心脏淀粉样变性诊疗进展

Cardiac Amyloidosis: Diagnosis and Therapy

文章描述了心肌淀粉样变性常见的两种分型:轻链型淀粉样变性和转甲状腺素蛋白型(ATTR)淀粉样变性;少见的分型:急性炎症诱发、心房相关型和Apo A-I型淀粉样变性。心肌淀粉样变的传统诊断方法:心电图、超声心动图、心脏磁共振、核素心肌显像、右心导管和心肌活检;基因精准诊断的出现将显著改善患者的诊疗体验和诊疗效果。标准的心力衰竭药物治疗方案对其可能无益;对于心脏和多器官受累的患者,自体干细胞移植可以提高生存率;针对不同分型制定不同的化疗方案亦可以缓解症状,但提高生存率还需进一步研究;因为供体缺乏,心脏移植没有广泛应用。基因治疗相关研究仍然处于基础研究阶段,仍然需要进行更多的机制和临床探索,在此基础上研发出新的治疗策略来治疗这种疾病。

There are 2 most common types of cardiac amyloidosis（CA）：light chain immunoglobulin（AL）and transthyretin（ATTR）amyloidoses.The noninvasive imaging electrocardiogram,echocardiography,cardiac magnetic resonance imaging,nuclear imaging and invasive diagnostic evaluation biopsy are traditional methods to dignose CA.Most medications used for heart failure management are not benefit to patients with CA.Optimized chemotherapy maybe relieve the symptoms.Orthotopic heart transplantation is infrequently used because of organ shortage.Precision medical treatment a new method to diagnose CA is still under research.