Clinical Phenotype and Prognostic Significance of Frailty in Transthyretin Cardiac Amyloidosis

IF 12 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

Jacc: Cardiooncology Pub Date : 2025-04-01 DOI:10.1016/j.jaccao.2025.01.018

Carlo Fumagalli MD , Adam Ioannou MBBS, BSc , Francesco Cappelli MD , Mathew S. Maurer MD , Yousuf Razvi MBChB , Aldostefano Porcari MD , Mattia Zampieri MD , Federico Perfetto MD, PhD , Muhammad U. Rauf MBBS , Ana Martinez-Naharro MD, PhD , Lucia Venneri MD, PhD , Aviva Petrie MSc , Carol Whelan MD , Ashutosh Wechalekar MD , Helen Lachmann MD , Philip N. Hawkins MD, PhD , Iacopo Olivotto MD , Raffaele Marfella MD, PhD , Andrea Ungar MD, PhD , Niccolò Marchionni MD , Marianna Fontana MD, PhD

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Abstract

Background

The prevalence and clinical impact of frailty in transthyretin cardiac amyloidosis (ATTR-CA) remains poorly characterized.

Objectives

This study aimed to evaluate the prevalence, clinical determinants, and prognostic significance of frailty in a large cohort of patients with ATTR-CA.

Methods

Frailty was assessed in 880 patients with ATTR-CA (median age 80 years [Q1-Q3: 75-84 years], 719 [81.7%] male) using the Clinical Frailty Scale (CFS). Frailty was analyzed as a continuous variable and categorized as CFS 1 to 3, CFS 4 or 5, CFS 6 or 7, and CFS 8 or 9.

Results

Frailty was observed in 502 (57.1%) patients (CFS 4 or 5: 364 [41.4%]; CFS 6 or 7: 129 [14.7%]; CFS 8 or 9: 9 [1.0%]). Independent predictors of worsening frailty included older age, female sex, non-p.(V142I) hereditary ATTR-CA variants, and National Amyloidosis Centre stage 3 disease. Mortality rates increased incrementally with frailty severity (deaths per 100 person-years: 2.9 vs 11.0 vs 21.1 vs 40.9; log-rank P < 0.001). Frailty was independently associated with higher mortality risk across all age groups, genotypes, and disease stages.

Conclusions

Frailty is common in ATTR-CA and is independently linked to increased mortality risk. Incorporating frailty assessment alongside traditional markers enhances prognostication across genotypes and disease severities, particularly for short-term risk estimation.

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经甲状腺素型心脏淀粉样变性患者虚弱的临床表型及预后意义

转甲状腺素型心脏淀粉样变性（atr - ca）的患病率和虚弱的临床影响仍然缺乏明确的特征。目的：本研究旨在评估atr - ca患者的患病率、临床决定因素和预后意义。方法采用临床衰弱量表（CFS）对880例atr - ca患者（中位年龄80岁[Q1-Q3: 75-84岁]，719例（81.7%）男性）进行衰弱评估。虚弱作为一个连续变量进行分析，并分类为CFS 1至3、CFS 4或5、CFS 6或7、CFS 8或9。结果502例（57.1%）患者出现虚弱(CFS 4、5:364例（41.4%）；CFS 6或7:129 [14.7%]；CFS 8或9:9[1.0%])。衰弱恶化的独立预测因素包括年龄较大、女性、非p （V142I）遗传性atr - ca变异和国家淀粉样变中心3期疾病。死亡率随着衰弱严重程度的增加而增加(每100人年死亡人数：2.9 vs 11.0 vs 21.1 vs 40.9；log-rank P <；0.001)。在所有年龄组、基因型和疾病分期中，虚弱与较高的死亡风险独立相关。结论：虚弱在atr - ca中很常见，并与死亡风险增加独立相关。将衰弱评估与传统标记结合起来，可以增强跨基因型和疾病严重程度的预测，特别是短期风险评估。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Jacc: Cardiooncology Multiple-

CiteScore

12.50

自引率

6.30%

发文量

106

期刊介绍： JACC: CardioOncology is a specialized journal that belongs to the esteemed Journal of the American College of Cardiology (JACC) family. Its purpose is to enhance cardiovascular care for cancer patients by publishing high-quality, innovative scientific research and sharing evidence-based knowledge. The journal aims to revolutionize the field of cardio-oncology and actively involve and educate professionals in both cardiovascular and oncology fields. It covers a wide range of topics including pre-clinical, translational, and clinical research, as well as best practices in cardio-oncology. Key areas of focus include understanding disease mechanisms, utilizing in vitro and in vivo models, exploring novel and traditional therapeutics (across Phase I-IV trials), studying epidemiology, employing precision medicine, and investigating primary and secondary prevention. Amyloidosis, cardiovascular risk factors, heart failure, and vascular disease are some examples of the disease states that are of particular interest to the journal. However, it welcomes research on other relevant conditions as well.