转甲状腺素淀粉样心肌病左心室流出道梗阻:诊断和治疗的挑战和酒精室间隔消融的作用1例报告。

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
European Heart Journal: Case Reports Pub Date : 2025-05-12 eCollection Date: 2025-05-01 DOI:10.1093/ehjcr/ytaf233
Ali Hussein Jaber Mejren, Sie Kronborg Fensman, Steen Hvitfeldt Poulsen
{"title":"转甲状腺素淀粉样心肌病左心室流出道梗阻:诊断和治疗的挑战和酒精室间隔消融的作用1例报告。","authors":"Ali Hussein Jaber Mejren, Sie Kronborg Fensman, Steen Hvitfeldt Poulsen","doi":"10.1093/ehjcr/ytaf233","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Transthyretin amyloid cardiomyopathy (ATTR-CM) is a restrictive cardiomyopathy caused by amyloid deposition in the myocardium. Its phenotypical overlap with hypertrophic cardiomyopathy, particularly in cases involving left ventricular outflow tract obstruction (LVOTO), challenges accurate diagnosis. Medical management of LVOTO in ATTR-CM is challenged by the opposing effects of beta-blockers and diuretics.</p><p><strong>Case summary: </strong>A 79-year-old male with left ventricular hypertrophy and LVOTO presented with worsening dyspnoea. Full diagnostic work-up confirmed wild-type ATTR-CM. A conservative medical approach with diuretics and beta-blockers proved challenging. Alcohol septal ablation was successfully performed without major complication, resolving the LVOTO and improving symptoms.</p><p><strong>Discussion: </strong>The diagnosis and management of ATTR-CM with LVOTO are complex. A thorough diagnostic approach is needed to avoid mismanagement. Diuretics and beta-blockers must be carefully balanced to achieve optimal clinical results. Alcohol septal ablation may be considered in patients with persistent symptoms and high LVOT gradients despite optimal medical therapy.</p>","PeriodicalId":11910,"journal":{"name":"European Heart Journal: Case Reports","volume":"9 5","pages":"ytaf233"},"PeriodicalIF":0.8000,"publicationDate":"2025-05-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12100115/pdf/","citationCount":"0","resultStr":"{\"title\":\"Left ventricular outflow tract obstruction in transthyretin amyloid cardiomyopathy: a case report on diagnostic and treatment challenges and role of alcohol septal ablation.\",\"authors\":\"Ali Hussein Jaber Mejren, Sie Kronborg Fensman, Steen Hvitfeldt Poulsen\",\"doi\":\"10.1093/ehjcr/ytaf233\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Transthyretin amyloid cardiomyopathy (ATTR-CM) is a restrictive cardiomyopathy caused by amyloid deposition in the myocardium. Its phenotypical overlap with hypertrophic cardiomyopathy, particularly in cases involving left ventricular outflow tract obstruction (LVOTO), challenges accurate diagnosis. Medical management of LVOTO in ATTR-CM is challenged by the opposing effects of beta-blockers and diuretics.</p><p><strong>Case summary: </strong>A 79-year-old male with left ventricular hypertrophy and LVOTO presented with worsening dyspnoea. Full diagnostic work-up confirmed wild-type ATTR-CM. A conservative medical approach with diuretics and beta-blockers proved challenging. Alcohol septal ablation was successfully performed without major complication, resolving the LVOTO and improving symptoms.</p><p><strong>Discussion: </strong>The diagnosis and management of ATTR-CM with LVOTO are complex. A thorough diagnostic approach is needed to avoid mismanagement. Diuretics and beta-blockers must be carefully balanced to achieve optimal clinical results. Alcohol septal ablation may be considered in patients with persistent symptoms and high LVOT gradients despite optimal medical therapy.</p>\",\"PeriodicalId\":11910,\"journal\":{\"name\":\"European Heart Journal: Case Reports\",\"volume\":\"9 5\",\"pages\":\"ytaf233\"},\"PeriodicalIF\":0.8000,\"publicationDate\":\"2025-05-12\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12100115/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"European Heart Journal: Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1093/ehjcr/ytaf233\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/5/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Heart Journal: Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1093/ehjcr/ytaf233","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/5/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0

摘要

背景:转甲状腺素淀粉样心肌病(atr - cm)是一种由淀粉样蛋白沉积在心肌引起的限制性心肌病。其表型与肥厚性心肌病重叠,特别是在涉及左心室流出道梗阻(LVOTO)的病例中,挑战准确诊断。atr - cm中LVOTO的医疗管理受到β受体阻滞剂和利尿剂的相反作用的挑战。病例总结:一名79岁男性左室肥厚伴左室呼吸困难加重。全面诊断证实为野生型atr - cm。使用利尿剂和受体阻滞剂的保守医学方法被证明是具有挑战性的。酒精性室间隔消融术成功,无重大并发症,解决了LVOTO并改善了症状。讨论:atr - cm合并LVOTO的诊断和治疗是复杂的。需要彻底的诊断方法以避免管理不善。利尿剂和阻滞剂必须仔细平衡,以达到最佳的临床效果。尽管有最佳药物治疗,但对于持续症状和LVOT梯度高的患者,可考虑酒精性室间隔消融。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Left ventricular outflow tract obstruction in transthyretin amyloid cardiomyopathy: a case report on diagnostic and treatment challenges and role of alcohol septal ablation.

Background: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a restrictive cardiomyopathy caused by amyloid deposition in the myocardium. Its phenotypical overlap with hypertrophic cardiomyopathy, particularly in cases involving left ventricular outflow tract obstruction (LVOTO), challenges accurate diagnosis. Medical management of LVOTO in ATTR-CM is challenged by the opposing effects of beta-blockers and diuretics.

Case summary: A 79-year-old male with left ventricular hypertrophy and LVOTO presented with worsening dyspnoea. Full diagnostic work-up confirmed wild-type ATTR-CM. A conservative medical approach with diuretics and beta-blockers proved challenging. Alcohol septal ablation was successfully performed without major complication, resolving the LVOTO and improving symptoms.

Discussion: The diagnosis and management of ATTR-CM with LVOTO are complex. A thorough diagnostic approach is needed to avoid mismanagement. Diuretics and beta-blockers must be carefully balanced to achieve optimal clinical results. Alcohol septal ablation may be considered in patients with persistent symptoms and high LVOT gradients despite optimal medical therapy.

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
European Heart Journal: Case Reports
European Heart Journal: Case Reports Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.30
自引率
10.00%
发文量
451
审稿时长
14 weeks
×
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
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
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 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信