六年来转甲状腺素心脏淀粉样变性诊断轨迹的变化。

IF 1.4 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Heart and Vessels Pub Date : 2024-10-01 Epub Date: 2024-05-06 DOI:10.1007/s00380-024-02408-3
Anouk Achten, Vanessa P M van Empel, Jerremy Weerts, Sanne Mourmans, Fabienne Beckers-Wesche, Mireille Spanjers, Arno Gingele, Hans-Peter Brunner-La Rocca, Sandra Sanders-van Wijk, Christian Knackstedt
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引用次数: 0

摘要

近年来,随着诊断和治疗技术的发展,人们对转甲状腺素淀粉样变性心肌病(ATTR-CM)的认识不断提高。及时启动新型疾病修饰治疗可改善发病率和死亡率,这就强调了及时诊断的必要性。然而,ATTR-CM 的早期诊断仍具有挑战性。这是一项针对确诊为 ATTR-CM 患者的回顾性队列观察研究。2016年至2023年期间,共有87名患者被诊断为心脏淀粉样变性,其中65名(75%)为ATTR-CM患者,22名(25%)为轻链淀粉样变性患者。本研究纳入了 65 名 ATTR-CM 患者(平均年龄 77 ± 7 岁;86% 为男性),其中 59 人(91%)患有野生型 ATTR-CM (ATTRwt),6 人(9%)患有变异型 ATTR-CM。我们观察到,在 ATTRwt 的推动下,ATTR-CM 的诊断率从 3 例/年(2016-2020 年)激增至 16 例/年(2021-2023 年)。尽管如此,受转诊时间的影响,心衰症状出现与 ATTR-CM 诊断之间的时间间隔并无明显变化(2016-2020 年 27.3 个月 [18.6-62.4];2021-2023 年 30.0 个月 [8.6-57.2];p = 0.546)。ATTR-CM的红旗比诊断早几年:左心室肥大(79%,5.8年[3.3-7.0])和腕管综合征(49%,6.8年[2.3-12.1])。尽管存在典型的红旗信号,但症状到确诊的持续时间与转诊时间相似。提高对 ATTR-CM 红旗信号的识别率可缩短诊断时间并提高整体识别率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Changes in the diagnostic trajectory of transthyretin cardiac amyloidosis over six years.

Changes in the diagnostic trajectory of transthyretin cardiac amyloidosis over six years.

Awareness of transthyretin amyloid cardiomyopathy (ATTR-CM) has increased over the years due to diagnostic and therapeutic developments. Timely initiation of novel disease-modifying treatments improves both morbidity and mortality, which underlines the necessity for a prompt diagnosis. Nevertheless, early diagnosis of ATTR-CM remains challenging. This is a retrospective observational cohort study of patients diagnosed with ATTR-CM. Between 2016 and 2023, 87 patients were diagnosed with cardiac amyloidosis of which 65 (75%) patients with ATTR-CM and 22 (25%) patients with light chain amyloidosis. This study included 65 ATTR-CM patients (mean age 77 ± 7 years; 86% male) of whom 59 (91%) with wild-type ATTR-CM (ATTRwt) and six (9%) with variant ATTR-CM. We observed a surge in ATTR-CM diagnoses from 3 patients/year (2016-2020) to 16 patients/year (2021-2023), driven by ATTRwt. Nevertheless, the interval between the onset of heart failure symptoms and ATTR-CM diagnosis has not changed significantly (2016-2020 27.3 months [18.6-62.4]; 2021-2023 30.0 months [8.6-57.2]; p = 0.546), driven by time to referral. Red flags for ATTR-CM preceded diagnosis by several years: left ventricular hypertrophy (79%, 5.8 years [3.3-7.0]) and carpal tunnel syndrome (49%, 6.8 years [2.3-12.1]). Despite the presence of typical red flags, symptom-to-diagnosis duration has remained similar driven by time to referral. Improved recognition of red flags for ATTR-CM could reduce the time to diagnosis and improve overall recognition.

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来源期刊
Heart and Vessels
Heart and Vessels 医学-外周血管病
CiteScore
3.10
自引率
13.30%
发文量
211
审稿时长
2 months
期刊介绍: Heart and Vessels is an English-language journal that provides a forum of original ideas, excellent methods, and fascinating techniques on cardiovascular disease fields. All papers submitted for publication are evaluated only with regard to scientific quality and relevance to the heart and vessels. Contributions from those engaged in practical medicine, as well as from those involved in basic research, are welcomed.
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