| | - Jamieson M Bourque
- Departments of Medicine and Radiology, Cardiovascular Imaging Center, University of Virginia, Charlottesville, Virginia
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Dorbala S, Cuddy S, Falk RH. How to Image Cardiac Amyloidosis: A Practical Approach. JACC Cardiovasc Imaging 2019; 13:1368-1383. [PMID: 31607664 DOI: 10.1016/j.jcmg.2019.07.015] [Citation(s) in RCA: 173] [Impact Index Per Article: 28.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2018] [Revised: 06/21/2019] [Accepted: 07/28/2019] [Indexed: 12/21/2022]
Abstract
Cardiac amyloidosis (CA) is one of the most rapidly progressive forms of heart disease, with a median survival from diagnosis, if untreated, ranging from <6 months for light chain amyloidosis to 3 to 5 years for transthyretin amyloidosis. Early diagnosis and accurate typing of CA are necessary for optimal management of these patients. Emerging novel disease modifying therapies increase the urgency to diagnose CA at an early stage and identify patients who may benefit from these life-saving therapies. The goal of this review is to provide a practical approach to echocardiography, cardiac magnetic resonance, and radionuclide imaging in patients with known or suspected CA.
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Affiliation(s)
- Sharmila Dorbala
- Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts; CV Imaging Program, Cardiovascular Division, Brigham and Women's Hospital, Boston, Massachusetts; Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts.
| | - Sarah Cuddy
- Division of Nuclear Medicine, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts; CV Imaging Program, Cardiovascular Division, Brigham and Women's Hospital, Boston, Massachusetts; Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts
| | - Rodney H Falk
- Cardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts
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Kotecha T, Martinez-Naharro A, Treibel TA, Francis R, Nordin S, Abdel-Gadir A, Knight DS, Zumbo G, Rosmini S, Maestrini V, Bulluck H, Rakhit RD, Wechalekar AD, Gilbertson J, Sheppard MN, Kellman P, Gillmore JD, Moon JC, Hawkins PN, Fontana M. Myocardial Edema and Prognosis in Amyloidosis. J Am Coll Cardiol 2019; 71:2919-2931. [PMID: 29929616 DOI: 10.1016/j.jacc.2018.03.536] [Citation(s) in RCA: 139] [Impact Index Per Article: 23.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Revised: 03/26/2018] [Accepted: 03/26/2018] [Indexed: 12/15/2022]
Abstract
BACKGROUND Prognosis in light-chain (AL) and transthyretin (ATTR) amyloidosis is influenced by cardiac involvement. ATTR amyloidosis has better prognosis than AL amyloidosis despite more amyloid infiltration, suggesting additional mechanisms of damage in AL amyloidosis. OBJECTIVES The aim of the study was to assess the presence and prognostic significance of myocardial edema in patients with amyloidosis. METHODS The study recruited 286 patients: 100 with systemic AL amyloidosis, 163 with cardiac ATTR amyloidosis, 12 with suspected cardiac ATTR amyloidosis (grade 1 on 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid), 11 asymptomatic individuals with amyloidogenic TTR gene mutations, and 30 healthy volunteers. All subjects underwent cardiovascular magnetic resonance with T1 and T2 mapping and 16 underwent endomyocardial biopsy. RESULTS Myocardial T2 was increased in amyloidosis with the degree of elevation being highest in untreated AL patients (untreated AL amyloidosis 56.6 ± 5.1 ms; treated AL amyloidosis 53.6 ± 3.9 ms; ATTR amyloidosis 54.2 ± 4.1 ms; each p < 0.01 compared with control subjects: 48.9 ± 2.0 ms). Left ventricular (LV) mass and extracellular volume fraction were higher in ATTR amyloidosis compared with AL amyloidosis while LV ejection fraction was lower (p < 0.001). Histological evidence of edema was present in 87.5% of biopsy samples ranging from 5% to 40% myocardial involvement. Using Cox regression models, myocardial T2 predicted death in AL amyloidosis (hazard ratio: 1.48; 95% confidence interval: 1.20 to 1.82) and remained significant after adjusting for extracellular volume fraction and N-terminal pro-B-type natriuretic peptide (hazard ratio: 1.32; 95% confidence interval: 1.05 to 1.67). CONCLUSIONS Myocardial edema is present in cardiac amyloidosis by histology and cardiovascular magnetic resonance T2 mapping. T2 is higher in untreated AL amyloidosis compared with treated AL and ATTR amyloidosis, and is a predictor of prognosis in AL amyloidosis. This suggests mechanisms additional to amyloid infiltration contributing to mortality in amyloidosis.
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Affiliation(s)
- Tushar Kotecha
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom; Royal Free Hospital, London, United Kingdom
| | - Ana Martinez-Naharro
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom
| | - Thomas A Treibel
- Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, London, United Kingdom
| | - Rohin Francis
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom
| | - Sabrina Nordin
- Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, London, United Kingdom
| | - Amna Abdel-Gadir
- Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, London, United Kingdom
| | - Daniel S Knight
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Royal Free Hospital, London, United Kingdom
| | - Giulia Zumbo
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom
| | | | - Viviana Maestrini
- Barts Heart Centre, London, United Kingdom; Department of Cardiovascular, Respiratory, Nephrology, Anesthesiology & Geriatric Sciences, "Sapienza" University of Rome, Rome, Italy
| | - Heerajnarain Bulluck
- Institute of Cardiovascular Science, University College London, London, United Kingdom
| | - Roby D Rakhit
- Institute of Cardiovascular Science, University College London, London, United Kingdom; Royal Free Hospital, London, United Kingdom
| | - Ashutosh D Wechalekar
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Royal Free Hospital, London, United Kingdom
| | - Janet Gilbertson
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom
| | - Mary N Sheppard
- Molecular and Clinical Sciences Research Institute, St. George's, University of London, London, United Kingdom
| | - Peter Kellman
- National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland
| | - Julian D Gillmore
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Royal Free Hospital, London, United Kingdom
| | | | - Philip N Hawkins
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Royal Free Hospital, London, United Kingdom
| | - Marianna Fontana
- National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom; Royal Free Hospital, London, United Kingdom.
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Mrsic Z, Mousavi N, Hulten E, Bittencourt MS. The Prognostic Value of Late Gadolinium Enhancement in Nonischemic Heart Disease. Magn Reson Imaging Clin N Am 2019; 27:545-561. [DOI: 10.1016/j.mric.2019.04.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
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Cholet C, Damy T, Legou F, Kobeiter H, Rahmouni A, Deux JF. Quantification of Myocardial Enhancement on Cine-MRI: Diagnostic Value in Cardiac Amyloidosis. Acad Radiol 2019; 26:e98-e107. [PMID: 30072291 DOI: 10.1016/j.acra.2018.06.021] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/01/2018] [Revised: 06/25/2018] [Accepted: 06/25/2018] [Indexed: 01/15/2023]
Abstract
RATIONALE AND OBJECTIVES Diagnosis of cardiac amyloidosis (CA) on cardiac magnetic resonance (CMR) can be challenging and quantitative indexes are relevant to further characterize the myocardium. We hypothesize that the relative myocardial enhancement measured from pre and post contrast cine imaging provides diagnostic information for CA in the setting of left ventricular hypertrophy (LVH). MATERIALS AND METHODS Patients with LVH referred to our center and control subjects with normal CMR were retrospectively included. Percentage of myocardial enhancement (percentage ME) was obtained from pre and post contrast (5 minutes) cine sequences. Post contrast myocardial T1 and LGE extent were also recorded. RESULTS Twenty-one patients with CA, 25 patients with non-amyloid left ventricular myocardial hypertrophy (CH) and 20 controls with normal CMR were analyzed. Percentage ME was significantly higher in CA patients (200% (174-238)) than in CH patients (122% (88-151); p = 0.0001) and control patients (104% (90-149); p = 0.0001). Percentage ME was significantly correlated with the LGE extent (Rho Spearman coefficient = 0.66; p = 0.0001) and with the post contrast myocardial T1 (Rho Spearman coefficient = -0.61; p = 0.0001). With a cutoff value of 152%, the sensitivity and specificity of percentage ME for detection of CA were 90% and 80%, respectively. CONCLUSION Percentage ME obtained from pre and post contrast cine imaging is correlated to LGE extent and myocardial T1 and may represent an additional diagnostic parameter to consider CA in patients with LVH.
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Affiliation(s)
- Clément Cholet
- Assistance Publique - Hôpitaux de Paris (AP-HP), Service d'Imagerie Médicale, CHU Henri Mondor, 51 avenue du Maréchal de Lattre de Tassigny, Créteil, 94010, France.
| | - Thibaud Damy
- Assistance Publique - Hôpitaux de Paris (AP-HP), Service de Cardiologie, CHU Henri Mondor, 51 avenue du Maréchal de Lattre de Tassigny, Créteil, 94010, France; Université Paris-Est Créteil (UPEC), Créteil, 94010, France; GRC Amyloid Research Institute and Réseau Amylose Mondor, CHU Henri Mondor, UPEC, Créteil, 94010, France; DHU, ATVB, UPEC, Créteil, 94010, France.
| | - François Legou
- Assistance Publique - Hôpitaux de Paris (AP-HP), Service d'Imagerie Médicale, CHU Henri Mondor, 51 avenue du Maréchal de Lattre de Tassigny, Créteil, 94010, France.
| | - Hicham Kobeiter
- Assistance Publique - Hôpitaux de Paris (AP-HP), Service d'Imagerie Médicale, CHU Henri Mondor, 51 avenue du Maréchal de Lattre de Tassigny, Créteil, 94010, France; Université Paris-Est Créteil (UPEC), Créteil, 94010, France; DHU, ATVB, UPEC, Créteil, 94010, France.
| | - Alain Rahmouni
- Assistance Publique - Hôpitaux de Paris (AP-HP), Service d'Imagerie Médicale, CHU Henri Mondor, 51 avenue du Maréchal de Lattre de Tassigny, Créteil, 94010, France; Université Paris-Est Créteil (UPEC), Créteil, 94010, France.
| | - Jean-François Deux
- Assistance Publique - Hôpitaux de Paris (AP-HP), Service d'Imagerie Médicale, CHU Henri Mondor, 51 avenue du Maréchal de Lattre de Tassigny, Créteil, 94010, France; Université Paris-Est Créteil (UPEC), Créteil, 94010, France; GRC Amyloid Research Institute and Réseau Amylose Mondor, CHU Henri Mondor, UPEC, Créteil, 94010, France; DHU, ATVB, UPEC, Créteil, 94010, France.
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Lipshultz SE, Law YM, Asante-Korang A, Austin ED, Dipchand AI, Everitt MD, Hsu DT, Lin KY, Price JF, Wilkinson JD, Colan SD. Cardiomyopathy in Children: Classification and Diagnosis: A Scientific Statement From the American Heart Association. Circulation 2019; 140:e9-e68. [PMID: 31132865 DOI: 10.1161/cir.0000000000000682] [Citation(s) in RCA: 190] [Impact Index Per Article: 31.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
In this scientific statement from the American Heart Association, experts in the field of cardiomyopathy (heart muscle disease) in children address 2 issues: the most current understanding of the causes of cardiomyopathy in children and the optimal approaches to diagnosis cardiomyopathy in children. Cardiomyopathies result in some of the worst pediatric cardiology outcomes; nearly 40% of children who present with symptomatic cardiomyopathy undergo a heart transplantation or die within the first 2 years after diagnosis. The percentage of children with cardiomyopathy who underwent a heart transplantation has not declined over the past 10 years, and cardiomyopathy remains the leading cause of transplantation for children >1 year of age. Studies from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry have shown that causes are established in very few children with cardiomyopathy, yet genetic causes are likely to be present in most. The incidence of pediatric cardiomyopathy is ≈1 per 100 000 children. This is comparable to the incidence of such childhood cancers as lymphoma, Wilms tumor, and neuroblastoma. However, the published research and scientific conferences focused on pediatric cardiomyopathy are sparcer than for those cancers. The aim of the statement is to focus on the diagnosis and classification of cardiomyopathy. We anticipate that this report will help shape the future research priorities in this set of diseases to achieve earlier diagnosis, improved clinical outcomes, and better quality of life for these children and their families.
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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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Abstract
Heart failure is a clinical syndrome with a broad spectrum of presentations. Cardiovascular imaging techniques such as echocardiography, cardiovascular magnetic resonance, computed tomography, and nuclear imaging play a crucial role in diagnosis, guiding management, and providing prognostic information. Each of these imaging modalities has their own respective strengths and weaknesses. Cardiac imaging can help differentiate between ischemic and nonischemic cardiomyopathies. Additionally, imaging techniques can display disease-specific findings, aiding in diagnosis of nonischemic cardiomyopathies and can provide a means to monitor response to therapy. The choice of imaging modality in the workup of cardiomyopathy should be based on the specific clinical question and the knowledge of the strengths and limitations of each imaging modality.
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Affiliation(s)
- Kate Rankin
- Division of Cardiology, Peter Munk Cardiac Center, Ted Rogers Program in Cardiotoxicity Prevention, Toronto General Hospital, University Health Network, University of Toronto, 4N-490, 585 University Avenue, Toronto, Ontario M5G 2N2, Canada
| | - Babitha Thampinathan
- Division of Cardiology, Peter Munk Cardiac Center, Ted Rogers Program in Cardiotoxicity Prevention, Toronto General Hospital, University Health Network, University of Toronto, 4N-490, 585 University Avenue, Toronto, Ontario M5G 2N2, Canada
| | - Paaladinesh Thavendiranathan
- Division of Cardiology, Peter Munk Cardiac Center, Ted Rogers Program in Cardiotoxicity Prevention, Toronto General Hospital, University Health Network, University of Toronto, 4N-490, 585 University Avenue, Toronto, Ontario M5G 2N2, Canada.
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Correction to: Reference Ranges for the Size of the Fetal Cardiac Outflow Tracts From 13 to 36 Weeks Gestation: A Single-Center Study of Over 7000 Cases. Circ Cardiovasc Imaging 2019; 12:e000025. [PMID: 30866649 DOI: 10.1161/hci.0000000000000025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
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Abstract
PURPOSE OF REVIEW The aim is to provide a description of the most important echocardiographic features in systemic amyloidosis. RECENT FINDINGS Amyloidosis is a heterogeneous group of multisystem disorders, characterized by an extracellular deposition of amyloid fibrils. Several imaging tests are available for the diagnosis; however, echocardiography is the cornerstone of the non-invasive imaging modality for cardiac amyloidosis. So far, little is known about the diagnosis of cardiac amyloidosis through imaging modalities. We summarized the most important echocardiographic findings in cardiac amyloidosis. Hence, we offered a systematic report of the diagnostic performance of cardiac amyloidosis using echocardiography.
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Mahalingam H, Chacko BR, Irodi A, Joseph E, Vimala LR, Thomson VS. Myocardial nulling pattern in cardiac amyloidosis on time of inversion scout magnetic resonance imaging sequence - A new observation of temporal variability. Indian J Radiol Imaging 2019; 28:427-432. [PMID: 30662203 PMCID: PMC6319092 DOI: 10.4103/ijri.ijri_84_18] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023] Open
Abstract
Context: The pattern of myocardial nulling in the inversion scout sequence [time of inversion scout (TIS)] of cardiac magnetic resonance imaging (MRI) is an accurate tool to detect cardiac amyloidosis. The pattern of nulling of myocardium and blood at varying times post gadolinium injection and its relationship with left ventricular mass (LVM) in amyloidosis have not been described previously. Aims: The aim is to study the nulling pattern of myocardium and blood at varying times in TIS and assess its relationship with LVM and late gadolinium enhancement (LGE) in amyloidosis. Materials and Methods: This was a retrospective study of 109 patients with clinical suspicion of cardiac amyloidosis who underwent MRI. Of these, 30 had MRI features of amyloidosis. The nulling pattern was assessed at 5 (TIS5min) and 10 (TIS10min) minutes (min) post contrast injection. Nulling pattern was also assessed at 3min (TIS3min) in four patients and 7min (TIS7min) in five patients. Myocardial mass index was calculated. Mann-Whitney U test was done to assess statistical difference in the myocardial mass index between patients with and without reversed nulling pattern (RNP) at TIS5min. Results: RNP was observed in 58% at TIS5min and 89.6% at TIS10min. Myocardial mass index was significantly higher in patients with RNP at TIS5min[mean = 94.87 g/m2; standard deviation (SD) =17.63) when compared with patients with normal pattern (mean = 77.61 g/m2; SD = 17.21) (U = 18; P = 0.0351). Conclusion: In cardiac amyloidosis, TIS sequence shows temporal variability in nulling pattern. Earlier onset of reverse nulling pattern shows a trend toward more LVM and possibly more severe amyloid load.
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Affiliation(s)
| | - Binita Riya Chacko
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Aparna Irodi
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Elizabeth Joseph
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Leena R Vimala
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Viji Samuel Thomson
- Department of Cardiology, Christian Medical College, Vellore, Tamil Nadu, India
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Massalha S, Ruddy TD. Emerging role of echocardiography, cardiac magnetic resonance imaging and 99mTc-labeled bone tracer scintigraphy for the diagnosis of cardiac amyloidosis. J Nucl Cardiol 2018; 25:2080-2083. [PMID: 28585032 DOI: 10.1007/s12350-017-0943-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2017] [Accepted: 05/22/2017] [Indexed: 10/19/2022]
Affiliation(s)
- Samia Massalha
- Division of Cardiology, University of Ottawa Heart Institute, 40 Ruskin Street, Room H1220, Ottawa, ON, K1Y 4W7, Canada
- Department of Medicine, University of Ottawa, Ottawa, ON, Canada
| | - Terrence D Ruddy
- Division of Cardiology, University of Ottawa Heart Institute, 40 Ruskin Street, Room H1220, Ottawa, ON, K1Y 4W7, Canada.
- Department of Medicine, University of Ottawa, Ottawa, ON, Canada.
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Méndez C, Soler R, Rodríguez E, Barriales R, Ochoa JP, Monserrat L. Differential diagnosis of thickened myocardium: an illustrative MRI review. Insights Imaging 2018; 9:695-707. [PMID: 30302634 PMCID: PMC6206373 DOI: 10.1007/s13244-018-0655-9] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2018] [Revised: 07/18/2018] [Accepted: 08/07/2018] [Indexed: 02/07/2023] Open
Abstract
Objectives The purpose of this article is to describe the key cardiac magnetic resonance imaging (MRI) features to differentiate hypertrophic cardiomyopathy (HCM) phenotypes from other causes of myocardial thickening that may mimic them. Conclusions Many causes of myocardial thickening may mimic different HCM phenotypes. The unique ability of cardiac MRI to facilitate tissue characterisation may help to establish the aetiology of myocardial thickening, which is essential to differentiate it from HCM phenotypes and for appropriate management. Teaching points • Many causes of myocardial thickening may mimic different HCM phenotypes. • Differential diagnosis between myocardial thickening aetiology and HCM phenotypes may be challenging. • Cardiac MRI is essential to differentiate the aetiology of myocardial thickening from HCM phenotypes.
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Affiliation(s)
- Cristina Méndez
- Radiology Department, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña (INIBIC), Servizo Galego de Saúde (SERGAS), Universidade da Coruña, Xubias de Arriba 86, 15006, A Coruña, Spain
| | - Rafaela Soler
- Radiology Department, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña (INIBIC), Servizo Galego de Saúde (SERGAS), Universidade da Coruña, Xubias de Arriba 86, 15006, A Coruña, Spain
| | - Esther Rodríguez
- Radiology Department, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña (INIBIC), Servizo Galego de Saúde (SERGAS), Universidade da Coruña, Xubias de Arriba 86, 15006, A Coruña, Spain.
| | - Roberto Barriales
- Cardiology Department, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña (INIBIC), Servizo Galego de Saúde (SERGAS), Universidade da Coruña, Xubias de Arriba, 84, 15006, A Coruña, Spain
| | - Juan Pablo Ochoa
- Cardiology Department, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña (INIBIC), Servizo Galego de Saúde (SERGAS), Universidade da Coruña, Xubias de Arriba, 84, 15006, A Coruña, Spain
| | - Lorenzo Monserrat
- Cardiology Department, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña (INIBIC), Servizo Galego de Saúde (SERGAS), Universidade da Coruña, Xubias de Arriba, 84, 15006, A Coruña, Spain
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