背景:在轻链(AL)淀粉样变性中,功能状态和心力衰竭相关生活质量(HF-QOL)是否与心肌病严重程度相关,改善与治疗,并且与超过验证评分的主要不良心脏事件(MACE)相关,目前尚不为人所知.
目的:作者旨在:1)将功能状态和HF-QOL与心肌病严重程度相关联;2)分析其纵向变化;3)评估其与MACE的独立关联。
方法:这项研究包括106名患有AL淀粉样变性的参与者,81%患有AL心肌病。使用NYHA功能类别评估功能状态,Karnofsky量表,和6分钟步行距离(6MWD),和使用MLWHFQ(明尼苏达州心力衰竭生活问卷)的HF-QOL。通过心脏18F-florbetapir正电子发射断层扫描/计算机断层扫描评估心肌病的严重程度,心脏磁共振,超声心动图,和血清心脏生物标志物。MACE被定义为全因死亡,心力衰竭住院,或者心脏移植.
结果:NYHA功能类,Karnofsky量表,6MWD,在最近诊断为AL心肌病的参与者中,MLWHFQ明显受损(P<0.001),并与心肌病严重程度的所有标志物相关(P≤0.010)。NYHA功能类,6MWD,心肌病参与者在12个月时MLWHFQ改善(P≤0.013).功能状态和HF-QOL的所有测量均与MACE相关(P≤0.017),6MWD和MLWHFQ与Mayo分期无关(P≤0.006)。
结论:功能状态和HF-QOL与AL心肌病严重程度相关,在12个月内改善治疗,并与MACE有关,独立于Mayo阶段的6MWD和MLWHFQ。除了预后评分外,它们还可以进一步验证,并作为未来研究的替代结果。
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.
OBJECTIVE: 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.