Functional Status and Quality of Life in Light-Chain Amyloidosis: Advanced Imaging, Longitudinal Changes, and Outcomes

Olivier F Clerc; Shilpa Vijayakumar; Sarah A M Cuddy; Giada Bianchi; Jocelyn Canseco Neri; Alexandra Taylor; Dominik C Benz; Yesh Datar; Marie Foley Kijewski; Andrew J Yee; Frederick L Ruberg; Ronglih Liao; Rodney H Falk; Vaishali Sanchorawala; Sharmila Dorbala

doi:10.1016/j.jchf.2024.07.007

Functional Status and Quality of Life in Light-Chain Amyloidosis: Advanced Imaging, Longitudinal Changes, and Outcomes

JACC Heart Fail. 2024 Aug 20:S2213-1779(24)00562-6. doi: 10.1016/j.jchf.2024.07.007. Online ahead of print.

Authors

Affiliations

¹ Cardiac 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.
² Cardiac 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; CV imaging program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
³ Cardiac Amyloidosis Program, Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA; Division of Hematology, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
⁴ Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
⁵ Division of Hematology and Oncology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA.
⁶ Section of Cardiovascular Medicine, Department of Medicine, Boston Medical Center, Boston University Chobanian and Avedisian School of Medicine, Boston, Massachusetts, USA; Amyloidosis Center, Boston University Chobanian and Avedisian School of Medicine, Boston, Massachusetts, USA.
⁷ Amyloidosis Program, Stanford University, Stanford, California, USA.
⁸ Cardiac Amyloidosis Program, Cardiovascular Division, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
⁹ Cardiac 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; CV imaging program, Cardiovascular Division and Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA. Electronic address: [email protected].

PMID: 39243245
DOI: 10.1016/j.jchf.2024.07.007

Abstract

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.

Keywords: Light-chain (AL) amyloidosis; cardiomyopathy; functional status; heart failure–related quality of life; longitudinal changes; major adverse cardiac outcomes.