Ana Martinez-Naharro1, Thomas A Treibel2, Amna Abdel-Gadir2, Heerajnarain Bulluck3, Giulia Zumbo4, Daniel S Knight4, Tushar Kotecha5, Rohin Francis6, David F Hutt4, Tamer Rezk4, Stefania Rosmini7, Candida C Quarta4, Carol J Whelan4, Peter Kellman8, Julian D Gillmore1, James C Moon2, Philip N Hawkins1, Marianna Fontana9. 1. National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Division of Medicine, University College London, London, United Kingdom. 2. Institute of Cardiovascular Science, University College London, London, United Kingdom; Barts Heart Centre, West Smithfield, London, United Kingdom. 3. The Hatter Cardiovascular Institute, Institute of Cardiovascular Science, University College London, United Kingdom. 4. National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom. 5. National Amyloidosis Centre, University College London, Royal Free Hospital, London, United Kingdom; Institute of Cardiovascular Science, University College London, London, United Kingdom. 6. 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. 7. Barts Heart Centre, West Smithfield, London, United Kingdom. 8. National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland. 9. 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.
Abstract
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 99mTc-DPD (99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 99mTc-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.
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 99mTc-DPD (99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 99mTc-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.
Authors: Frederick L Ruberg; Martha Grogan; Mazen Hanna; Jeffery W Kelly; Mathew S Maurer Journal: J Am Coll Cardiol Date: 2019-06-11 Impact factor: 24.094
Authors: Sharmila Dorbala; Yukio Ando; Sabahat Bokhari; Angela Dispenzieri; Rodney H Falk; Victor A Ferrari; Marianna Fontana; Olivier Gheysens; Julian D Gillmore; Andor W J M Glaudemans; Mazen A Hanna; Bouke P C Hazenberg; Arnt V Kristen; Raymond Y Kwong; Mathew S Maurer; Giampaolo Merlini; Edward J Miller; James C Moon; Venkatesh L Murthy; C Cristina Quarta; Claudio Rapezzi; Frederick L Ruberg; Sanjiv J Shah; Riemer H J A Slart; Hein J Verberne; Jamieson M Bourque Journal: J Nucl Cardiol Date: 2019-12 Impact factor: 5.952
Authors: Riemer H J A Slart; Andor W J M Glaudemans; Walter Noordzij; Johan Bijzet; Bouke P C Hazenberg; Hans L A Nienhuis Journal: Eur J Nucl Med Mol Imaging Date: 2019-04-23 Impact factor: 9.236