Kim Hyue Mee, Sohn Dae-Won, Paeng Jin Chul
Division of Cardiology, Cardiovascular Center and Department of Internal Medicine, Seoul National University Hospital.
Department of Nuclear Medicine, Seoul National University Hospital.
Int Heart J. 2019 May 30;60(3):643-647. doi: 10.1536/ihj.18-345. Epub 2019 Apr 25.
Senile or wild-type transthyretin (wtTTR) amyloidosis is an age-related disease caused by the deposition of wtTTR amyloid protein. In contrast to light chain amyloidosis, Tc-DPD scintigraphy (DPD scan) is a useful diagnostic modality for wtTTR amyloidosis.We retrospectively analyzed patients older than 30 years who underwent DPD scanning for non-cardiac reasons at our hospital between June 2014 and March 2017 (n = 9,581). Transthoracic echocardiography was used to assess left ventricular hypertrophy (LVH), as well as systolic and diastolic function.A positive DPD scan was observed in only six patients (0.06%). All six of these patients were older than 70 years, and they constitute only 0.4% of patients in this age group (6/1652). Among the patients with a positive DPD scan, four showed concentric LVH and two showed a normal wall thickness. With respect to the severity of diastolic dysfunction and pulmonary artery pressure, patients with a positive DPD scan showed the expected E' and pulmonary artery systolic pressure for their age.Even considering the limited sensitivity of a positive DPD scan detecting wtTTR amyloidosis, the incidence of a positive DPD scan in non-cardiac patients indicated that wtTTR amyloid deposition does not seem to be a major cause for age-related diastolic dysfunction, nor does appear to have a high incidence in patients with heart failure with preserved EF in the elderly.
老年型或野生型转甲状腺素蛋白(wtTTR)淀粉样变性是一种与年龄相关的疾病,由wtTTR淀粉样蛋白沉积引起。与轻链淀粉样变性不同,锝[99mTc]3,3-二膦酸乙烷(Tc-DPD)闪烁扫描(DPD扫描)是wtTTR淀粉样变性的一种有用诊断方法。我们回顾性分析了2014年6月至2017年3月在我院因非心脏原因接受DPD扫描的30岁以上患者(n = 9581)。采用经胸超声心动图评估左心室肥厚(LVH)以及收缩和舒张功能。仅6例患者(0.06%)DPD扫描呈阳性。所有这6例患者年龄均超过70岁,且仅占该年龄组患者的0.4%(6/1652)。在DPD扫描阳性的患者中,4例表现为向心性LVH,2例表现为壁厚度正常。关于舒张功能障碍的严重程度和肺动脉压力,DPD扫描阳性的患者显示出与其年龄相符的E'和肺动脉收缩压。即使考虑到DPD扫描阳性检测wtTTR淀粉样变性的敏感性有限,非心脏患者中DPD扫描阳性的发生率表明,wtTTR淀粉样蛋白沉积似乎不是与年龄相关的舒张功能障碍的主要原因,在老年射血分数保留的心力衰竭患者中也似乎没有高发生率。