Myocardial native T2 measurement to differentiate light-chain and transthyretin cardiac amyloidosis and assess prognosis

Abstract

BackgroundTo assess the diagnostic and prognosis value of myocardial native T2 measurement in the distinction between Light-chain (AL) and Transthyretin (ATTR) cardiac amyloidosis (CA).MethodsForty-four patients with CA (24 AL; 20 ATTR) and 40 healthy subjects underwent 1.5 T cardiovascular magnetic resonance (CMR). They all underwent T1 and T2 mapping (modified Look-Locker inversion recovery), cine and late gadolinium enhancement (LGE) imaging. The Query Amyloid Late Enhancement (QALE) score, myocardial native T2, T1 and extra cellular volume fraction (ECV) were calculated for all patients.ResultsOf the 44 patients, 36 (82%) exhibited enhancement on LGE images. Mean QALE score of AL (7.9 ± 6) and ATTR (10.5 ± 5) patients were similar (p = 0.6). Myocardial native T2 was significantly (p < 0.0001) higher in AL (63.2 ± 4.7 ms) than in ATTR (56.2 ± 3.1 ms) patients, and both higher (p < 0.001) than healthy subjects (51.1 ± 3.1 ms). Myocardial native T2 was highly correlated with myocardial native T1 (Spearman’s rho = 0.79; p < 0.001) and exhibited higher diagnostic performance than T1 to separate AL and ATTR patients: the area under curve (AUC) of T2 was 0.94 (95% CI: 0.86–1, p < 0.001) and the AUC of T1 was 0.77 (95% CI: 0.62–0.91, p = 0.03). Myocardial native T2 did not impact overall survival in patients (HR 1.03 (0.94–1.12); p = 0.53) in contrast to ECV that was the best predictor of outcome (HR 1.66 per 0.1 increase in ECV (1.24–2.22); p = 0.0006).ConclusionsMyocardial native T2 significantly is increased in CA, especially in AL patients in comparison to ATTR patients. Myocardial native T2 does not impact survival in CA patients in contrast to ECV that was the best predictor of outcome.Trial registrationTrial Registration and unique number: CNIL cardio 1778041. Date of registration: 20 December 2012.

Cardiovascular magnetic resonance native T2 and T2* quantitative values for cardiomyopathies and heart transplantations: a systematic review and meta-analysis

“…In patchy diseases, such as amyloidosis and Anderson-Fabry disease, the recommendations state that the T 2 analysis should also include a single 3 chamber or 4 chamber view acquisition additionally to basal and mid-ventricular SAx slices [22]. Only one study actually followed these recommendations [158], while for the other cardiac patchy disease studies one or more recommended slices were not included [155][156][157]. In focal diseases, such as MI and myocarditis, the ROI differs between patients because the location of the abnormality is different, and therefore the guideline recommends multiple SAx acquisition to cover the whole myocardium and to place the ROI in visually abnormal myocardium [22].…”

“…The weighted mean T 2 values at 1.5 T in amyloidosis patients was 55.3 ± 4.2 ms and 50.2 ± 2.7 ms in controls [155,156] (Table 1, Fig. 5).…”

Reference ranges (“normal values”) for cardiovascular magnetic resonance (CMR) in adults and children: 2020 update

“… ECV extracellular volume fraction, FS field strength, T Tesla, n number of study subjects included in the weighted mean values, mean p pooled weighted mean, SD p pooled standard deviation, LL lower limit, UL upper limit, MOLLI modified look locker inversion-recovery, ShMOLLI short MOLLI, Siemens Siemens Medical Solutions, Erlangen, Germany, Philips Philips Healthcare, Best, The Netherlands a Pooled weighted values from references [ 118 , 121 , 129 , 132 , 136 , 141 , 142 ] b Pooled weighted values from references [ 116 , 122 – 124 , 128 , 130 , 131 , 135 , 142 , 147 , 148 ] c Pooled weighted values from references [ 127 , 139 , 145 , 146 ] d Pooled weighted values from references [ 125 , 133 , 136 , 144 , 149 ] e Pooled weighted values from references [ 137 , 138 ] f Pooled weighted values from references [ 126 , 134 , 140 , 145 ] g Pooled weighted values from references [ 121 , 129 , 142 ] h Pooled weighted values from references [ 124 , 128 , 130 , 131 , 142 ] i Values from reference [ 127 ] j Pooled weighted values from references [ 143 , 144 …”

Transthyretin cardiac amyloidosis: an update on diagnosis and treatment

“…Baseline characteristics and survival of patients with different types of cardiac amyloidosisThe numerical values of circulating NT-proBNP levels are described in mean ± standard deviation,,7,19,31,32,35 median (range, min-max), 18 or median (Q1-Q3 percentile),19,31,33,34 according to the description in the original literature. Yamamoto and T. Yokochi usefulness 45. Actually, LGE cannot detect diffuse myocardial fibrosis due to significant extracellular amyloid infiltration, and patients with renal dysfunction cannot utilize this technique.…”