Department of Medicine, Westchester Medical Center, New York Medical College, Valhalla, NY, USA.
Department of Cardiology, Westchester Medical Center, New York Medical College, 100 Woods Rd, Macy Pavilion, Valhalla, NY, 10595, USA.
J Nucl Cardiol. 2023 Oct;30(5):1986-1991. doi: 10.1007/s12350-023-03316-1. Epub 2023 Jun 20.
Technetium-99mm pyrophosphate (Tc-PYP) scintigraphy is a highly accurate non-invasive method for the diagnosis of transthyretin (ATTR) cardiac amyloidosis. Prognosis for this disease is improved following treatment with the transthyretin (TTR) stabilizer tafamidis. Although tafamidis slows disease progression, its effects on myocardial amyloid and Tc-PYP uptake remain unclear. We present a patient with ATTR cardiac amyloidosis who had a strongly positive initial Tc-PYP scan, with a dramatic decrease in Tc-PYP uptake on repeat scan after 3 years of tafamidis treatment. However, myocardial biopsy showed persistent diffuse amyloid deposits. This case highlights the need for further studies regarding the utility of serial Tc-PYP scans in monitoring the progress of ATTR cardiomyopathy.
锝-99mm 焦磷酸盐（Tc-PYP）闪烁显像术是诊断转甲状腺素蛋白（ATTR）心脏淀粉样变性的一种高度准确的非侵入性方法。用转甲状腺素（TTR）稳定剂他法米汀治疗后，该病的预后得到改善。尽管他法米汀可减缓疾病进展，但它对心肌淀粉样变和 Tc-PYP 摄取的影响仍不清楚。我们报告了一例 ATTR 心脏淀粉样变性患者，其初始 Tc-PYP 扫描呈强烈阳性，在接受他法米汀治疗 3 年后重复扫描时 Tc-PYP 摄取量明显下降。然而，心肌活检显示持续弥漫性淀粉样沉积物。该病例强调了需要进一步研究连续 Tc-PYP 扫描在监测 ATTR 心肌病进展中的作用。
