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心脏淀粉样变性诊断的镶嵌模式:影像学在疾病亚型和分期中的作用。

The mosaic of the cardiac amyloidosis diagnosis: role of imaging in subtypes and stages of the disease.

机构信息

Clinical and Experimental Medicine Department, Cardiology Unit, University of Messina, Azienda Ospedaliera Universitaria 'Policlinico G. Martino', Via Consolare Valeria 1, Messina 98125, Italy.

Department of Biomedical Sciences and of Morphologic and Functional Images, University of Messina, Azienda Ospedaliera Universitaria 'Policlinico G. Martino', Via Consolare Valeria 1, Messina 98125, Italy.

出版信息

Eur Heart J Cardiovasc Imaging. 2014 Dec;15(12):1307-15. doi: 10.1093/ehjci/jeu158. Epub 2014 Sep 4.

Abstract

Cardiac amyloidosis is a rare, infiltrative cardiomyopathy that presents with thickened ventricular walls and progressive heart failure. The morphological findings and clinical features are shared with many other diseases (i.e. hypertrophic cardiomyopathy, 'athlete's heart,' Fabry disease, and hypertensive cardiomyopathy), and misdiagnosis occurs frequently. Cardiologists have many instruments that can help reach a correct diagnosis in a relatively short time. As tiles of a mosaic are placed to create an image, thoughtful and smart use of the different diagnostic tools available allows the opportunity to identify amyloid infiltration of the myocardium. When the myocardium is involved, prognosis is poor, so identification of its involvement is crucial for disease management. The diagnostic process begins with an accurate evaluation of clinical elements and includes cardiovascular imaging (echocardiography, magnetic resonance, and nuclear medicine), electrocardiography, serological assays, and myocardial biopsy; only the appropriate integration of these instruments can reveal the diagnosis to an expert physician. The latest improvements in non-invasive diagnostic techniques with increased diagnostic power have reduced the need for biopsy.

摘要

心脏淀粉样变是一种罕见的浸润性心肌病,表现为心室壁增厚和进行性心力衰竭。其形态学发现和临床特征与许多其他疾病(如肥厚型心肌病、“运动员心脏”、法布里病和高血压性心肌病)相似,误诊较为常见。心脏病专家有许多工具可以帮助在相对较短的时间内做出正确的诊断。就像马赛克拼图一样,明智地使用不同的诊断工具可以识别心肌淀粉样变的浸润。当心肌受累时,预后较差,因此识别心肌受累对疾病管理至关重要。诊断过程首先要对临床要素进行准确评估,包括心血管影像学(超声心动图、磁共振成像和核医学)、心电图、血清学检测和心肌活检;只有适当整合这些工具,才能为专家医生揭示诊断结果。随着诊断能力的提高,非侵入性诊断技术的最新进展减少了对活检的需求。

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