Volpentesta Eugenia, Kharoubi Mounira, Donadio Cristiano, Rebiai Kahina, Fanen Pascale, Funalot Benoit, Gendre Thierry, Audard Vincent, Canoui-Poitrine Florence, Itti Emmanuel, Teiger Emmanuel, Planté-Bordeneuve Violaine, Oghina Silvia, Tixier Denis, Mallet Sophie, Broussier Amaury, Damy Thibaud, Zaroui Amira
Departement of Geriatrics, AP-HP (Assistance Publique-Hôpitaux de Paris), Henri-Mondor/Albert-Chenevier Hospital, Créteil, France.
Departement of Geriatrics, AP-HP (Assistance Publique-Hôpitaux de Paris), Charles Foix Hospital, Ivry-sur-seine, France.
ESC Heart Fail. 2024 Dec;11(6):3814-3832. doi: 10.1002/ehf2.14793. Epub 2024 Jul 17.
Transthyretin cardiac amyloidosis (ATTR-CM) may be an underestimated cause of heart failure among geriatric patients and represent a unique phenotype and prognostic profile.
This retrospective, observational, cohort study characterizes cardiac and extracardiac disorders at diagnosis and assesses prognosis among ATTR-CM patients based on age (geriatric vs. non-geriatric) and amyloidosis subtype (wild type, ATTRwt and hereditary, ATTRv). In total, 943 patients with ATTR-CM were included, of which 306 had ATTRv and 637 had ATTRwt. Among these, 331 (35.1%) were non-geriatric (<75 years), and 612 (64.9%) were geriatric (≥75 years). The population exhibited conduction abnormalities, atrial fibrillation and ischaemic heart disease that progressively deteriorated with age. Among ATTRwt patients, peripheral neuropathy, neurovegetative symptoms, and hearing loss were present across all age groups, but reports of carpal tunnel symptoms or surgery decreased with age. Conversely, among ATTRv patients, reports of extracardiac symptoms increased with age and Val122ILe mutation was highly prevalent among geriatric patients. The 3-year survival was higher among non-geriatric ATTR-CM patients (76%) than geriatric patients (55%) and predictors of 3-year mortality differed. Notably, predictors identified among geriatric patients were alkaline phosphatase (ALP) (HR = 1.004, 95% CI: [0.001-1.100)], troponin T hs (HR = 1.005, 95% CI: [1.001-1.120)] and tricuspid insufficiency (HR = 1.194, 95% CI: [1.02-1.230)]. Whereas, among non-geriatric patients, NT-proBNP (HR = 1.002, 95% CI: [1.02-1.04], global longitudinal strain (HR = 0.95, 95% CI: [0.922-0.989], and glomerular filtration rate (HR = 0.984, 95% CI: [0.968-1.00) were identified. We propose a 3-stage prognostic staging system combining troponin T hs (≥44 ng/L) and ALP levels (≥119 UI/L). In the geriatric population, this model discriminated survival more precisely than the National Amyloidosis Centre staging, particularly for classifying between stage 1 (82%), stage 2 (50%) and stage 3 (32%) for ATTRv and ATTRwt.
These diagnostic and prognostic indicators, along with ATTR subtype, highlight the distinct characteristics of this important, geriatric ATTR-CM patient group. Recognizing these mortality markers can be valuable for geriatricians to improve the prognostic quality management of geriatric patients with ATTR-CM.
转甲状腺素蛋白心脏淀粉样变性（ATTR-CM）可能是老年患者心力衰竭的一个被低估的病因，并且代表一种独特的表型和预后特征。
这项回顾性、观察性队列研究对诊断时的心脏和心脏外疾病进行了特征描述，并根据年龄（老年与非老年）和淀粉样变性亚型（野生型，ATTRwt和遗传性，ATTRv）评估了ATTR-CM患者的预后。总共纳入了943例ATTR-CM患者，其中306例为ATTRv，637例为ATTRwt。在这些患者中，331例（35.1%）为非老年患者（<75岁），612例（64.9%）为老年患者（≥75岁）。该人群表现出传导异常、心房颤动和缺血性心脏病，且这些疾病随年龄增长而逐渐恶化。在ATTRwt患者中，所有年龄组均存在周围神经病变、神经植物症状和听力损失，但腕管综合征症状或手术的报告随年龄增长而减少。相反，在ATTRv患者中，心脏外症状的报告随年龄增长而增加，且Val122ILe突变在老年患者中高度流行。非老年ATTR-CM患者的3年生存率（76%）高于老年患者（55%），且3年死亡率的预测因素有所不同。值得注意的是，在老年患者中确定的预测因素为碱性磷酸酶（ALP）（HR = 1.004，95% CI：[0.001 - 1.100]）、高敏肌钙蛋白T（HR = 1.005，95% CI：[1.001 - 1.120]）和三尖瓣关闭不全（HR = 1.194，95% CI：[1.02 - 1.230]）。而在非老年患者中，确定的因素为N末端B型利钠肽原（NT-proBNP）（HR = 1.002，95% CI：[1.02 - 1.04]）、整体纵向应变（HR = 0.95，95% CI：[0.922 - 0.989]）和肾小球滤过率（HR = 0.984，95% CI：[0.968 - 1.00]）。我们提出了一个结合高敏肌钙蛋白T（≥44 ng/L）和ALP水平（≥119 UI/L）的3阶段预后分期系统。在老年人群中，该模型比国家淀粉样变性中心分期更精确地区分生存率，特别是对于ATTRv和ATTRwt的1期（82%）、2期（50%）和3期（32%）进行分类。
这些诊断和预后指标，连同ATTR亚型，突出了这一重要的老年ATTR-CM患者群体的独特特征。认识这些死亡标志物对于老年病医生改善老年ATTR-CM患者的预后质量管理可能具有重要价值。
