Natural course and effect of tafamidis treatment in real-life octogenarians with wild-type transthyretin cardiac amyloidosis: an international multi-centre analysis

IF 8.9 2区 环境科学与生态学 Q1 ENGINEERING, ENVIRONMENTAL Environmental Science & Technology Letters Environ. Pub Date : 2024-10-28 DOI:10.1093/eurheartj/ehae666.2069
P Debonnaire, K Dujardin, N Verheyen, A C Pouleur, S Droogmans, M Claeys, M El Haddad, E Christiaen, D Zach, L Buytaert, A Jacobs, I Buysschaert, S Trenson, R Van Hoeyweghen, R Tavernier
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Abstract

Background and aims In real-life, wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) is increasingly diagnosed in elderly patients above 80 years old. Nevertheless, specific data on natural course and outcome under tafamidis treatment in octogenarians are particularly scarce. We aimed to study tafamidis impact on mortality and its determinants in real-life ATTRwt-CM octogenarians. Methods International multi-centric cohort study of 710 consecutive ATTRwt-CM patients, stratified between octogenarians and non-octogenarians at diagnose and tafamidis naive or treatment. Mean follow-up was 2.2±1.8 years to all-cause mortality endpoint. Results The cohort consisted of 58.3% octogenarians (median 84 years, 74.2% male). Before tafamidis availability, natural course in octogenarians (148/257) versus non-octogenarians (109/257) was poor with 41% three-year and 70% five-year mortality versus 19% and 42%, respectively (p<0.001). Since tafamidis availability, 70.1% (253/361) octogenarians were initiated on tafamidis, less than 83.7% (231/276) non-octogenarians (p<0.001). Tafamidis discontinuation was similar (octogenarians 11.4%, non-octogenarians 7.9%, p=0.260). Overall tafamidis treated octogenarians had better survival than untreated octogenarians (p<0.001) and untreated non-octogenarians (p=0.026), with 23% three-year and 51% five-year mortality. Tafamidis treatment related to lower mortality in octogenarians (HR 0.37, 95%CI 0.20-0.67, p=0.001), after correcting for age, NAC stage and NYHA class, the other independent predictors. Propensity-score matching for those variables in 218 subjects confirmed tafamidis mortality benefit in octogenarians (p<0.001). Octogenarians had poorer survival under tafamidis, when initiated at ≥90 years old (p<0.001), NAC stage ≥3 (p=0.001) or NYHA class ≥3 (p=0.055). Conclusions Tafamidis treatment in real-life octogenarians independently improves survival, with better effect of early initiation at lower disease stage and age.Structured graphical abstractDistributions within age category
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野生型转甲状腺素心脏淀粉样变性八旬老人的自然病程和他法米迪治疗效果:国际多中心分析
背景和目的 在现实生活中,越来越多 80 岁以上的老年患者被诊断为野生型转甲状腺素淀粉样变性心肌病(ATTRwt-CM)。然而,有关八旬老人接受他法米迪治疗后的自然病程和预后的具体数据却特别少。我们的目的是研究塔法米地对真实的 ATTRwt-CM 八旬老人死亡率的影响及其决定因素。方法 对 710 名连续的 ATTRwt-CM 患者进行国际多中心队列研究,在诊断时对八旬老人和非八旬老人进行分层,对他法米迪进行天真或治疗。至全因死亡终点的平均随访时间为 2.2±1.8 年。结果 组群中有58.3%的八旬老人(中位数84岁,74.2%为男性)。在他法米迪上市之前,八旬老人(148/257)与非八旬老人(109/257)的自然病程较差,三年死亡率为41%,五年死亡率为70%,而非八旬老人分别为19%和42%(p<0.001)。自从他法米迪上市以来,70.1%(253/361)的八旬老人开始服用他法米迪,低于 83.7%(231/276)的非八旬老人(p<0.001)。停用他法米迪的情况相似(八旬老人 11.4%,非八旬老人 7.9%,p=0.260)。总体而言,接受过他法米迪治疗的八旬老人的生存率高于未接受过治疗的八旬老人(p<0.001)和未接受过治疗的非八旬老人(p=0.026),三年死亡率为23%,五年死亡率为51%。在校正年龄、NAC分期和NYHA分级等其他独立预测因素后,他法米迪治疗可降低八旬老人的死亡率(HR 0.37,95%CI 0.20-0.67,p=0.001)。在 218 名受试者中,针对这些变量的倾向分数匹配证实了他法米迪对八旬老人死亡率的益处(p<0.001)。八旬老人在≥90岁(p<0.001)、NAC分期≥3期(p=0.001)或NYHA分级≥3级(p=0.055)时开始接受他法米迪治疗,生存率较低。结论 在现实生活中,八旬老人接受塔法米地治疗可独立提高生存率,在疾病分期和年龄较低时尽早开始治疗效果更好。
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来源期刊
Environmental Science & Technology Letters Environ.
Environmental Science & Technology Letters Environ. ENGINEERING, ENVIRONMENTALENVIRONMENTAL SC-ENVIRONMENTAL SCIENCES
CiteScore
17.90
自引率
3.70%
发文量
163
期刊介绍: Environmental Science & Technology Letters serves as an international forum for brief communications on experimental or theoretical results of exceptional timeliness in all aspects of environmental science, both pure and applied. Published as soon as accepted, these communications are summarized in monthly issues. Additionally, the journal features short reviews on emerging topics in environmental science and technology.
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