Regional Amyloid Center AOU Careggi Firenze, Florence, Italy.
Intern Emerg Med. 2013 Apr;8(3):191-203. doi: 10.1007/s11739-011-0647-y. Epub 2011 Jul 8.
Amyloidosis comprises a unique group of diseases that share in common the extracellular deposition of insoluble fibrillar proteins in organs and tissue including the heart. Cardiac amyloidosis could be primary a part of systemic acquired amyloidosis, or a result of heredofamilial amyloidosis. Although the infiltration of the heart from different types of amyloid results in restrictive cardiomyopathy that manifests with refractory congestive heart failure and conduction abnormalities, unequivocal identification of the deposited amyloidogenic protein is mandatory in order to avoid misdiagnosis and inappropriate treatment. Recent developments in imaging techniques and extracardiac tissue biopsy have minimized the need for invasive endomyocardial biopsy for amyloidosis. Despite advances in treatment, the prognosis of a patient with amyloidosis is still poor and depends upon the underlying disease, and the type and degree of dysfunction of the involved organs. Thus, early diagnosis is mandatory because patients with advanced disease are usually too ill for intensive therapy. This review outlines current approaches to diagnosis, assessment of disease severity, and treatment of cardiac amyloidosis.
淀粉样变包括一组独特的疾病，它们共同的特点是在器官和组织（包括心脏）中沉积不可溶的纤维状蛋白质。心脏淀粉样变可能是全身性获得性淀粉样变的一部分，也可能是遗传性家族性淀粉样变的结果。尽管不同类型的淀粉样物质浸润心脏会导致限制型心肌病，表现为难治性充血性心力衰竭和传导异常，但为了避免误诊和不适当的治疗，明确沉积的淀粉样变性蛋白是必需的。成像技术和心脏外组织活检的最新进展已经最大限度地减少了对淀粉样变进行有创性心内膜心肌活检的需求。尽管在治疗方面取得了进展，但淀粉样变患者的预后仍然很差，取决于潜在疾病以及受累器官的功能类型和程度。因此，早期诊断是必要的，因为晚期疾病患者通常病情太重，无法接受强化治疗。本文综述了心脏淀粉样变的诊断、疾病严重程度评估和治疗的当前方法。
