Fumagalli Carlo, Zampieri Mattia, Presta Roberto, Pini Greta, Vetere Giulia, Argirò Alessia, Longhi Simone, Tini Giacomo, Musumeci Beatrice, Limongelli Giuseppe, Palmiero Giuseppe, Verrillo Federica, Beltrami Matteo, Bo Mario, De Ferrari Gaetano, Tofani Lorenzo, Sardu Celestino, Marfella Raffaele, Marchionni Niccolò, Perfetto Federico, Olivotto Iacopo, Fumagalli Stefano, Maurer Mathew S, Fontana Marianna, Ungar Andrea, Cappelli Francesco
Department of Advanced Medical and Surgical Sciences, University of Campania, "Luigi Vanvitelli," Naples, Italy (C.F., C.S., R.M.).
Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy. (C.F., M.Z., G. Pini, A.A., M. Beltrami, F.C.).
Circ Heart Fail. 2025 Jul 2:e012777. doi: 10.1161/CIRCHEARTFAILURE.125.012777.
The prevalence of transthyretin cardiac amyloidosis among older adults (often octogenarians) is increasing. We aimed to determine whether age and geriatric syndromes bear any impact on the management and outcomes in transthyretin cardiac amyloidosis and assess the risk of ageism.
In a prospective, multicenter cohort study, 256 patients diagnosed with transthyretin cardiac amyloidosis from March 2021 to March 2024 underwent comprehensive geriatric assessment (CGA). The study evaluated the prevalence and clinical associations of CGAs across different disease stages (National Amyloidosis Centre stage). Key CGA domains included disability, malnutrition, depression, frailty, Short Physical Performance Battery, and cumulative deficits (sum of the single CGA items). Associations of these measures with disease-modifying therapy and overall mortality were analyzed.
Median age was 82 years (men: n=223 [87%]; variant: n=19 [7.4%]); 129 (50.3%) patients received disease modifiers. Those ≥85 years had significantly lower odds of receiving disease-modifying therapy even after adjusting for disability, frailty, and cumulative deficits. Over 1.9 (interquartile range, 1.0-2.3) years, 45 (17.6%) patients died. After adjustment for National Amyloidosis Centre stage, diuretics and disease modifiers, CGA domains of disability, malnutrition, Short Physical Performance Battery, frailty, and number of deficits, but not age, were significantly associated with mortality. Assessment of CGA domains improved National Amyloidosis Centre prognostic accuracy.
In a national prospective cohort of patients with transthyretin cardiac amyloidosis, older age was associated with lower prescription of disease modifiers, even among individuals with a low burden of geriatric syndromes. However, when adjusted for geriatric domains, age was not associated with survival, indicating potential ageism. Because some geriatric syndromes may be modifiable, a CGA could enhance risk stratification, reduce age-related bias, and improve outcomes.
老年人（通常为八旬老人）中甲状腺转运蛋白型心脏淀粉样变的患病率正在上升。我们旨在确定年龄和老年综合征是否会对甲状腺转运蛋白型心脏淀粉样变的管理及预后产生影响，并评估年龄歧视的风险。
在一项前瞻性多中心队列研究中，256例于2021年3月至2024年3月被诊断为甲状腺转运蛋白型心脏淀粉样变的患者接受了全面老年评估（CGA）。该研究评估了不同疾病阶段（国家淀粉样变中心分期）CGA的患病率及临床相关性。关键的CGA领域包括残疾、营养不良、抑郁、衰弱、简短体能测试以及累积缺陷（单个CGA项目的总和）。分析了这些指标与疾病修饰治疗及全因死亡率的关联。
中位年龄为82岁（男性：n = 223 [87%]；变异型：n = 19 [7.4%]）；129例（50.3%）患者接受了疾病修饰治疗。即便在对残疾、衰弱和累积缺陷进行校正后，年龄≥85岁的患者接受疾病修饰治疗的几率仍显著较低。在1.9（四分位间距，1.0 - 2.3）年的时间里，45例（17.6%）患者死亡。在对国家淀粉样变中心分期、利尿剂和疾病修饰治疗进行校正后，残疾、营养不良、简短体能测试、衰弱和缺陷数量等CGA领域，但不包括年龄，与死亡率显著相关。对CGA领域的评估提高了国家淀粉样变中心的预后准确性。
在一项全国性的甲状腺转运蛋白型心脏淀粉样变患者前瞻性队列研究中，即便在老年综合征负担较低的个体中，年龄较大也与疾病修饰治疗的处方率较低相关。然而，在对老年领域进行校正后，年龄与生存率无关，这表明存在潜在的年龄歧视。由于一些老年综合征可能是可改变的，CGA可加强风险分层，减少与年龄相关的偏倚，并改善预后。
