PDF(Pubmed)
Abstract：
Background: Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) is a life-threatening progressive disease. Recent studies have shown that the detection of transthyretin (TTR) amyloid in tenosynovial tissue may play an important role in the diagnosis of cardiac amyloidosis. The aim of this study was to determine the prevalence of TTR amyloid deposits in surgical tissue of patients undergoing carpal tunnel surgery and to clarify the clinical significance of concomitant cardiac examination with 99 mTc-labeled pyrophosphate (99 mTc-PYP) scintigraphy in those patients with TTR deposition. Methods and Results: We evaluated 79 consecutive patients undergoing carpal tunnel release surgery and biopsy of tenosynovial tissue. The mean (±SD) age of the patients at surgery was 71.6±12.5 years (range 30-95 years); 32 patients (41%) were male. TTR amyloid deposition in tenosynovial tissue was observed in 27 patients (34%). Sixteen of those 27 patients underwent 99 mTc-PYP scintigraphy. Of those 16 patients, 3 (19%) had Grade 2 uptake on 99 mTc-PYP scintigraphy. None of the 3 patients with a diagnosis of ATTRwt-CA had apparent cardiac symptoms and left ventricular wall thickness >13 mm. Conclusions: Concomitant cardiac examination with 99 mTc-PYP scintigraphy in patients who had TTR amyloid deposition in tenosynovial tissue resulted in the identification of 19% of patients with a diagnosis of ATTRwt-CA. This diagnostic approach seems to be useful for the early diagnosis of the disease.
摘要：
背景：野生型甲状腺素运载蛋白心脏淀粉样变性（ATTRwt-CA）是一种威胁生命的进行性疾病。最近的研究表明，在腱膜组织中检测甲状腺素运载蛋白（TTR）淀粉样蛋白可能在心脏淀粉样变性的诊断中起重要作用。这项研究的目的是确定接受腕管手术的患者手术组织中TTR淀粉样蛋白沉积的患病率，并阐明99mTc标记的焦磷酸盐（99mTc-PYP）闪烁显像伴随心脏检查的临床意义那些有TTR沉积的患者。方法和结果：我们评估了79例连续接受腕管松解术和腱膜组织活检的患者。手术患者的平均（±SD）年龄为71.6±12.5岁（范围30-95岁）；32例（41％）为男性。在27例患者（34％）中观察到TTR淀粉样蛋白在腱膜组织中的沉积。这27例患者中有16例接受了99mTc-PYP闪烁显像。在这16名患者中，3（19％）在99mTc-PYP闪烁显像中具有2级摄取。诊断为ATTRwt-CA的3例患者均未出现明显的心脏症状且左心室壁厚度>13mm。结论：在腱膜组织中TTR淀粉样蛋白沉积的患者中，伴随99mTc-PYP闪烁显像的心脏检查可鉴定出19％的诊断为ATTRwt-CA的患者。这种诊断方法似乎对疾病的早期诊断有用。
