Andrea Baggiano1, Michele Boldrini2, Ana Martinez-Naharro3, Tushar Kotecha4, Aviva Petrie5, Tamer Rezk3, Maurizio Gritti3, Cristina Quarta3, Daniel S Knight4, Ashutosh D Wechalekar3, Helen J Lachmann3, Stefano Perlini6, Gianluca Pontone7, James C Moon8, Peter Kellman9, Julian D Gillmore3, Philip N Hawkins3, Marianna Fontana10. 1. National Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom; Centro Cardiologico Monzino, IRCCS, Milan, Italy. 2. National Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom; Emergency Department, Amyloid Research and Treatment Center, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy. 3. National Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom. 4. National Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom; Department of Cardiology, Royal Free Hospital, London, United Kingdom. 5. University College London Eastman Dental Institute, London, United Kingdom. 6. Emergency Department, Amyloid Research and Treatment Center, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy. 7. Centro Cardiologico Monzino, IRCCS, Milan, Italy. 8. Barts Heart Centre, St. Bartholomew's Hospital, London, United Kingdom. 9. National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland. 10. National Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom. Electronic address: m.fontana@ucl.ac.uk.
Abstract
OBJECTIVES: This study aimed to assess the diagnostic use of native T1 to detect cardiac amyloidosis (CA) in a large prospective cohort of patients referred for suspected systemic amyloidosis. BACKGROUND: CA is a progressive and fatal underdiagnosed cause of heart failure. Cardiovascular magnetic resonance (CMR) has emerged as an extremely useful test for the non-invasive diagnosis of CA, but administration of contrast is still required to make a diagnosis. METHODS: In this study, 868 patients with suspected CA referred between 2015 and 2017 underwent CMR with late gadolinium enhancement (LGE), T1 mapping, and an array of clinical investigations. RESULTS: The final diagnosis was cardiac light-chain (AL) amyloidosis in 222, cardiac transthyretin (ATTR) amyloidosis in 214, and no cardiac involvement in 427 cases. T1 was significantly elevated in both types of CA and this was associated with high diagnostic accuracy in the overall population (area under the curve, 0.93). A native T1 <1,036 ms was associated with 98% negative predictive value for CA whereas a native T1 >1,164 ms was associated with 98% positive predictive value for CA. We propose the use of these cut-offs to exclude or confirm CA and to restrict the administration of contrast only to patients with intermediate probability (native T1 between 1,036 and 1,164 ms), 58% of patients in this population. CONCLUSIONS: Native myocardial T1 enables diagnosis of CA to be made without need for gadolinium contrast in a large proportion of patients with suspected systemic amyloidosis. We propose a diagnostic algorithm for non-contrast CMR applicable to patients with suspected amyloidosis.
OBJECTIVES: This study aimed to assess the diagnostic use of native T1 to detect cardiac amyloidosis (CA) in a large prospective cohort of patients referred for suspected systemic amyloidosis. BACKGROUND: CA is a progressive and fatal underdiagnosed cause of heart failure. Cardiovascular magnetic resonance (CMR) has emerged as an extremely useful test for the non-invasive diagnosis of CA, but administration of contrast is still required to make a diagnosis. METHODS: In this study, 868 patients with suspected CA referred between 2015 and 2017 underwent CMR with late gadolinium enhancement (LGE), T1 mapping, and an array of clinical investigations. RESULTS: The final diagnosis was cardiac light-chain (AL) amyloidosis in 222, cardiac transthyretin (ATTR) amyloidosis in 214, and no cardiac involvement in 427 cases. T1 was significantly elevated in both types of CA and this was associated with high diagnostic accuracy in the overall population (area under the curve, 0.93). A native T1 <1,036 ms was associated with 98% negative predictive value for CA whereas a native T1 >1,164 ms was associated with 98% positive predictive value for CA. We propose the use of these cut-offs to exclude or confirm CA and to restrict the administration of contrast only to patients with intermediate probability (native T1 between 1,036 and 1,164 ms), 58% of patients in this population. CONCLUSIONS: Native myocardial T1 enables diagnosis of CA to be made without need for gadolinium contrast in a large proportion of patients with suspected systemic amyloidosis. We propose a diagnostic algorithm for non-contrast CMR applicable to patients with suspected amyloidosis.
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Authors: Anke Busse; Rengarajan Rajagopal; Seyrani Yücel; Ebba Beller; Alper Öner; Felix Streckenbach; Daniel Cantré; Hüseyin Ince; Marc-André Weber; Felix G Meinel Journal: Radiologe Date: 2020-11 Impact factor: 0.635