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规范化甲状腺素运载蛋白淀粉样心肌病的治疗和护理。

Standardising Care and Treatment of Transthyretin Amyloid Cardiomyopathy.

机构信息

National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK.

Centre for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI), University of Trieste, Trieste 34149, IT.

出版信息

Glob Heart. 2023 Nov 20;18(1):63. doi: 10.5334/gh.1275. eCollection 2023.

Abstract

Transthyretin cardiac amyloidosis (ATTR-CA) has been traditionally considered a rare and inexorably fatal condition. ATTR-CA now is an increasingly recognised cause of heart failure and mortality worldwide with effective pharmacological treatments. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have transformed the diagnosis of ATTR-CA, which is now possible without recourse to endomyocardial biopsy in around 70% of cases. Many patients are now diagnosed at an earlier stage. Echocardiography and cardiac magnetic resonance have enabled identification of patients with possible ATTR-CA and more accurate prognostic stratification. Therapies able to slow or halt ATTR-CA progression and increase survival are now available and there is also evidence that patients may benefit from specific conventional heart failure medications. A wide horizon of possibilities is unfolding and awaits discovery.

摘要

转甲状腺素蛋白心脏淀粉样变(ATTR-CA)传统上被认为是一种罕见且不可避免的致命疾病。随着有效的药物治疗,ATTR-CA 现已成为全球心力衰竭和死亡率日益公认的原因。非侵入性诊断的进步,加上有效治疗方法的发展,改变了ATTR-CA 的诊断方式,现在约 70%的病例无需进行心内膜心肌活检即可进行诊断。许多患者现在在早期就得到了诊断。超声心动图和心脏磁共振成像使人们能够识别出可能患有ATTR-CA 的患者,并进行更准确的预后分层。现在有能够减缓或阻止ATTR-CA 进展并提高生存率的治疗方法,并且也有证据表明,患者可能受益于特定的常规心力衰竭药物。广阔的可能性正在展开,有待发现。

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