Curr Probl Cardiol. 2018 Jan;43(1):10-34. doi: 10.1016/j.cpcardiol.2017.04.003. Epub 2017 Apr 13.
Cardiac amyloidosis occurs because of abnormal protein (amyloid) deposition in the cardiac tissue. Even with advanced diagnostic techniques and treatments, the prognosis of amyloidosis remains poor. The diagnosis of cardiac amyloidosis particularly needs to be in the differential in patients presenting with heart failure with preserved ejection fraction. This entity remains underdiagnosed due to lack of suspicion on the part of many clinicians. Involvement of cardiac tissue is the utmost determinant factor for available treatment options and prognosis. Many cases of cardiac amyloidosis usually remain undiagnosed or diagnosed only in advanced stages when treatment options are limited and associated with poor survival. Hence, early recognition of cardiac amyloidosis is indispensable in halting the disease process before irreversible changes occur. The purpose of this review is to summarize the recent updates in the evaluation and management of cardiac amyloidosis and to discuss potential future treatments options.
心脏淀粉样变是由于心脏组织中异常蛋白(淀粉样物质)沉积所致。即使有先进的诊断技术和治疗方法,淀粉样变性的预后仍然很差。心脏淀粉样变性的诊断特别需要在射血分数保留的心力衰竭患者中进行鉴别诊断。由于许多临床医生缺乏怀疑,这种疾病仍然诊断不足。心脏组织受累是决定治疗选择和预后的最重要因素。许多心脏淀粉样变性病例通常未被诊断,或仅在晚期诊断,此时治疗选择有限,且与生存率低相关。因此,在不可逆变化发生之前,早期识别心脏淀粉样变性对于阻止疾病进程是不可或缺的。本文的目的是总结心脏淀粉样变性评估和管理方面的最新进展,并讨论潜在的未来治疗选择。