Prognostic value of CMR-derived extracellular volume in AL amyloidosis: a multicenter study.

IF 5.2 2区医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-12-01 Epub Date: 2024-09-25 DOI:10.1080/13506129.2024.2406842

Martin Nicol, Cassiel Kitzinger, Mathilde Baudet, Alyssa Faradji, Théo Pezel, David Lavergne, Arnaud Jaccard, Giuseppe Vergaro, Alberto Aimo, Michele Emdin, Stephanie Harel, Bruno Royer, Alexis Talbot, Valérie Bousson, Laurent Macron, Bertrand Arnulf, Damien Logeart

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Abstract

Background: This study aimed to assess the prognostic value of cardiac magnetic resonance (CMR) variables and compare them with biological and echocardiographic markers in patients with AL cardiac amyloidosis (CA).

Methods: We conducted a prospective study across three tertiary centres, where patients underwent clinical examination, blood tests, echocardiography, and CMR. The primary endpoint was all-cause mortality.

Results: A total of 176 patients with AL CA were included, with a median age of 68 years (IQR 58-75). According to the 2004 Mayo Clinic staging, 121 patients (69%) were in stage 3. During a median follow-up of 22 months (IQR 8-48), 45 patients died, and 55 were hospitalized for heart failure. Patients who died had higher NT-proBNP and troponin levels, and lower LVEF, cardiac output, and longitudinal strain. Among CMR variables, extracellular volume (ECV) was most strongly associated with all-cause mortality. In multivariate Cox models, including Mayo Clinic staging, ECV ≥ 0.45 was independently associated with mortality (HR 2.36, CI 95% 1.47-5.60) and also with heart failure hospitalizations (HR 4.10, 95%CI 2.15-8.8).

Conclusion: ECV is a powerful predictor of outcomes in AL CA, providing additional prognostic value on top of Mayo Clinic staging.

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AL淀粉样变性中CMR衍生细胞外体积的预后价值：一项多中心研究。

研究背景本研究旨在评估心脏磁共振（CMR）变量的预后价值，并将其与 AL 型心脏淀粉样变性（CA）患者的生物标记物和超声心动图标记物进行比较：我们在三个三级医疗中心开展了一项前瞻性研究，对患者进行了临床检查、血液化验、超声心动图检查和心脏磁共振检查。主要终点是全因死亡率：共纳入176名AL CA患者，中位年龄为68岁（IQR 58-75）。根据梅奥诊所 2004 年的分期，121 名患者（69%）处于第 3 期。在中位 22 个月（IQR 8-48）的随访期间，45 名患者死亡，55 名患者因心力衰竭住院。死亡患者的 NT-proBNP 和肌钙蛋白水平较高，LVEF、心输出量和纵向应变较低。在 CMR 变量中，细胞外容积（ECV）与全因死亡率的关系最为密切。在包括梅奥诊所分期在内的多变量 Cox 模型中，ECV ≥ 0.45 与死亡率（HR 2.36，CI 95% 1.47-5.60）和心力衰竭住院率（HR 4.10，95%CI 2.15-8.8）独立相关：ECV是预测AL CA预后的有力指标，在梅奥临床分期的基础上提供了额外的预后价值。

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.