Key result
Extracellular volume ≥ 44.0% (HR 7.249) and global late gadolinium enhancement (HR 4.804) were independently prognostic for all-cause mortality in patients with light chain amyloidosis.
Why the study?
Do CMR parameters including ECV and LGE predict all-cause mortality in patients with AL amyloidosis?
Cohort (n=102)
No
Do CMR parameters including ECV and LGE predict all-cause mortality in patients with AL amyloidosis?
Hazard Ratio: 7.249 (95% CI 1.751–13.179)
p-value: p=0.002
In patients with AL amyloidosis, CMR-derived extracellular volume (ECV) and global late gadolinium enhancement (LGE) are independent predictors of short-term mortality.
May support ECV- and LGE-based risk stratification in AL amyloidosis; hypothesis-generating and requires prospective validation before guiding therapy.
BACKGROUND: Cardiac 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. METHODS: Eighty-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. RESULTS: The 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). CONCLUSIONS: During 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.
No takes yet. Share an insight, caveat, or question.
Lü et al. (2018) conducted a cohort in Light chain (AL) amyloidosis (n=102). Extracellular volume (ECV) ≥ 44.0% vs. ECV < 44.0% was evaluated on All-cause mortality (HR 7.249, 95% CI 1.751-13.179, p=0.002). Extracellular volume ≥ 44.0% (HR 7.249) and global late gadolinium enhancement (HR 4.804) were independently prognostic for all-cause mortality in patients with light chain amyloidosis.