Prognostic role of cardiopulmonary exercise testing in wild-type transthyretin amyloid cardiomyopathy patients treated with tafamidis.

IF 7.4 2区医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY

Amyloid-Journal of Protein Folding Disorders Pub Date : 2025-08-20 DOI:10.1080/13506129.2025.2547381

Tarun Dalia, Zafar Ali, Stefano H Byer, Rachel Holder, Shaden Daloub, Anureet Malhotra, Daniel Holtz, Tanner Robl, Zubair Shah

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引用次数: 0

Abstract

Objectives: Tafamidis has demonstrated survival benefits in transthyretin amyloid cardiomyopathy (ATTR-CM), yet variability in therapeutic response underscores the need for reliable tools to predict outcomes. This study evaluates the prognostic utility of cardiopulmonary exercise testing (CPET) in this population.

Methods: This retrospective study included tafamidis treated wild-type (ATTRwt) CM patients who completed CPET. Univariable and multivariable Cox regression models were used to evaluate predictors of mortality and composite secondary endpoint (mortality and heart failure admissions).

Results: Total of 105 ATTRwt-CM patients were enrolled with median age of 80 years. During median follow-up of 2.1 years, 33 (31%) died and 67 (63.8%) had composite outcome of mortality and heart failure hospitalisations. Multivariable analysis showed VE/VCO₂ slope (HR 1.61 per SD, 95% CI: 1.13-2.30, p = 0.01), peak circulatory power [CP] (HR 0.43 per SD, 95% CI 0.21-0.89, p = 0.02), Mayo stage 3 (HR 5.34, 95% CI: 1.07-26.7, p = 0.04) were independent predictors of mortality. The VE/VCO₂ slope (HR 1.57 per SD, 95% CI 1.2, 2.05, p= <0.01), creatinine (HR 1.37 per SD, 95% CI 1.07, 1.76, p = 0.01) and Mayo stage 3 (HR 2.49, 95% CI 1.10, 5.66, p = 0.03) were independent predictors of composite outcome.

Conclusions: CPET provides prognostic insights for ATTRwt-CM patients in addition to known prognostic laboratory factors.

查看原文本刊更多论文

心肺运动试验对野生型转甲状腺蛋白淀粉样心肌病患者接受他法非地治疗的预后作用。

目的：他法非底斯已经证明在转甲状腺素淀粉样心肌病（atr - cm）中具有生存益处，但治疗反应的可变性强调了对可靠工具预测预后的需求。本研究评估心肺运动试验（CPET）在该人群中的预后效用。方法：本回顾性研究包括他非他汀治疗的完成CPET的野生型（ATTRwt） CM患者。使用单变量和多变量Cox回归模型评估死亡率和复合次要终点（死亡率和心力衰竭入院率）的预测因子。结果：共纳入105例attrt - cm患者，中位年龄为80岁。在中位随访2.1年期间，33例（31%）死亡，67例（63.8%）有死亡和心力衰竭住院的复合结局。多变量分析显示，VE/VCO2斜率（HR 1.61 / SD, 95% CI: 1.13-2.30, p = 0.01）、峰值循环功率[CP] （HR 0.43 / SD, 95% CI: 0.21-0.89, p = 0.02）、Mayo期3 （HR 5.34, 95% CI: 1.07-26.7, p = 0.04）是死亡率的独立预测因子。VE/VCO2斜率（HR 1.57 / SD, 95% CI 1.2, 2.05, p= p= 0.01）和Mayo 3期（HR 2.49, 95% CI 1.10, 5.66, p= 0.03）是综合结局的独立预测因子。结论：除了已知的预后实验室因素外，CPET还为attrt - cm患者提供了预后见解。

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

Amyloid-Journal of Protein Folding Disorders 生物-生化与分子生物学

CiteScore

10.60

自引率

10.90%

发文量

审稿时长

6-12 weeks

期刊介绍： Amyloid: the Journal of Protein Folding Disorders is dedicated to the study of all aspects of the protein groups and associated disorders that are classified as the amyloidoses as well as other disorders associated with abnormal protein folding. The journals major focus points are: etiology, pathogenesis, histopathology, chemical structure, nature of fibrillogenesis; whilst also publishing papers on the basic and chemical genetic aspects of many of these disorders. Amyloid is recognised as one of the leading publications on amyloid protein classifications and the associated disorders, as well as clinical studies on all aspects of amyloid related neurodegenerative diseases and major clinical studies on inherited amyloidosis, especially those related to transthyretin. The Journal also publishes book reviews, meeting reports, editorials, thesis abstracts, review articles and symposia in the various areas listed above.