Department of Cardiology, Heart Centre Lucerne, Lucerne Cantonal Hospital, Lucerne, Switzerland.
Praxis (Bern 1994). 2023 Apr;112(5-6):357-361. doi: 10.1024/1661-8157/a004001.
Cardiac wtATTR is caused by extracellular deposition of misfolded proteins in the heart. It mostly affects elderly men and is still clearly underdiagnosed. Recognizing red flags suggesting wtATTR is key for a timely diagnosis, enabling the patient to profit from effective therapies. If general practitioners suspect cardiac amyloidosis, it is crucial to rapidly exclude AL-amyloidosis by immunoelectrophoresis, immunofixation as well as light-chain assay, because AL-amyloidosis needs urgent hematologic therapy. After that, the patient should be referred to the cardiologist for further assessment.
心肌 wtATTR 由心脏中错误折叠的蛋白质的细胞外沉积引起。它主要影响老年男性,且仍明显诊断不足。识别提示 wtATTR 的危险信号是及时诊断的关键,使患者能够从有效治疗中获益。如果全科医生怀疑心脏淀粉样变性,通过免疫电泳、免疫固定和轻链测定迅速排除 AL-淀粉样变性至关重要,因为 AL-淀粉样变性需要紧急血液学治疗。之后,患者应转介给心脏病专家进行进一步评估。