Related Experiment Video
Updated: May 12, 2026
Noninvasive Assessment of Cardiac Abnormalities in Experimental Autoimmune Myocarditis by Magnetic Resonance Microscopy Imaging in the Mouse
Published on: June 20, 2014
Assessing microvascular dysfunction and predicting long-term prognosis in patients with cardiac amyloidosis by
Leting Tang1, Wenjin Zhao1, Kang Li1
1Department of Radiology, The Second Xiangya Hospital, Central South University, Changsha, China.
Insights
Cardiac light-chain amyloidosis prognosis is improved by assessing extracellular volume fraction (ECV) and myocardial perfusion reserve (MPR) using cardiovascular magnetic resonance (CMR). Higher ECV and lower MPR indicate a worse prognosis in AL-CA patients.
Area of Science:
- Cardiology
- Medical Imaging
- Oncology
Background:
- Cardiac involvement is a key prognostic factor in light chain amyloidosis (AL).
- Amyloid deposition increases extracellular volume fraction (ECV) and impairs microvascular function.
- Quantitative stress perfusion is crucial for assessing microvascular dysfunction in AL-cardiac amyloidosis (AL-CA).
Purpose of the Study:
- To investigate microvascular dysfunction extent in AL-CA.
- To determine the incremental prognostic value of quantitative stress perfusion parameters.
- To correlate cardiovascular magnetic resonance (CMR) findings with patient outcomes.
Main Methods:
- 126 AL amyloidosis patients underwent CMR with LGE, T1 mapping, and stress perfusion.
- Extracellular volume fraction (ECV) and myocardial perfusion reserve (MPR) were semi-automatically measured.
- Prognostic value of clinical, laboratory, and CMR parameters was assessed using Cox regression.
Main Results:
- Higher MPR and lower ECV were observed in survivors.
- MPR was significantly lower in patients with transmural LGE.
- ECV, MPR, and LGE were independent predictors of mortality.
- MPR >1.5 and ECV ≤53.6% correlated with improved survival.
Conclusions:
- ECV and MPR provide additive prognostic value in advanced AL-CA.
- CMR phenotypes with higher ECV and lower MPR are associated with worse outcomes.
- MPR enhances survival assessment, particularly when ECV and Mayo staging are similar.
Background:
Cardiac involvement in light chain amyloidosis (AL) is the main determinant of prognosis. Amyloid can be deposited in the extracellular space and cause an increase in extracellular volume fraction (ECV). At the same time, amyloid can also be deposited in the wall of small vessels and cause microvascular dysfunction. This study sought to investigate the extent of microvascular dysfunction and its incremental prognostic value in cardiac light-chain amyloidosis (AL-CA) by quantitative stress perfusion.
Methods:
A total of 126 AL amyloidosis patients (61.13 ± 8.46 years, 81 male) confirmed by pathology were prospectively recruited. All subjects underwent cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE), T1 mapping, and stress perfusion on a 3T scanner. ECV and myocardial perfusion reserve (MPR) were measured semi-automatically using a dedicated CMR software. Clinical, laboratory, and CMR parameters were analyzed for their prognostic value in the assessment of AL-CA patients. Mortality-associated markers were analyzed by univariate and multivariable Cox regression.
Results:
The median follow-up time was 37 (33.6-40.4) months, and 62 patients died. The ECV of survivors was significantly reduced, but the stress myocardial blood flow and MPR were higher (P < 0.001). The MPR of the transmural LGE group was significantly lower than that of the no LGE and subendocardial LGE groups (P < 0.001). In multivariable analysis, ECV, MPR, and LGE were independently predictive. MPR of >1.5 and ECV of ≤53.6% were associated with improved overall survival, both of which provided predictive incremental value in patients with advanced disease. With equal Mayo staging and degree of ECV, MPR improves assessment of patient survival.
Conclusion:
ECV and MPR showed additive incremental values and further discriminated prognosis of patients in advanced stages. CMR phenotypes with higher ECV and lower MPR had a worse prognosis.
More Related Videos
08:13In Vivo Quantitative Assessment of Myocardial Structure, Function, Perfusion and Viability Using Cardiac Micro-computed Tomography
Published on: February 16, 2016
05:07Author Spotlight: Improved Localization and Monitoring of Coronary Flow Reserve Using Modified PLAX View in Mice
Published on: August 25, 2023
