Key result
Combined high ECV and impaired 3D GLS identify light-chain cardiac amyloidosis patients with poorest survival.
Why the study?
Risk stratification in light-chain cardiac amyloidosis (AL-CA) remains challenging. Although three-dimensional speckle-tracking echocardiography (3D-STE) and cardiac magnetic resonance extracellular volume (ECV) are emerging prognostic tools, their precise threshold values and combined prognostic utility in AL-CA were not fully defined.
Does combined assessment of 3D global longitudinal strain and cardiac magnetic resonance extracellular volume improve prediction of all-cause mortality in patients with light-chain cardiac amyloidosis?
Cohort (n=248)
Does combined assessment of 3D global longitudinal strain and cardiac magnetic resonance extracellular volume improve prediction of all-cause mortality in patients with light-chain cardiac amyloidosis?
Combining cardiac MRI extracellular volume (≥53%) and 3D echocardiographic global longitudinal strain (> -11.9%) provides synergistic prognostic value to identify light-chain cardiac amyloidosis patients at highest risk of short-term mortality.
May support combined ECV and 3D GLS for mortality risk stratification in light-chain amyloidosis; leaves open prospective validation before practice change.
Background Risk stratification in light-chain cardiac amyloidosis (AL-CA) remains challenging. Three-dimensional speckle-tracking echocardiography (3D-STE) and cardiac magnetic resonance extracellular volume (ECV) are emerging prognostic tools, but their precise threshold values and combined prognostic utility in AL-CA are not fully defined. Purpose This study aimed to identify optimal threshold values for 3D-STE parameters and ECV, and to evaluate their combined prognostic utility in predicting all-cause mortality in patients with AL-CA. Methods This prospective study included patients with biopsy-confirmed AL-CA (2022–2025). All participants underwent comprehensive echocardiography prior to standard chemotherapy initiation. 3D-STE parameters —global longitudinal strain (GLS), global circumferential strain (GCS), left ventricular ejection fraction (LVEF)—were quantified using dedicated software (4D LV-Function 3, Ultrasound Workspace). The primary study endpoint was all-cause mortality. Results A total of 248 patients (mean age: 63.09 ± 9.82; 73.39% male) were included, with 124 reaching the primary endpoint. Ninety patients underwent cardiovascular magnetic resonance and 3D-STE within seven days prior to initiation of chemotherapy. Median follow-up was 211 days. Restricted cubic spline analysis revealed significant inflection points where mortality risk increased: 3D GLS > -11.9% (P for nonlinearity = 0.223), 3D GCS > -19.1% (P = 0.593), 3D LVEF < 45% (P = 0.153), and ECV > 53% (P for nonlinearity = 0.880). Kaplan-Meier analysis confirmed that patients with impaired parameters (below inflection points for strain/EF, above for ECV) had significantly worse survival (all Log-rank P < 0.05). Furthermore, combined stratification using both ECV (53%) and 3D GLS (-11.9%) identified a very high-risk subgroup (ECV ≥53% and 3D GLS > -11.9%) with the poorest prognosis (Log-rank, P = 0.041). Conclusion In AL-CA, specific thresholds of 3D-STE parameters (GLS= -11.9%, GCS= -19.1%, LVEF=45%) and ECV (53%) are critically associated with survival. The combination of elevated ECV and impaired 3D GLS effectively identifies patients at the highest risk, suggesting a synergistic prognostic value of myocardial tissue characterization and functional assessment.
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Meng et al. (2026) conducted a cohort in Light-chain cardiac amyloidosis (AL-CA) (n=248). 3D speckle-tracking echocardiography (3D GLS > -11.9%, 3D GCS > -19.1%, 3D LVEF < 45%) and cardiac MRI extracellular volume (ECV ≥ 53%) vs. Preserved 3D-STE parameters (3D GLS ≤ -11.9%, 3D GCS ≤ -19.1%, 3D LVEF ≥ 45%) and ECV < 53% was evaluated on All-cause mortality (p=0.041). Combined ECV ≥53% and 3D GLS >-11.9% identified the highest-risk light-chain cardiac amyloidosis subgroup with the poorest survival for all-cause mortality (Log-rank P=0.041).
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