希腊转甲状腺素淀粉样变性心肌病:来自国家转诊中心的临床见解。

IF 2.7 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Hellenic Journal of Cardiology Pub Date : 2024-09-01 DOI:10.1016/j.hjc.2023.09.019
{"title":"希腊转甲状腺素淀粉样变性心肌病:来自国家转诊中心的临床见解。","authors":"","doi":"10.1016/j.hjc.2023.09.019","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><p>Clinical characteristics and outcomes of patients with transthyretin amyloidosis cardiomyopathy (ATTR-CM) vary by region, necessitating the acquisition of country-specific evidence for proper management.</p></div><div><h3>Methods</h3><p>This is an observational study including sequential patients presenting in the Amyloidosis Reference Center of Greece, from 01/2014 to 12/2022. ATTR-CM was diagnosed by positive scintigraphy and exclusion of light-chain amyloidosis or positive biopsy typing. Genetic testing was performed in all cases.</p></div><div><h3>Results</h3><p>In total, 109 ATTR-CM patients were included (median age, 81 years) of which 15 carried <em>TTR</em> mutations (27% Val30Met). Most patients (82%) presented with heart failure and 59% with atrial fibrillation, while 10% had aortic stenosis. Importantly, 78 (71.6%) had clinically significant extracardiac manifestations (45% musculoskeletal disorder, 40% peripheral neuropathy, and 33% gastrointestinal symptoms). Sixty-five (60%) received disease-specific treatment with tafamidis. Estimated median survival was 48 months; advanced NYHA class, National Amyloidosis Center stage, eGFR&lt;45 ml/kg/1.73 m<sup>2</sup>, NT-pro-BNP&gt;5000 pg/mL were associated with worse survival, while tafamidis treatment was associated with improved survival in patients with IVS≥ 12 mm.</p></div><div><h3>Discussion</h3><p>These are the first data describing the characteristics, management, and outcomes of patients with ATTR-CM in Greece, which could influence local guidelines.</p></div>","PeriodicalId":55062,"journal":{"name":"Hellenic Journal of Cardiology","volume":"79 ","pages":"Pages 25-34"},"PeriodicalIF":2.7000,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1109966623001902/pdfft?md5=3a3da55ff2cbab019ccb1dba4a84b69c&pid=1-s2.0-S1109966623001902-main.pdf","citationCount":"0","resultStr":"{\"title\":\"Transthyretin amyloidosis cardiomyopathy in Greece: Clinical insights from the National Referral Center\",\"authors\":\"\",\"doi\":\"10.1016/j.hjc.2023.09.019\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Background</h3><p>Clinical characteristics and outcomes of patients with transthyretin amyloidosis cardiomyopathy (ATTR-CM) vary by region, necessitating the acquisition of country-specific evidence for proper management.</p></div><div><h3>Methods</h3><p>This is an observational study including sequential patients presenting in the Amyloidosis Reference Center of Greece, from 01/2014 to 12/2022. ATTR-CM was diagnosed by positive scintigraphy and exclusion of light-chain amyloidosis or positive biopsy typing. Genetic testing was performed in all cases.</p></div><div><h3>Results</h3><p>In total, 109 ATTR-CM patients were included (median age, 81 years) of which 15 carried <em>TTR</em> mutations (27% Val30Met). Most patients (82%) presented with heart failure and 59% with atrial fibrillation, while 10% had aortic stenosis. Importantly, 78 (71.6%) had clinically significant extracardiac manifestations (45% musculoskeletal disorder, 40% peripheral neuropathy, and 33% gastrointestinal symptoms). Sixty-five (60%) received disease-specific treatment with tafamidis. Estimated median survival was 48 months; advanced NYHA class, National Amyloidosis Center stage, eGFR&lt;45 ml/kg/1.73 m<sup>2</sup>, NT-pro-BNP&gt;5000 pg/mL were associated with worse survival, while tafamidis treatment was associated with improved survival in patients with IVS≥ 12 mm.</p></div><div><h3>Discussion</h3><p>These are the first data describing the characteristics, management, and outcomes of patients with ATTR-CM in Greece, which could influence local guidelines.</p></div>\",\"PeriodicalId\":55062,\"journal\":{\"name\":\"Hellenic Journal of Cardiology\",\"volume\":\"79 \",\"pages\":\"Pages 25-34\"},\"PeriodicalIF\":2.7000,\"publicationDate\":\"2024-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.sciencedirect.com/science/article/pii/S1109966623001902/pdfft?md5=3a3da55ff2cbab019ccb1dba4a84b69c&pid=1-s2.0-S1109966623001902-main.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Hellenic Journal of Cardiology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S1109966623001902\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Hellenic Journal of Cardiology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1109966623001902","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0

摘要

背景:转甲状腺素淀粉样变性心肌病(ATTR-CM)患者的临床特征和结果因地区而异,因此需要获得国家特定的证据进行适当的治疗。方法:这是一项观察性研究,包括2014年1月至2022年12月在希腊淀粉样变性参考中心就诊的连续患者。ATTR-CM是通过阳性闪烁扫描和排除轻链淀粉样变性或阳性活检分型诊断的。所有病例均进行了基因检测。结果:包括109名ATTR-CM患者(中位年龄81岁),其中15人携带TTR突变(27%的Val30Met)。大多数患者(82%)出现心力衰竭,59%出现心房颤动,10%出现主动脉狭窄。重要的是,78例(71.6%)有临床意义的心外表现(45%为肌肉骨骼疾病,40%为周围神经病变,33%为胃肠道症状)。六十五名(60%)患者接受了针对疾病的tafamidis治疗。估计中位生存期为48个月;晚期NYHA分级、国家淀粉样变性中心分期、eGFR2、NT-pro-BBNP>5000pg/mL与IVS≥12mm患者的生存率提高相关。讨论:这些是描述希腊ATTR-CM患者特征、管理和结果的第一批数据,可能会影响当地指南。简称:希腊的转甲状腺素心肌病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

摘要图片

摘要图片

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Transthyretin amyloidosis cardiomyopathy in Greece: Clinical insights from the National Referral Center

Background

Clinical characteristics and outcomes of patients with transthyretin amyloidosis cardiomyopathy (ATTR-CM) vary by region, necessitating the acquisition of country-specific evidence for proper management.

Methods

This is an observational study including sequential patients presenting in the Amyloidosis Reference Center of Greece, from 01/2014 to 12/2022. ATTR-CM was diagnosed by positive scintigraphy and exclusion of light-chain amyloidosis or positive biopsy typing. Genetic testing was performed in all cases.

Results

In total, 109 ATTR-CM patients were included (median age, 81 years) of which 15 carried TTR mutations (27% Val30Met). Most patients (82%) presented with heart failure and 59% with atrial fibrillation, while 10% had aortic stenosis. Importantly, 78 (71.6%) had clinically significant extracardiac manifestations (45% musculoskeletal disorder, 40% peripheral neuropathy, and 33% gastrointestinal symptoms). Sixty-five (60%) received disease-specific treatment with tafamidis. Estimated median survival was 48 months; advanced NYHA class, National Amyloidosis Center stage, eGFR<45 ml/kg/1.73 m2, NT-pro-BNP>5000 pg/mL were associated with worse survival, while tafamidis treatment was associated with improved survival in patients with IVS≥ 12 mm.

Discussion

These are the first data describing the characteristics, management, and outcomes of patients with ATTR-CM in Greece, which could influence local guidelines.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Hellenic Journal of Cardiology
Hellenic Journal of Cardiology CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
4.90
自引率
7.30%
发文量
86
审稿时长
56 days
期刊介绍: The Hellenic Journal of Cardiology (International Edition, ISSN 1109-9666) is the official journal of the Hellenic Society of Cardiology and aims to publish high-quality articles on all aspects of cardiovascular medicine. A primary goal is to publish in each issue a number of original articles related to clinical and basic research. Many of these will be accompanied by invited editorial comments. Hot topics, such as molecular cardiology, and innovative cardiac imaging and electrophysiological mapping techniques, will appear frequently in the journal in the form of invited expert articles or special reports. The Editorial Committee also attaches great importance to subjects related to continuing medical education, the implementation of guidelines and cost effectiveness in cardiology.
期刊最新文献
Association between preoperative uric acid concentration and the occurrence of atrial fibrillation following cardiac surgery: An observational prospective study. Impact of the COVID-19 pandemic on CTO PCI: analysis from the PROGRESS-CTO registry. Sports cardiology: not a sprint but a marathon-and, above all, a team sport. In Memoriam: George L. Bakris (1952-2024). Verification of persistent pulmonary vein isolation with electroanatomical mapping 3 months after ablation using a novel PFA platform.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1