BACKGROUND: Cardiac involvement in systemic amyloidosis is caused by the extracellular deposition of misfolded proteins, mainly immunoglobulin light chains (AL) or transthyretin (ATTR), and may be detected by cardiovascular magnetic resonance (CMR). The aim of this study was to measure myocardial extracellular volume (ECV) in amyloid patients with a novel T1 mapping CMR technique and to determine the correlation between ECV and disease severity. METHODS: Thirty-six patients with biopsy-proven systemic amyloidosis (mean age 70 ± 9 years, 31 men, 30 with AL and six with ATTR amyloidosis) and seven patients with possible amyloidosis (mean age 64 ± 10 years, six men) underwent comprehensive clinical and CMR assessment, with ECV estimation from pre- and postcontrast T1 mapping. Thirty healthy subjects (mean age 39 ± 17 years, 21 men) served as the control group. RESULTS: Amyloid patients presented with left ventricular (LV) concentric hypertrophy with impaired biventricular systolic function. Cardiac ECV was higher in amyloid patients (definite amyloidosis, 0.43 ± 0.12; possible amyloidosis, 0.34 ± 0.11) than in control subjects (0.26 ± 0.04, P < 0.05); even in amyloid patients without late gadolinium enhancement (0.35 ± 0.10), ECV was significantly higher than in the control group (P < 0.01). A cut-off value of myocardial ECV >0.316, corresponding to the 95th percentile in normal subjects, showed a sensitivity of 79% and specificity of 97% for discriminating amyloid patients from control subjects (area under the curve of 0.884). Myocardial ECV was significantly correlated with LV ejection fraction (R(2) = 0.16), LV mean wall thickness (R(2) = 0.41), LV diastolic function (R(2) = 0.21), right ventricular ejection fraction (R(2) = 0.13), N-terminal fragment of the pro-brain natriuretic peptides (R(2) = 0.23) and cardiac troponin (R(2) = 0.33). CONCLUSION: Myocardial ECV was increased in amyloid patients and correlated with disease severity. Thus, measurement of myocardial ECV represents a potential noninvasive index of amyloid burden for use in early diagnosis and disease monitoring.
BACKGROUND: Cardiac involvement in systemic amyloidosis is caused by the extracellular deposition of misfolded proteins, mainly immunoglobulin light chains (AL) or transthyretin (ATTR), and may be detected by cardiovascular magnetic resonance (CMR). The aim of this study was to measure myocardial extracellular volume (ECV) in amyloid patients with a novel T1 mapping CMR technique and to determine the correlation between ECV and disease severity. METHODS: Thirty-six patients with biopsy-proven systemic amyloidosis (mean age 70 ± 9 years, 31 men, 30 with AL and six with ATTRamyloidosis) and seven patients with possible amyloidosis (mean age 64 ± 10 years, six men) underwent comprehensive clinical and CMR assessment, with ECV estimation from pre- and postcontrast T1 mapping. Thirty healthy subjects (mean age 39 ± 17 years, 21 men) served as the control group. RESULTS: Amyloid patients presented with left ventricular (LV) concentric hypertrophy with impaired biventricular systolic function. Cardiac ECV was higher in amyloid patients (definite amyloidosis, 0.43 ± 0.12; possible amyloidosis, 0.34 ± 0.11) than in control subjects (0.26 ± 0.04, P < 0.05); even in amyloid patients without late gadolinium enhancement (0.35 ± 0.10), ECV was significantly higher than in the control group (P < 0.01). A cut-off value of myocardial ECV >0.316, corresponding to the 95th percentile in normal subjects, showed a sensitivity of 79% and specificity of 97% for discriminating amyloid patients from control subjects (area under the curve of 0.884). Myocardial ECV was significantly correlated with LV ejection fraction (R(2) = 0.16), LV mean wall thickness (R(2) = 0.41), LV diastolic function (R(2) = 0.21), right ventricular ejection fraction (R(2) = 0.13), N-terminal fragment of the pro-brain natriuretic peptides (R(2) = 0.23) and cardiac troponin (R(2) = 0.33). CONCLUSION: Myocardial ECV was increased in amyloid patients and correlated with disease severity. Thus, measurement of myocardial ECV represents a potential noninvasive index of amyloid burden for use in early diagnosis and disease monitoring.
Authors: Francesco Sardanelli; Simone Schiaffino; Moreno Zanardo; Francesco Secchi; Paola Maria Cannaò; Federico Ambrogi; Giovanni Di Leo Journal: Eur Radiol Date: 2019-05-02 Impact factor: 5.315
Authors: David L Narotsky; Adam Castano; Jonathan W Weinsaft; Sabahat Bokhari; Mathew S Maurer Journal: Can J Cardiol Date: 2016-05-13 Impact factor: 5.223
Authors: Alex F Goodall; David A Broadbent; Raluca B Dumitru; David L Buckley; Ai Lyn Tan; Maya H Buch; John D Biglands Journal: Br J Radiol Date: 2020-05-11 Impact factor: 3.039
Authors: Marianna Fontana; Silvia Pica; Patricia Reant; Amna Abdel-Gadir; Thomas A Treibel; Sanjay M Banypersad; Viviana Maestrini; William Barcella; Stefania Rosmini; Heerajnarain Bulluck; Rabya H Sayed; Ketna Patel; Shameem Mamhood; Chiara Bucciarelli-Ducci; Carol J Whelan; Anna S Herrey; Helen J Lachmann; Ashutosh D Wechalekar; Charlotte H Manisty; Eric B Schelbert; Peter Kellman; Julian D Gillmore; Philip N Hawkins; James C Moon Journal: Circulation Date: 2015-09-11 Impact factor: 29.690