PMID- 28728692 OWN - NLM STAT- MEDLINE DCOM- 20170901 LR - 20220409 IS - 1558-3597 (Electronic) IS - 0735-1097 (Linking) VI - 70 IP - 4 DP - 2017 Jul 25 TI - Magnetic Resonance in Transthyretin Cardiac Amyloidosis. PG - 466-477 LID - S0735-1097(17)37596-4 [pii] LID - 10.1016/j.jacc.2017.05.053 [doi] AB - BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations. Fifty patients with cardiac light-chain (AL) amyloidosis acted as disease comparators. RESULTS: Unlike cardiac AL amyloidosis, asymmetrical septal left ventricular hypertrophy (LVH) was present in 79% of patients with ATTR (70% sigmoid septum and 30% reverse septal contour), whereas symmetrical LVH was present in 18%, and 3% had no LVH. In patients with cardiac amyloidosis, the pattern of LGE was always typical for amyloidosis (29% subendocardial, 71% transmural), including right ventricular LGE (96%). During follow-up (19 +/- 14 months), 65 patients died. ECV independently correlated with mortality and remained independent after adjustment for age, N-terminal pro-B-type natriuretic peptide, ejection fraction, E/E', and left ventricular mass (hazard ratio: 1.164; 95% confidence interval: 1.066 to 1.271; p < 0.01). CONCLUSIONS: Asymmetrical hypertrophy, traditionally associated with hypertrophic cardiomyopathy, was the commonest pattern of ventricular remodeling in ATTR. LGE imaging was typical in all patients with cardiac ATTR. ECV correlated with amyloid burden and was an independent prognostic factor for survival in this cohort of patients. CI - Copyright (c) 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. FAU - Martinez-Naharro, Ana AU - Martinez-Naharro A AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Division of Medicine, University College London, London, United Kingdom. FAU - Treibel, Thomas A AU - Treibel TA AD - Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, West Smithfield, London, United Kingdom. FAU - Abdel-Gadir, Amna AU - Abdel-Gadir A AD - Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, West Smithfield, London, United Kingdom. FAU - Bulluck, Heerajnarain AU - Bulluck H AD - The Hatter Cardiovascular Institute, Institute of Cardiovascular Science, University College London, United Kingdom. FAU - Zumbo, Giulia AU - Zumbo G AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. FAU - Knight, Daniel S AU - Knight DS AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. FAU - Kotecha, Tushar AU - Kotecha T AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom. FAU - Francis, Rohin AU - Francis R AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; The Hatter Cardiovascular Institute, Institute of Cardiovascular Science, University College London, United Kingdom. FAU - Hutt, David F AU - Hutt DF AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. FAU - Rezk, Tamer AU - Rezk T AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. FAU - Rosmini, Stefania AU - Rosmini S AD - Barts Heart Centre, West Smithfield, London, United Kingdom. FAU - Quarta, Candida C AU - Quarta CC AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. FAU - Whelan, Carol J AU - Whelan CJ AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. FAU - Kellman, Peter AU - Kellman P AD - National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland. FAU - Gillmore, Julian D AU - Gillmore JD AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Division of Medicine, University College London, London, United Kingdom. FAU - Moon, James C AU - Moon JC AD - Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, West Smithfield, London, United Kingdom. FAU - Hawkins, Philip N AU - Hawkins PN AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Division of Medicine, University College London, London, United Kingdom. FAU - Fontana, Marianna AU - Fontana M AD - National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Division of Medicine, University College London, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom. Electronic address: m.fontana@ucl.ac.uk. LA - eng GR - DRF-2013-06-102/DH_/Department of Health/United Kingdom GR - FS/12/56/29723/BHF_/British Heart Foundation/United Kingdom PT - Journal Article PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 RN - Amyloidosis, Hereditary, Transthyretin-Related SB - IM CIN - J Am Coll Cardiol. 2017 Jul 25;70(4):478-480. PMID: 28728693 MH - Aged MH - Amyloid Neuropathies, Familial/*diagnosis MH - Cardiomyopathies/*diagnosis MH - Diagnosis, Differential MH - Female MH - Follow-Up Studies MH - Humans MH - Magnetic Resonance Imaging, Cine/*methods MH - Male MH - Middle Aged MH - Myocardium/*pathology MH - Prognosis MH - Retrospective Studies OTO - NOTNLM OT - ATTR OT - N-terminal pro-B-type natriuretic peptide OT - extracellular volume fraction OT - late gadolinium enhancement OT - left ventricular hypertrophy EDAT- 2017/07/22 06:00 MHDA- 2017/09/02 06:00 CRDT- 2017/07/22 06:00 PHST- 2017/02/21 00:00 [received] PHST- 2017/05/21 00:00 [revised] PHST- 2017/05/24 00:00 [accepted] PHST- 2017/07/22 06:00 [entrez] PHST- 2017/07/22 06:00 [pubmed] PHST- 2017/09/02 06:00 [medline] AID - S0735-1097(17)37596-4 [pii] AID - 10.1016/j.jacc.2017.05.053 [doi] PST - ppublish SO - J Am Coll Cardiol. 2017 Jul 25;70(4):466-477. doi: 10.1016/j.jacc.2017.05.053.