Garcia-Pavia Pablo, Rapezzi Claudio, Adler Yehuda, Arad Michael, Basso Cristina, Brucato Antonio, Burazor Ivana, Caforio Alida L P, Damy Thibaud, Eriksson Urs, Fontana Marianna, Gillmore Julian D, Gonzalez-Lopez Esther, Grogan Martha, Heymans Stephane, Imazio Massimo, Kindermann Ingrid, Kristen Arnt V, Maurer Mathew S, Merlini Giampaolo, Pantazis Antonis, Pankuweit Sabine, Rigopoulos Angelos G, Linhart Ales
Heart Failure and Inherited Cardiac Diseases Unit, Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, CIBERCV, Manuel de Falla, 2, 28222 Madrid, Spain.
Universidad Francisco de Vitoria (UFV), Pozuelo de Alarcon, Spain.
Eur Heart J. 2021 Apr 21;42(16):1554-1568. doi: 10.1093/eurheartj/ehab072.
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.
心脏淀粉样变性是一种严重的进行性浸润性疾病,由淀粉样纤维在心脏水平沉积所致。它可能源于遗传性形式中的罕见基因变异,或后天性疾病的结果。得益于成像技术的进步以及实现无创诊断的可能性,我们现在知道心脏淀粉样变性是一种比传统认知中更为常见的疾病。在本立场文件中,心肌和心包疾病工作组提出了心脏淀粉样变性的有创和无创定义,阐述了怀疑该疾病的临床情况和场景,并提出了一种辅助诊断的算法。此外,我们还回顾了如何监测和治疗心脏淀粉样变性,试图弥合该领域最新进展与临床实践之间的差距。