The prognostic value of T1 mapping and late gadolinium enhancement cardiovascular magnetic resonance imaging in patients with light chain amyloidosis

Abstract

BackgroundCardiac impairment is associated with high morbidity and mortality in immunoglobulin light chain (AL) type amyloidosis, for which early identification and risk stratification is vital. For myocardial tissue characterization, late gadolinium enhancement (LGE) is a classic and most commonly performed cardiovascular magnetic resonance (CMR) parameter. T1 mapping with native T1 and extracellular volume (ECV) are recently developed quantitative parameters. We aimed to investigate the prognostic value of native T1, ECV and LGE in patients with AL amyloidosis.MethodsEighty-two patients (55.5 ± 8.5 years; 52 M) and 20 healthy subjects (53.2 ± 11.7 years; 10 M) were prospectively recruited. All subjects underwent CMR with LGE imaging and T1 mapping using a Modified Look-Locker Inversion-recovery (MOLLI) sequence on a 3 T scanner. Native T1 and ECV were measured semi-automatically using a dedicated CMR software. The left ventricular (LV) LGE pattern was classified as none, patchy, and global groups. Global LGE was considered when there was diffuse, transmural LGE in more than half of the short axis images. Follow-up was performed for all-cause mortality using Cox proportional hazards regression analysis and Kaplan-Meier survival curves.ResultsThe patients demonstrated an increase in native T1 (1438 ± 120 ms vs. 1283 ± 46 ms, P = 0.001) and ECV (43.9 ± 10.9% vs. 27.0 ± 1.7%, P = 0.001) compared to healthy controls. Native T1, ECV and LGE showed significant correlation with Mayo Stage, and ECV and LGE showed significant correlation with echocardiographic E/E’ and LV ejection fraction. During the follow-up for a median time of 8 months, 21 deaths occurred. ECV ≥ 44.0% (hazard ratio [HR] 7.249, 95% confidence interval (CI) 1.751–13.179, P = 0.002) and global LGE (HR 4.804, 95% CI 1.971–12.926, P = 0.001) were independently prognostic for mortality over other clinical and imaging parameters. In subgroups with the same LGE pattern, ECV ≥ 44.0% remained prognostic (log rank P = 0.029). Median native T1 (1456 ms) was not prognostic for mortality (Tarone-Ware, P = 0.069).ConclusionsDuring a short-term follow-up, both ECV and LGE are independently prognostic for mortality in AL amyloidosis. In patients with a similar LGE pattern, ECV remained prognostic. Native T1 was not found to be a prognostic factor.

Outcome of Cardiac Light-Chain Amyloidosis in the Era of Novel Therapy ― A Single-Center Cohort Study of 227 Patients ―

“…Therefore, CMR evaluation is recommended as the first-line non-invasive diagnostic tool for CAL. 19 In our cohort, endomyocardial biopsy was performed in 26.0%, and CMR was done in 32.6% of all patients, which were high percentages, enhancing the credibility of the present study.…”

Characterizing cardiac involvement in amyloidosis using cardiovascular magnetic resonance diffusion tensor imaging

“…The correlation between MD and native T1 suggests that the pathomechanism of amyloid infiltration affects both parameters to a similar extent. While native T1 is a useful diagnostic marker for CA, recent studies have shown that it is inferior to ECV as an independent predictor of prognosis in AL-amyloidosis as well as ATTR-amyloidosis patients [30, 31]. However, the significant number of amyloidosis patients suffering from renal failure stresses the need for a gadolinium-free substitute for ECV with potential prognostic value.…”

Myocardial structural and functional changes in patients with liver cirrhosis awaiting liver transplantation: a comprehensive cardiovascular magnetic resonance and echocardiographic study

“…Recently, extracellular volume fraction (ECV) quantification by CMR T1 mapping technique has been introduced to evaluate DMF in vivo. Previous studies demonstrated that ECV reliably reflect the degree of DMF and, more importantly, is associated with prognosis in various cardiac diseases [12][13][14]. Thus, in this study, we hypothesized that myocardium in liver cirrhosis has structural alterations such as DMF, which could be effectively addressed by CMR.…”