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.

中文翻译：

---

轻链淀粉样变性的功能状态和生活质量：高级成像、纵向变化和结果。


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、随治疗改善以及与超出验证分数的主要不良心脏事件 (MACE) 相关，目前尚不清楚。不知名。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) 评估它们与 MACE 的独立关联。方法 本研究纳入了 106 名患有 AL 淀粉样变性的参与者，其中 81% 患有 AL 心肌病。使用 NYHA 功能等级、卡诺夫斯基量表和 6 分钟步行距离 (6MWD) 评估功能状态，并使用 MLWHFQ（明尼苏达心力衰竭生活问卷）评估 HF-QOL。通过心脏 18F-氟倍他吡正电子发射断层扫描/计算机断层扫描、心脏磁共振、超声心动图和血清心脏生物标志物评估心肌病的严重程度。 MACE 被定义为全因死亡、心力衰竭住院或心脏移植。结果 近期诊断出 AL 心肌病的参与者的 NYHA 功能分级、卡诺夫斯基量表、6MWD 和 MLWHFQ 显着受损 (P < 0.001)，并且与心肌病严重程度的所有标志物相关 (P ≤ 0.010)。心肌病参与者的 NYHA 功能分级、6MWD 和 MLWHFQ 在 12 个月时有所改善（P ≤ 0.013）。所有功能状态和 HF-QOL 测量均与 MACE 相关（P ≤ 0.017），与 6MWD 和 MLWHFQ 的 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，治疗后 12 个月内有所改善，并且与 MACE 相关，与 6MWD 和 MLWHFQ 的 Mayo 分期无关。 除了预后评分之外，它们还可以得到进一步验证，并作为未来研究的替代结果。