超声心动图AMYLI评分在全身性轻链淀粉样变性：临床相关性和风险分层。

IF 3.2 2区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

International journal of cardiology Pub Date : 2025-09-13 DOI:10.1016/j.ijcard.2025.133889

Shichu Liang , Keying Bi , Ke Wan , Zhiyue Liu , Yaqiong Zhou , Saeed Abdulmalek Ahmed Ghaithan , Jiayu Sun , Yuchi Han , He Huang , Yucheng Chen

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引用次数: 0

摘要

背景：AMYLoidosis Index （AMYLI）被广泛用于筛选心脏淀粉样变性（CA），但其与淀粉样蛋白负荷和预后价值的关系尚不清楚。方法：这项单中心、前瞻性、观察性研究于2011年11月至2023年9月进行。连续的新诊断的活检证实的AL淀粉样变患者接受超声心动图和心脏磁共振（CMR）检查。通过经胸超声心动图计算AMYLI评分为相对壁厚（RWT）和E/ E '的乘积。结果：共纳入AL淀粉样变患者263例（平均年龄58.84 ± 9.63 岁，男性158例[50.08% %]）。AMYLI评分与cmr衍生的淀粉样蛋白负担（细胞外体积）呈中等正相关（r = 0.57,P ）结论：AMYLI评分是CA患者淀粉样蛋白负担的可靠指标，也是独立的预后因素，为CA患者的风险分层提供了一种替代的、方便的基于超声心动图的成像标记。试验注册：中国临床试验注册中心，ChiCTR1900024764，由四川大学华西医院伦理委员会注册。

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Echocardiographic AMYLI score in systemic light-chain amyloidosis: Clinical relevance and risk stratification

Background

The AMYLoidosis Index (AMYLI) is widely used for screening cardiac amyloidosis, but its relationship with amyloid burden and prognostic value is unclear.

Methods

This single-center, prospective, observational study was conducted from November 2011 to September 2023. Consecutive patients with newly-diagnosed biopsy-proven light-chain (AL) amyloidosis patients who underwent both echocardiography and cardiac magnetic resonance (CMR) were enrolled. The AMYLI score was calculated as the product of relative wall thickness (RWT) and E/e' via transthoracic echocardiography.

Results

A total of 263 AL amyloidosis patients (mean age 58.84 ± 9.63 years, 158 [60.1 %] males) were included in the study. The AMYLI score showed a moderately positive correlation with CMR-derived amyloid burden (extracellular volume; r = 0.57, P < 0.001). During a median follow-up of 42 (interquartile range: 35–49) months, 3 patients were lost to follow-up, and 171 patients died. An AMYLI score ≥ 7.85 strongly predicted all-cause mortality (HR 2.80, 95 % CI: 1.80–4.35, P < 0.001) and remained an independent prognostic factor after adjusting for clinical, biochemical, echocardiographic, CMR imaging-related, and therapeutic factors. AMYLI score ≥ 7.85 added incremental prognostic value to conventional clinical and imaging risk factors.

Conclusions

The AMYLI score is a reliable indicator of amyloid burden in patients with AL amyloidosis and independent prognostic factor, offering an alternative and convenient echocardiography-based imaging marker for the risk stratification of AL amyloidosis patients.

Trial registration

Chinese Clinical Trial Registry, ChiCTR1900024764 by the ethics committee of West China Hospital, Sichuan University.

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来源期刊

International journal of cardiology 医学-心血管系统

CiteScore

6.80

自引率

5.70%

发文量

758

审稿时长

44 days

期刊介绍： The International Journal of Cardiology is devoted to cardiology in the broadest sense. Both basic research and clinical papers can be submitted. The journal serves the interest of both practicing clinicians and researchers. In addition to original papers, we are launching a range of new manuscript types, including Consensus and Position Papers, Systematic Reviews, Meta-analyses, and Short communications. Case reports are no longer acceptable. Controversial techniques, issues on health policy and social medicine are discussed and serve as useful tools for encouraging debate.