心脏淀粉样变性患者猝死的发生率和预测因素。

IF 5.2 2区医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY

Amyloid-Journal of Protein Folding Disorders Pub Date : 2024-10-24 DOI:10.1080/13506129.2024.2414295

Fernando de Frutos, Giulia Saturi, Esther Gonzalez-Lopez, Maurizio Sguazzotti, Fernando Dominguez, Alberto Ponziani, Eva Cabrera-Romero, Angelo Giuseppe Caponetti, Sara Lozano, Paolo Massa, Belen Peiro-Aventin, Antonella Accietto, Nerea Mora-Ayestarán, Alessandro Giovannetti, Victor Castro-Urda, Christian Gagliardi, Marta Cobo-Marcos, Rafael Rios-Tamayo, Elena Biagini, Manuel Gomez-Bueno, Nazzareno Galiè, Javier Segovia-Cubero, Simone Longhi, Pablo Garcia-Pavia

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引用次数: 0

摘要

导言：尽管猝死（SD）是公认的心脏淀粉样变性并发症，但有关其发生率、机制和预测因素的数据却很少。本研究旨在描述猝死的发生率并分析可能的风险因素：方法：对在欧洲两个中心接受评估的ATTR或AL心脏淀粉样变性患者进行连续鉴定。SD定义为临床病情稳定患者的意外死亡。在单变量分析中，对风险因素进行了Cox比例危险回归评估。然后通过年龄调整后的多变量分析评估具有统计学意义的风险因素：分析包括784例患者，其中569例为ATTR淀粉样变性患者（平均年龄为74.1 ± 12.1岁），215例为AL淀粉样变性患者（平均年龄为64.5 ± 10.8岁）。中位随访 1.9 年后，ATTR 患者 2 年后的 SD 率为 1.8%。经年龄调整分析后，曾植入起搏器（PPM）与风险增加有关（HR 4.97；95%CI：1.39-17.7；P = 0.01）。中位随访 1.2 年后，AL 淀粉样变性患者 2 年后的 SD 率为 8.0%。经年龄调整后的多变量分析显示，倍他受体阻滞剂和NYHA III-IV分别与风险增加有关（HR 7.06 95%CI (2.31-21.5) p = 0.001）和（HR 4.56 95%CI (1.51-13.8) p = 0.007）：与ATTR心脏淀粉样变性相比，SD在AL中更为常见。结论：SD在AL比在ATTR心脏淀粉样变性中更常见，SD与这两种疾病的不同风险因素有关。

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Incidence and predictors of sudden death in patients with cardiac amyloidosis.

Introduction: Although sudden death (SD) is a recognized complication of cardiac amyloidosis, there is scarce data about its incidence, mechanisms, and predictors. The aim of this study was to describe incidence of SD and to analyze possible risk factors.

Methods: Consecutive patients with ATTR or AL cardiac amyloidosis evaluated at two European centers were identified. SD was defined as unexpected death in clinically stable patients. Cox proportional hazard regression was performed to assess risk factors in univariate analysis. Those statistically significant were then assessed through age-adjusted multivariate analysis.

Results: Analysis included 784 patients, 569 with ATTR amyloidosis (mean age 74.1 ± 12.1 years) and 215 with AL amyloidosis (mean age 64.5 ± 10.8 years). After a median follow-up of 1.9 years, SD rate at 2 years was 1.8% in ATTR. Previous pacemaker implantation (PPM) was associated with increased risk after age-adjusted analysis (HR 4.97; 95%CI: 1.39-17.7; p = 0.01). SD rate in AL amyloidosis patients at 2 years was 8.0% after a median follow-up of 1.2 years. Betablockers and NYHA III-IV were independently associated with an increased risk after age-adjusted multivariate analysis (HR 7.06 95%CI (2.31-21.5) p = 0.001) and (HR 4.56 95%CI (1.51-13.8) p = 0.007) respectively.

Conclusions: SD is more frequent in AL than in ATTR cardiac amyloidosis. SD is associated with different risk factors in both entities.

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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.