OBJECTIVE: This study aimed to assess the prognostic importance of the 6MWT in patients with ATTR-CA.
METHODS: A retrospective analysis of patients diagnosed with ATTR-CA at the National Amyloidosis Centre who underwent a baseline 6MWT between 2011 and 2023 identified 2,141 patients, of whom 1,118 had follow-up at 1 year.
RESULTS: The median baseline 6MWT distance was 347 m (Q1-Q3: 250-428 m) and analysis by quartiles demonstrated an increased death rate with each distance reduction (deaths per 100 person-years: 6.3 vs 9.2 vs 13.6 vs 19.0; log-rank P < 0.001). A 6MWT distance of <350 m was associated with a 2.2-fold higher risk of mortality (HR: 2.15; 95% CI: 1.85-2.50; P < 0.001), with a similar increased risk across National Amyloidosis Centre disease stages (P for interaction = 0.761) and genotypes (P for interaction = 0.172). An absolute (reduction of >35 m) and relative worsening (reduction of >5%) of 6MWT at 1 year was associated with an increased risk of mortality (HR: 1.80; 95% CI: 1.51-2.15; P < 0.001 and HR: 1.89; 95% CI: 1.59-2.24; P < 0.001, respectively), which was similar across the aforementioned subgroups. When combined with established measures of disease progression (N-terminal pro-B-type natriuretic peptide progression and outpatient diuretic intensification), each incremental increase in progression markers was associated with an increased death rate (deaths per 100 person-years: 7.6 vs 13.9 vs 22.4 vs 32.9; log-rank P < 0.001).
CONCLUSIONS: The baseline 6MWT distance can refine risk stratification beyond traditional prognosticators. A worsening 6MWT distance can stratify disease progression and, when combined with established markers, identifies patients at the highest risk of mortality.
目的:本研究旨在评估6MWT在ATTR-CA患者中的预后重要性。
方法:在2011年至2023年期间接受基线6MWT的国家淀粉样变性中心诊断为ATTR-CA的患者的回顾性分析确定了2,141例患者,其中1,118人在1年内进行了随访。
结果:基线6MWT距离中位数为347m(Q1-Q3:250-428m),四分位数分析显示死亡率随着距离的减少而增加(每100人年死亡:6.3vs9.2vs13.6vs19.0;对数秩P<0.001)。6MWT距离<350m与死亡风险高2.2倍相关(HR:2.15;95%CI:1.85-2.50;P<0.001),在国家淀粉样变性中心疾病分期(相互作用的P=0.761)和基因型(相互作用的P=0.172)中,风险增加相似。1年时6MWT的绝对(减少>35m)和相对恶化(减少>5%)与死亡风险增加有关(HR:1.80;95%CI:1.51-2.15;P<0.001和HR:1.89;95%CI:1.59-2.24;P<0.001),在上述亚组中相似。当与既定的疾病进展措施(N末端B型利钠肽前体进展和门诊利尿剂强化)相结合时,进展标志物的每一次递增增加与死亡率增加相关(每100人年死亡:7.6vs13.9vs22.4vs32.9;log-rankP<0.001).
结论:基线6MWT距离可以改善风险分层,超越传统的预测指标。恶化的6MWT距离可以对疾病进展进行分层,当与已建立的标记结合时,确定死亡风险最高的患者。