Author + information
- Received March 13, 2019
- Accepted March 16, 2019
- Published online January 6, 2020.
- Andrea Baggiano, MDa,b,∗,
- Michele Boldrini, MDa,c,∗,
- Ana Martinez-Naharro, MDa,
- Tushar Kotecha, MBChBa,d,
- Aviva Petrie, BSc (Hons), MSce,
- Tamer Rezk, MBBSa,
- Maurizio Gritti, MDa,
- Cristina Quarta, MD, PhDa,
- Daniel S. Knight, MBBS, MD (Res)a,d,
- Ashutosh D. Wechalekar, MD, PhDa,
- Helen J. Lachmann, MDa,
- Stefano Perlini, MD, PhDc,
- Gianluca Pontone, MD, PhDb,
- James C. Moon, MDg,
- Peter Kellman, PhDf,
- Julian D. Gillmore, MD, PhDa,
- Philip N. Hawkins, PhD, FMedScia and
- Marianna Fontana, MD, PhDa,∗∗ ()
- aNational Amyloidosis Centre, University College London, Royal Free Campus, London, United Kingdom
- bCentro Cardiologico Monzino, IRCCS, Milan, Italy
- cEmergency Department, Amyloid Research and Treatment Center, IRCCS Policlinico San Matteo Foundation, University of Pavia, Pavia, Italy
- dDepartment of Cardiology, Royal Free Hospital, London, United Kingdom
- eUniversity College London Eastman Dental Institute, London, United Kingdom
- fNational Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland
- gBarts Heart Centre, St. Bartholomew’s Hospital, London, United Kingdom
- ↵∗Address for correspondence:
Dr. Marianna Fontana, Cardiac MRI Unit, National Amyloidosis Centre, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, United Kingdom.
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.
↵∗ Drs. Baggiano and Boldrini are joint first authors.
Dr. Gillmore is an advisory board member for Alnylam, Akcea, and Eidos. Dr. Knight has received a research grant from Actelion Pharmaceuticals Ltd.; and has received advisory board and speaker fees from Actelion Pharmaceuticals Ltd. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received March 13, 2019.
- Accepted March 16, 2019.
- 2020 American College of Cardiology Foundation
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