Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV, Madrid, Spain.
Universidad Francisco de Vitoria (UFV), Pozuelo de Alarcon, Spain.
Eur J Heart Fail. 2021 Apr;23(4):512-526. doi: 10.1002/ejhf.2140. Epub 2021 Apr 7.
Cardiac amyloidosis is a serious and progressive infiltrative disease that is caused by the deposition of amyloid fibrils at the cardiac level. It can be due to rare genetic variants in the hereditary forms or as a consequence of acquired conditions. Thanks to advances in imaging techniques and the possibility of achieving a non-invasive diagnosis, we now know that cardiac amyloidosis is a more frequent disease than traditionally considered. In this position paper the Working Group on Myocardial and Pericardial Disease proposes an invasive and non-invasive definition of cardiac amyloidosis, addresses clinical scenarios and situations to suspect the condition and proposes a diagnostic algorithm to aid diagnosis. Furthermore, we also review how to monitor and treat cardiac amyloidosis, in an attempt to bridge the gap between the latest advances in the field and clinical practice.
心脏淀粉样变是一种严重且进行性浸润性疾病,由心脏水平的淀粉样纤维沉积引起。它可能是由于遗传性形式中的罕见遗传变异,或作为获得性疾病的后果。由于成像技术的进步和实现非侵入性诊断的可能性,我们现在知道心脏淀粉样变比传统上认为的更为常见。在这份立场文件中,心肌和心包疾病工作组提出了心脏淀粉样变的有创和无创定义,讨论了怀疑该疾病的临床情况和场景,并提出了一个诊断算法来辅助诊断。此外,我们还回顾了如何监测和治疗心脏淀粉样变,试图弥合该领域的最新进展与临床实践之间的差距。