Native T1 Mapping in Transthyretin Amyloidosis

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 ATT...

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Published in:JACC. Cardiovascular imaging 2014-02, Vol.7 (2), p.157-165
Main Authors: Fontana, Marianna, MD, Banypersad, Sanjay M., MB ChB, Treibel, Thomas A., MBBS, Maestrini, Viviana, MD, Sado, Daniel M., BSc, BM, White, Steven K., BSc, MB ChB, Pica, Silvia, MD, Castelletti, Silvia, MD, Piechnik, Stefan K., PhD, MScEE, Robson, Matthew D., PhD, Gilbertson, Janet A., CSci, Rowczenio, Dorota, CSci, Hutt, David F., BAppSc, Lachmann, Helen J., MD, Wechalekar, Ashutosh D., MD, Whelan, Carol J., MD, Gillmore, Julian D., MD, PhD, Hawkins, Philip N., PhD, Moon, James C., MD
Format: Article
Language:English
Subjects:
Amyloidosis - diagnosis
Cardiovascular
Female
Heart Diseases - diagnosis
Humans
Male
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Summary: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 
ISSN:1936-878X
1876-7591
DOI:10.1016/j.jcmg.2013.10.008