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心脏淀粉样变性的警示信号:所有心脏病专家都必须了解的内容。

Cardiac amyloidosis red flags: What all the cardiologist have to know.

作者信息

Maloberti Alessandro, Ciampi Claudio, Politi Francesco, Fabbri Saverio, Musca Francesco, Giannattasio Cristina

机构信息

School of Medicine and Surgery, Milan-Bicocca University, Milan, Italy.

Cardiology 4, ASST Niguarda Ca Granda Hospital, Milan, Italy.

出版信息

Int J Cardiol Cardiovasc Risk Prev. 2024 Apr 27;21:200271. doi: 10.1016/j.ijcrp.2024.200271. eCollection 2024 Jun.

Abstract

Cardiac amyloidosis is becoming increasingly important among cardiologist and an early diagnosis is very important. Amyloidosis is a systemic disease and many cardiac and extracardiac elements (red flags) should raise the suspicion of the disease. Electrocardiographic and imaging techniques (such as echocardiography, cardiac magnetic resonance and scintigraphy) are useful tools to make a diagnosis together with the presence of orthopedic issues, peripheral neuropathy or plasma cell dyscrasia. Cardiac amyloidosis is also often associated with valvular disorder, heart failure or cardiomyopathy. Red flags are crucial to raise suspicion and reach an early diagnosis, in order to start a targeted treatment strategy that could change the patient's outcome. Indeed, in the last years four new drugs were approved to treat transthyretin amyloidosis.

摘要

心脏淀粉样变性在心脏病专家中愈发重要,早期诊断非常关键。淀粉样变性是一种全身性疾病,许多心脏和心外因素(警示信号)应引起对该疾病的怀疑。心电图和成像技术(如超声心动图、心脏磁共振和闪烁扫描)是与存在骨科问题、周围神经病变或浆细胞异常增生一起用于诊断的有用工具。心脏淀粉样变性也常与瓣膜疾病、心力衰竭或心肌病相关。警示信号对于提高怀疑并实现早期诊断至关重要,以便启动可能改变患者预后的靶向治疗策略。事实上,在过去几年中,有四种新药被批准用于治疗转甲状腺素蛋白淀粉样变性。

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本文引用的文献

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Patisiran Treatment in Patients with Transthyretin Cardiac Amyloidosis.
N Engl J Med. 2023 Oct 26;389(17):1553-1565. doi: 10.1056/NEJMoa2300757.
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Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy.
N Engl J Med. 2018 Sep 13;379(11):1007-1016. doi: 10.1056/NEJMoa1805689. Epub 2018 Aug 27.

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