eprintid: 10051749 rev_number: 23 eprint_status: archive userid: 608 dir: disk0/10/05/17/49 datestamp: 2019-10-28 11:32:44 lastmod: 2021-10-11 22:41:53 status_changed: 2019-10-28 11:32:44 type: article metadata_visibility: show creators_name: Martinez-Naharro, A title: Native T1 and ECV in ATTR amyloidosis ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: C09 divisions: D14 keywords: Amyloidosis, ATTR, CMR, ECV note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. abstract: Objectives This study evaluated the prognostic potential of native myocardial T1 in cardiac transthyretin amyloidosis (ATTR) and compared native T1 with extracellular volume (ECV) in terms of diagnostic accuracy and prognosis. Background ATTR is an increasingly recognized cause of heart failure that has an overlapping clinical phenotype with hypertrophic cardiomyopathy (HCM). Native T1 mapping by cardiac magnetic resonance (CMR) is useful for diagnosis in cardiac amyloidosis but its prognostic potential has never been assessed. Methods A total of 134 patients with wild-type ATTR (ATTRwt) (122 men; age 76 ± 7 years), 81 patients with hereditary-type ATTR (ATTRm) (60 men; age 69 ± 11 years), 44 patients with HCM (32 men; age 51 ± 13 years), and 12 asymptomatic mutation carriers (4 men; age 47 ± 10 years) were studied. All subjects underwent CMR with T1 mapping and ECV measurement. ATTR patients also underwent 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy. Results Native T1 and ECV were elevated in ATTR compared with HCM (p < 0.001) and were both associated with a high diagnostic accuracy (area under the curve [AUC]: 0.87; 95% confidence interval [CI]: 0.82 to 0.91) for T1 and an AUC of 0.91 (95% CI: 0.87 to 0.94) for ECV. No significant difference in native T1 and ECV was found between ATTRwt and ATTRm, and ECV correlated well with 99mTc-DPD scintigraphy. During follow-up of a mean of 32 ± 17 months, 55 ATTRwt and 40 ATTRm patients died. Native T1 and ECV predicted death (T1: hazard ratio [HR]: 1.225 for each 59-ms increase; 95% CI: 1.010 to 1.486; p < 0.05; ECV: HR: 1.155 for each 3% increase; 95% CI: 1.097 to 1.216; p < 0.001), but only ECV remained independently predictive after adjustment for age, N-terminal pro−B-type natriuretic peptide, left ventricular ejection fraction, E/E′, left ventricular mass index, DPD grade, and late gadolinium enhancement. Conclusions Native T1 mapping and ECV are good diagnostic techniques for cardiac ATTR that are associated with prognosis. Both parameters correlated with mortality, but only ECV remained independently predictive of prognosis, suggesting that it is a more robust marker in cardiac ATTR. date: 2019-03-01 date_type: published publisher: Elsevier official_url: https://doi.org/10.1016/j.jcmg.2018.02.006 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green article_type_text: Article verified: verified_manual elements_id: 1565829 doi: 10.1016/j.jcmg.2018.02.006 lyricists_name: Knight, Daniel lyricists_name: Martinez De Azcona Naharro, Ana lyricists_id: DSKNI50 lyricists_id: AMARA76 actors_name: Martinez De Azcona Naharro, Ana actors_id: AMARA76 actors_role: owner full_text_status: public publication: JACC: Cardiovascular Imaging volume: 12 number: 5 pagerange: 810-819 issn: 1876-7591 citation: Martinez-Naharro, A; (2019) Native T1 and ECV in ATTR amyloidosis. JACC: Cardiovascular Imaging , 12 (5) pp. 810-819. 10.1016/j.jcmg.2018.02.006 <https://doi.org/10.1016/j.jcmg.2018.02.006>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10051749/1/Native%20T1%20and%20ECV%20in%20ATTR%20amyloidosis.pdf