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[心脏淀粉样变性与主动脉瓣狭窄]

[Cardiac amyloidosis and aortic valve stenosis].

作者信息

Erbel-Khurtsidze Sophiko, Seibel Rainer, Moka Detlef, Brandt-Mainz Ka T J A, Erbel Raimund, Lichtenberg Artur

机构信息

Klinik für Herzchirurgie, Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität, Moorenstr. 5, 40225, Düsseldorf, Deutschland.

Diagnosticum, Mülheim/Ruhr, Deutschland.

出版信息

Herz. 2021 Oct;46(5):485-496. doi: 10.1007/s00059-021-05054-x. Epub 2021 Sep 6.

Abstract

Aortic valve stenosis in old age has become a topic of interest for cardiology and cardiac surgery after the development of transvascular and transluminal minimally invasive techniques for aortic valve implantation. The observation of amyloid deposits in surgically excised valvular material led to the diagnostics of amyloidosis of the myocardium, which was discovered in up to 20% of the patients who underwent valve implantation. Clinical signs of cardiac amyloidosis, such as carpal tunnel syndrome and ruptured distal biceps tendon should be taken into account. In addition to the electrocardiogram (ECG), echocardiogram and magnetic resonance imaging, technetium bone scintigraphy plays a key diagnostic role. The simultaneous occurrence of severe aortic valve stenosis and amyloidosis explains the special hemodynamic situation of a low gradient with low blood flow in high-grade valve stenosis. The interventional or surgical valve implantation improves the prognosis for these patients, similarly to aortic valve stenosis alone, followed by a specific pharmaceutical treatment depending on the type of amyloidosis.

摘要

随着经血管和腔内微创主动脉瓣植入技术的发展,老年主动脉瓣狭窄已成为心脏病学和心脏外科关注的话题。在手术切除的瓣膜组织中观察到淀粉样沉积物,从而诊断出心肌淀粉样变性,在接受瓣膜植入的患者中,高达20%的人被发现患有这种疾病。应考虑心脏淀粉样变性的临床体征,如腕管综合征和肱二头肌远端肌腱断裂。除了心电图(ECG)、超声心动图和磁共振成像外,锝骨闪烁显像也起着关键的诊断作用。严重主动脉瓣狭窄和淀粉样变性同时出现,解释了重度瓣膜狭窄时低梯度、低血流量的特殊血流动力学情况。介入或外科瓣膜植入术改善了这些患者的预后,与单纯主动脉瓣狭窄相似,随后根据淀粉样变性的类型进行特定的药物治疗。

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