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心脏淀粉样变性。

Cardiac amyloidosis.

机构信息

Amyloidosis Network Zurich, University Hospital Zurich, Zurich, Switzerland.

University Heart Center, University Hospital Zurich, Zurich, Switzerland.

出版信息

Swiss Med Wkly. 2024 Nov 14;154:4186. doi: 10.57187/s.4186.

Abstract

Cardiac amyloidosis is a disease characterised by the accumulation of amyloid protein in the heart tissue. There are several types of amyloidosis, but the most common types affecting the heart are ATTR amyloidosis (caused by transthyretin protein) and AL amyloidosis (caused by abnormal immunoglobulin light chains). Cardiac amyloidosis causes typical signs and symptoms of heart failure. Diagnosis involves a combination of imaging tests such as echocardiography and cardiac magnetic resonance imaging, as well as nuclear imaging scans and tissue biopsies to confirm the presence of amyloid deposits in the heart. Treatment of cardiac amyloidosis depends on the type and severity of the disease and includes medications to manage symptoms as well as treatments targeting the underlying cause of amyloidosis. Importantly, cardiac amyloidosis is a serious condition requiring specialised care from a multidisciplinary team including cardiologists and haematologists as well as other specialists familiar with the management of this rare disease. This is crucial, as early diagnosis and treatment are important for improving outcomes.

摘要

心脏淀粉样变性是一种以心脏组织中淀粉样蛋白积聚为特征的疾病。有几种类型的淀粉样变性,但最常见的影响心脏的类型是ATTR 淀粉样变性(由转甲状腺素蛋白引起)和 AL 淀粉样变性(由异常免疫球蛋白轻链引起)。心脏淀粉样变性引起心力衰竭的典型体征和症状。诊断包括结合使用超声心动图和心脏磁共振成像等影像学检查,以及核成像扫描和组织活检,以确认心脏中淀粉样沉积物的存在。心脏淀粉样变性的治疗取决于疾病的类型和严重程度,包括药物治疗以控制症状以及针对淀粉样变性根本原因的治疗。重要的是,心脏淀粉样变性是一种严重的疾病,需要多学科团队的专业护理,包括心脏病专家和血液学家以及其他熟悉这种罕见疾病管理的专家。这一点至关重要,因为早期诊断和治疗对于改善预后很重要。

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