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射血分数保留的心力衰竭患者的心脏淀粉样变性筛查与管理:一项国际调查。

Cardiac Amyloidosis Screening and Management in Patients With Heart Failure With Preserved Ejection Fraction: An International Survey.

作者信息

Shchendrygina Anastasia, Mewton Nathan, Niederseer David, Kida Keisuke, Guidetti Federica, Duval Antoine Jobbe, Milinkovic Ivan, Oerlemans Marish I F J, Zaleska-Kociecka Marta, de Gracia Sydney Goldfeder, Palacio Maria Isabel, Giverts Ilya, Komarova Irina, Rustamova Yasmin, Bahouth Fadel, Mežnar Anja Zupan, Mapelli Massimo, Suvorov Alexandr, Dyachuk Irina, Shutov Michail, Sitnikova Violetta, Garnier-Crussard Antoine, Barasa Anders, Loncar Goran, Tokmakova Mariya, Skouri Hadi, Ruschitzka Frank, Saldarriaga Clara

机构信息

Department of Hospital Therapy 2, I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation.

Heart Failure Department and Clinical Investigation Center, Hôpital Cardiovasculaire Louis Pradel Hospices Civils de Lyon Heart Failure Department Clinical Investigation Center Inserm 1407 CarMeN Inserm 1060, University Claude Bernard Lyon, Bron, France.

出版信息

Am J Cardiol. 2025 Feb 1;236:42-48. doi: 10.1016/j.amjcard.2024.10.009. Epub 2024 Oct 29.

Abstract

Cardiac amyloidosis (CA) is still an underdiagnosed cause of heart failure (HF) and early disease recognition and timely disease-modifying therapy (DMT) administration translate to better outcomes. We aimed to assess CA screening and management approaches for patients with HF preserved ejection fraction (HFpEF) among physicians worldwide. An independent academic web-based survey was distributed worldwide between May 2023 and July 2023. Overall, 1,460 physicians (61% were men, median age was 42 [34 to 49] years) from 95 countries completed the survey. A total of 2/3 of respondents had experience diagnosing CA and reported having 10% of patients with CA in patients with HFpEF. Systematic screening for CA of all patients with HFpEF was performed by 10% of responders, whereas 24% did not consider the screening. Most responders (39%) used left ventricular hypertrophy as a screening criterion. Serum protein electrophoresis with immunofixation of free light chain and urine protein electrophoresis or cardiac magnetic resonance were selected by half of the responders as a first-line diagnostic tool. The combination of serum protein electrophoresis with immunofixation free light chain, urine protein electrophoresis, and bone scintigraphy was considered by 32% of the participants. CA DMT was available for 48% of the physicians. About 82% of responders would administrate HF to patients with HFpEF with CA, with the most preferable drugs being diuretics, sodium-glucose cotransporter-2 inhibitors, and renin-angiotensin-aldosterone system inhibitors. In conclusion, the results reveal the uncertainties among physicians worldwide regarding the need for CA screening of patients with HFpEF. CA remains a disease with very heterogeneous management, particularly, in the screening and diagnostic workup. The HF community should aim to educate on CA and improve access to DMT.

摘要

心脏淀粉样变性(CA)仍是心力衰竭(HF)的一个诊断不足的病因,早期疾病识别和及时给予疾病修饰治疗(DMT)可带来更好的预后。我们旨在评估全球范围内医生对射血分数保留的心力衰竭(HFpEF)患者进行CA筛查和管理的方法。2023年5月至2023年7月在全球范围内开展了一项基于网络的独立学术调查。总体而言,来自95个国家的1460名医生(61%为男性,中位年龄为42[34至49]岁)完成了调查。共有2/3的受访者有诊断CA的经验,并报告在HFpEF患者中有10%的患者患有CA。10%的受访者对所有HFpEF患者进行了CA的系统筛查,而24%的受访者未考虑进行筛查。大多数受访者(39%)将左心室肥厚作为筛查标准。一半的受访者选择血清蛋白电泳联合游离轻链免疫固定及尿蛋白电泳或心脏磁共振作为一线诊断工具。32%的参与者考虑血清蛋白电泳联合游离轻链免疫固定、尿蛋白电泳和骨闪烁显像的联合检查。48%的医生可获得CA DMT。约82%的受访者会对患有CA的HFpEF患者进行HF治疗,最常用的药物是利尿剂、钠-葡萄糖协同转运蛋白-2抑制剂和肾素-血管紧张素-醛固酮系统抑制剂。总之,结果揭示了全球范围内医生在HFpEF患者是否需要进行CA筛查方面存在不确定性。CA仍然是一种管理非常异质性的疾病,特别是在筛查和诊断检查方面。HF领域应致力于开展关于CA的教育并改善DMT的可及性。

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