Department of Medicine, Mayo Clinic, Rochester, MN, USA.
Am J Med. 2011 Nov;124(11):1006-15. doi: 10.1016/j.amjmed.2011.04.013.
Cardiac amyloidosis, the primary determinant of prognosis in systemic amyloidoses, is characterized by infiltration of myocardium by amyloid protein resulting in cardiomyopathy and conduction disturbances. Cardiac involvement is primarily encountered in immunoglobulin (AL) and transthyretin-associated (hereditary/familial and senile) amyloidoses. Although the latter variants could be indolent, untreated AL amyloidosis with clinical cardiac involvement is a rapidly fatal disease. The management decisions of cardiac amyloidosis are based on the underlying cause. Although treatment of senile systemic amyloidosis is largely supportive, the therapeutic approaches for AL amyloidosis include chemotherapy, autologous stem cell transplantation, and, rarely, cardiac transplantation. The familial variant is potentially curable with a liver ± cardiac transplantation. This narrative review outlines a practical approach to these challenging diagnoses in the face of rapidly evolving management strategies.
心脏淀粉样变性是全身性淀粉样变性预后的主要决定因素，其特征是心肌中淀粉样蛋白浸润导致心肌病和传导障碍。心脏受累主要见于免疫球蛋白 (AL) 和转甲状腺素相关 (遗传性/家族性和老年性) 淀粉样变性。虽然后者可能进展缓慢，但未经治疗的伴有临床心脏受累的 AL 淀粉样变性是一种快速致命的疾病。心脏淀粉样变性的治疗决策基于潜在病因。虽然老年性系统性淀粉样变性的治疗主要是支持性的，但 AL 淀粉样变性的治疗方法包括化疗、自体干细胞移植，极少数情况下还包括心脏移植。家族性变异通过肝 ± 心脏移植是可以治愈的。本综述概述了在快速发展的治疗策略背景下，对这些具有挑战性的诊断的实用方法。
