Author + information
- Received October 11, 2013
- Accepted October 17, 2013
- Published online February 1, 2014.
- Marianna Fontana, MD∗,†,‡,
- Sanjay M. Banypersad, MB ChB∗,†,‡,
- Thomas A. Treibel, MBBS∗,†,
- Viviana Maestrini, MD∗,§,
- Daniel M. Sado, BSc, BM∗,†,
- Steven K. White, BSc, MB ChB∗,†,
- Silvia Pica, MD∗,
- Silvia Castelletti, MD∗,
- Stefan K. Piechnik, PhD, MScEE‖,
- Matthew D. Robson, PhD‖,
- Janet A. Gilbertson, CSci‡,
- Dorota Rowczenio, CSci‡,
- David F. Hutt, BAppSc‡,
- Helen J. Lachmann, MD‡,
- Ashutosh D. Wechalekar, MD‡,
- Carol J. Whelan, MD‡,
- Julian D. Gillmore, MD, PhD‡,
- Philip N. Hawkins, PhD‡ and
- James C. Moon, MD∗,†∗ ()
- ∗The Heart Hospital, London, United Kingdom
- †Institute of Cardiovascular Science, University College London, London, United Kingdom
- ‡National Amyloidosis Centre, University College London, London, United Kingdom
- §Department of Cardiovascular, Respiratory, Nephrologic, Anaesthesiologic and Geriatric Science, La Sapienza University of Rome, Rome, Italy
- ‖Division of Cardiovascular Medicine, Radcliffe Department of Medicine, Oxford Centre for Clinical Magnetic Resonance Research, University of Oxford, John Radcliffe Hospital, Oxford, United Kingdom
- ↵∗Reprint requests and correspondence:
Dr. James C. Moon, The Heart Hospital Imaging Centre, 16-18 Westmoreland Street, London W1G 8PH, United Kingdom.
Objectives The aims of the study were to explore the ability of native myocardial T1 mapping by cardiac magnetic resonance to: 1) detect cardiac involvement in patients with transthyretin amyloidosis (ATTR amyloidosis); 2) track the cardiac amyloid burden; and 3) detect early disease.
Background ATTR amyloidosis is an underdiagnosed cause of heart failure, with no truly quantitative test. In cardiac immunoglobulin light-chain amyloidosis (AL amyloidosis), T1 has high diagnostic accuracy and tracks disease. Here, the diagnostic role of native T1 mapping in the other key type of cardiac amyloid, ATTR amyloidosis, is assessed.
Methods A total of 3 groups were studied: ATTR amyloid patients (n = 85; 70 males, age 73 ± 10 years); healthy individuals with transthyretin mutations in whom standard cardiac investigations were normal (n = 8; 3 males, age 47 ± 6 years); and AL amyloid patients (n = 79; 55 males, age 62 ± 10 years). These were compared with 52 healthy volunteers and 46 patients with hypertrophic cardiomyopathy (HCM). All underwent T1 mapping (shortened modified look-locker inversion recovery); ATTR patients and mutation carriers also underwent cardiac 3,3-diphosphono-1,2-propanodicarboxylicacid (DPD) scintigraphy.
Results T1 was elevated in ATTR patients compared with HCM and normal subjects (1,097 ± 43 ms vs. 1,026 ± 64 ms vs. 967 ± 34 ms, respectively; both p < 0.0001). In established cardiac ATTR amyloidosis, T1 elevation was not as high as in AL amyloidosis (AL 1,130 ± 68 ms; p = 0.01). Diagnostic performance was similar for AL and ATTR amyloid (vs. HCM: AL area under the curve 0.84 [95% confidence interval: 0.76 to 0.92]; ATTR area under the curve 0.85 [95% confidence interval: 0.77 to 0.92]; p < 0.0001). T1 tracked cardiac amyloid burden as determined semiquantitatively by DPD scintigraphy (p < 0.0001). T1 was not elevated in mutation carriers (952 ± 35 ms) but was in isolated DPD grade 1 (n = 9, 1,037 ± 60 ms; p = 0.001).
Conclusions Native myocardial T1 mapping detects cardiac ATTR amyloid with similar diagnostic performance and disease tracking to AL amyloid, but with lower maximal T1 elevation, and appears to be an early disease marker.
This paper was partially funded by the British Heart Foundation. Dr. Piechnik's institution has a research agreement with Siemens Medical. Drs. Piechnik and Robson have U.S. patents pending for cardiac gated mapping of T1 (U.S. patent pending 61/387,591; all rights sold exclusively to Siemens Medical) and a color map design method for cardiovascular T1 mapping images (U.S. patent pending 61/689,067). Dr. Moon has received grant funding from GlaxoSmithKline. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received October 11, 2013.
- Accepted October 17, 2013.
- American College of Cardiology Foundation