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哥伦比亚转诊中心的心脏淀粉样变性患者的临床特征。

Clinical characterization of patients with cardiac amyloidosis in a referral center of Colombia.

机构信息

Department of Heart Failure, Fundación Cardioinfantil-La Cardio, Bogotá, Colombia.

Vascular and Endovascular Surgery Investigation Group, Fundación Cardioinfantil-La Cardio, Bogotá, Colombia.

出版信息

Arch Cardiol Mex. 2024 Aug 1;94(4):451-458. doi: 10.24875/ACM.23000236.

DOI:10.24875/ACM.23000236
PMID:39088727
Abstract

OBJECTIVE

The objective of the study is to describe the characteristics of our first cohort of amyloidosis in a Latin America cardiovascular reference center in Colombia.

METHODS

This is a historic cohort study and data were taken from the electronic records of the Fundación Cardioinfantil-Instituto de cardiología; adult patients with a diagnosis of cardiac amyloidosis were included and a descriptive analysis was presented.

RESULTS

A total of 31 patients with amyloidosis were included. 17 were Transthyretin Amyloidosis (ATTR) subtype and 14 were AL subtype. An overall mortality of 25% was found. The mean age at diagnosis was 74 years, male sex predominant. More frequent comorbidities were hypertension and atrial fibrillation. The most frequent clinical presentation was congestive heart failure (75%), with mildly reduced ejection fraction (41.94%), followed by reduced ejection fraction (32.26%), and preserved ejection fraction (25.81%). In the ATTR subtype, a reduced ejection fraction was found at 41.18% and a mildly reduced ejection fraction at 35.29%.

CONCLUSION

These results provide information on the most frequent type of amyloidosis and the late timing to diagnose in our historic cohort study, we present some of the baseline characteristics and most frequent approaches to diagnose Cardiac Amyloidosis that represents all challenges in clinical practice. Improvements are needed in the diagnosis and early treatment of these patients.

摘要

目的

本研究旨在描述哥伦比亚一家拉丁美洲心血管参考中心首个人类淀粉样变性队列的特征。

方法

这是一项历史性队列研究,数据取自 Fundación Cardioinfantil-Instituto de cardiología 的电子病历;纳入诊断为心脏淀粉样变性的成年患者,并进行描述性分析。

结果

共纳入 31 例淀粉样变性患者。17 例为转甲状腺素蛋白淀粉样变性(ATTR)亚型,14 例为 AL 亚型。总死亡率为 25%。诊断时的平均年龄为 74 岁,以男性为主。更常见的合并症是高血压和心房颤动。最常见的临床表现为充血性心力衰竭(75%),射血分数轻度降低(41.94%),其次是射血分数降低(32.26%)和射血分数保留(25.81%)。在 ATTR 亚型中,射血分数降低为 41.18%,射血分数轻度降低为 35.29%。

结论

这些结果提供了关于我们历史性队列研究中最常见的淀粉样变性类型和诊断时间较晚的信息,我们介绍了一些诊断心脏淀粉样变性的基线特征和最常见方法,这些方法代表了临床实践中的所有挑战。需要改进这些患者的诊断和早期治疗。

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本文引用的文献

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Prevalence and Risk Factors of Cardiac Amyloidosis in Heart Failure: A Systematic Review and Meta-Analysis.心力衰竭中心脏淀粉样变的患病率及危险因素:系统评价和荟萃分析。
Heart Lung Circ. 2022 Nov;31(11):1450-1462. doi: 10.1016/j.hlc.2022.08.002. Epub 2022 Sep 20.
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Diagnosis and treatment of cardiac amyloidosis. A position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases.心脏淀粉样变性的诊断与治疗。欧洲心脏病学会心肌和心包疾病工作组立场声明。
Eur J Heart Fail. 2021 Apr;23(4):512-526. doi: 10.1002/ejhf.2140. Epub 2021 Apr 7.
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Efficacy of Tafamidis in Patients With Hereditary and Wild-Type Transthyretin Amyloid Cardiomyopathy: Further Analyses From ATTR-ACT.
他法米替尼在遗传性和野生型转甲状腺素蛋白淀粉样心肌病患者中的疗效:来自ATTR-ACT 的进一步分析。
JACC Heart Fail. 2021 Feb;9(2):115-123. doi: 10.1016/j.jchf.2020.09.011. Epub 2020 Dec 9.
4
Systemic Amyloidosis due to Monoclonal Immunoglobulins: Cardiac Involvement.由于单克隆免疫球蛋白引起的系统性淀粉样变性:心脏受累。
Hematol Oncol Clin North Am. 2020 Dec;34(6):1055-1068. doi: 10.1016/j.hoc.2020.07.006. Epub 2020 Sep 14.
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Endomyocardial Biopsy Characterization of Heart Failure With Preserved Ejection Fraction and Prevalence of Cardiac Amyloidosis.心肌活检对射血分数保留心力衰竭的特征分析及心脏淀粉样变性的患病率。
JACC Heart Fail. 2020 Sep;8(9):712-724. doi: 10.1016/j.jchf.2020.04.007. Epub 2020 Jul 8.
6
Cardiac Amyloidosis: Evolving Diagnosis and Management: A Scientific Statement From the American Heart Association.心脏淀粉样变性:不断发展的诊断和治疗——美国心脏协会的科学声明。
Circulation. 2020 Jul 7;142(1):e7-e22. doi: 10.1161/CIR.0000000000000792. Epub 2020 Jun 1.
7
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Rev Esp Cardiol (Engl Ed). 2021 Feb;74(2):149-158. doi: 10.1016/j.rec.2019.12.020. Epub 2020 Apr 18.
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Multiparametric Echocardiography Scores for the Diagnosis of Cardiac Amyloidosis.多参数超声心动图评分对心脏淀粉样变性的诊断价值。
JACC Cardiovasc Imaging. 2020 Apr;13(4):909-920. doi: 10.1016/j.jcmg.2019.10.011. Epub 2019 Dec 18.
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