Functional Status and Quality of Life in Light-Chain Amyloidosis: Advanced Imaging, Longitudinal Changes, and
Olivier F Clerc1, Shilpa Vijayakumar2, Sarah A M Cuddy1
1Cardiac Amyloidosis Program, Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA; Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
Insights
Functional status and heart failure quality of life in light-chain amyloidosis correlate with cardiomyopathy severity and improve with therapy. These measures independently predict major adverse cardiac events, offering potential as surrogate outcomes.
Area of Science:
- Cardiology
- Hematology
- Medical Imaging
Background:
- Light-chain (AL) amyloidosis can cause significant cardiac dysfunction.
- The relationship between functional status, quality of life, and cardiac outcomes in AL amyloidosis is not fully understood.
Purpose of the Study:
- To correlate functional status and heart failure-related quality of life (HF-QOL) with AL amyloidosis cardiomyopathy severity.
- To analyze longitudinal changes in functional status and HF-QOL.
- To assess the independent association of functional status and HF-QOL with major adverse cardiac events (MACE).
Main Methods:
- 106 participants with AL amyloidosis, 81% with AL cardiomyopathy, were assessed.
- Functional status evaluated via NYHA class, Karnofsky scale, and 6-minute walk distance (6MWD).
- HF-QOL assessed using the Minnesota Living with Heart Failure Questionnaire (MLWHFQ).
- Cardiomyopathy severity measured by advanced imaging and biomarkers.
- MACE defined as death, heart failure hospitalization, or cardiac transplantation.
Main Results:
- Impaired functional status and HF-QOL at diagnosis, correlating with cardiomyopathy severity (P < 0.001).
- Significant improvements in NYHA class, 6MWD, and MLWHFQ at 12 months post-therapy (P ≤ 0.013).
- All functional status and HF-QOL measures independently predicted MACE (P ≤ 0.017), including 6MWD and MLWHFQ independent of Mayo stage (P ≤ 0.006).
Conclusions:
- Functional status and HF-QOL reflect AL cardiomyopathy severity and improve with treatment.
- These measures are independent predictors of MACE, beyond established prognostic scores.
- Functional status and HF-QOL show promise as surrogate outcomes in clinical trials for AL amyloidosis.
Background:
In light-chain (AL) amyloidosis, whether functional status and heart failure-related quality of life (HF-QOL) correlate with cardiomyopathy severity, improve with therapy, and are associated with major adverse cardiac events (MACE) beyond validated scores is not well-known.
Objectives:
The authors aimed to: 1) correlate functional status and HF-QOL with cardiomyopathy severity; 2) analyze their longitudinal changes; and 3) assess their independent associations with MACE.
Methods:
This study included 106 participants with AL amyloidosis, with 81% having AL cardiomyopathy. Functional status was evaluated using the NYHA functional class, the Karnofsky scale, and the 6-minute walk distance (6MWD), and HF-QOL using the MLWHFQ (Minnesota Living with Heart Failure Questionnaire). Cardiomyopathy severity was assessed by cardiac 18F-florbetapir positron emission tomography/computed tomography, cardiac magnetic resonance, echocardiography, and serum cardiac biomarkers. MACE were defined as all-cause death, heart failure hospitalization, or cardiac transplantation.
Results:
NYHA functional class, Karnofsky scale, 6MWD, and MLWHFQ were impaired substantially in participants with recently diagnosed AL cardiomyopathy (P < 0.001), and correlated with all markers of cardiomyopathy severity (P ≤ 0.010). NYHA functional class, 6MWD, and MLWHFQ improved at 12 months in participants with cardiomyopathy (P ≤ 0.013). All measures of functional status and HF-QOL were associated with MACE (P ≤ 0.017), independent of Mayo stage for 6MWD and MLWHFQ (P ≤ 0.006).
Conclusions:
Functional status and HF-QOL were associated with AL cardiomyopathy severity, improved on therapy within 12 months, and were associated with MACE, independently of Mayo stage for 6MWD and MLWHFQ. They may be validated further in addition to prognostic scores and as surrogate outcomes for future studies.
