Institute of Cardiology, University of Bologna and S. Orsola-Malpighi Hospital, Bologna, Italy.
JACC Cardiovasc Imaging. 2011 Jun;4(6):659-70. doi: 10.1016/j.jcmg.2011.03.016.
In a cohort of patients with hereditary transthyretin-related amyloidosis (ATTR), we aimed to assess the role of (99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid ((99m)Tc-DPD) in detecting myocardial amyloid infiltration across a wide spectrum of cardiac involvement and in predicting major adverse cardiac events (MACE).
Hereditary transthyretin-related amyloidosis is a challenging and underdiagnosed condition where both early diagnosis and prognosis remain problematic.
We evaluated 63 patients with ATTR: 40 with and 23 without echocardiographically diagnosed amyloidotic cardiomyopathy (AC). Myocardial uptake of (99m)Tc-DPD scintigraphy was semiquantitatively and visually assessed at 5 min and 3 h.
All patients with AC showed moderate-to-severe myocardial tracer uptake (i.e., visual score ≥2). Within the subgroup without AC, only 4 patients (with Ala36Pro, Gly47Ala, Thr49Ala, and Glu89Gln transthyretin mutations) showed myocardial tracer uptake and abnormal heart/whole body retention (H/WB) values: in all these cases endomyocardial biopsies showed amyloidotic infiltration. The H/WB was positively correlated with left ventricular (LV) mean wall thickness (Pearson's r=0.695, p<0.001) and negatively with LV ejection fraction (r=-0.368, p=0.004). The H/WB was an unfavorable predictor of MACE-free survival at Cox univariate analysis and contributed to the multivariate model. Notably, LV wall thickness >12 mm in combination with H/WB >7.5 was associated with the highest event rate.
In ATTR, (99m)Tc-DPD scintigraphy can identify myocardial infiltration across a wide spectrum of morphologic/functional cardiac involvement, allowing an early diagnosis of the disease (even before the appearance of echocardiographic abnormalities). The (99m)Tc-DPD myocardial uptake is a prognostic determinant of "cardiac" outcome in ATTR, either alone or in combination with LV wall thickness.
在遗传性转甲状腺素蛋白相关淀粉样变性（ATTR）患者队列中，我们旨在评估（99m）Tc-3,3-二膦酸-1,2-丙烷二羧酸（（99m）Tc-DPD）在检测广泛心脏受累范围内心肌淀粉样浸润以及预测主要不良心脏事件（MACE）方面的作用。
遗传性转甲状腺素蛋白相关淀粉样变性是一种具有挑战性且诊断不足的疾病，早期诊断和预后仍然存在问题。
我们评估了 63 例 ATTR 患者：40 例伴有和 23 例不伴有超声心动图诊断的淀粉样心肌病（AC）。（99m）Tc-DPD 闪烁扫描在 5 分钟和 3 小时时进行半定量和视觉评估。
所有 AC 患者均显示中度至重度心肌示踪剂摄取（即，视觉评分≥2）。在无 AC 的亚组中，只有 4 例患者（携带 Ala36Pro、Gly47Ala、Thr49Ala 和 Glu89Gln 转甲状腺素蛋白突变）显示心肌示踪剂摄取和异常的心脏/全身保留（H/WB）值：在所有这些情况下，心内膜心肌活检均显示淀粉样浸润。H/WB 与左心室（LV）平均壁厚度呈正相关（Pearson r=0.695，p<0.001），与 LV 射血分数呈负相关（r=-0.368，p=0.004）。H/WB 在 Cox 单因素分析中是 MACE 无事件生存率的不利预测因素，并有助于多变量模型。值得注意的是，LV 壁厚度>12mm 与 H/WB>7.5 结合与最高的事件发生率相关。
在 ATTR 中，（99m）Tc-DPD 闪烁扫描可以识别广泛形态/功能心脏受累范围内的心肌浸润，从而实现疾病的早期诊断（甚至在出现超声心动图异常之前）。（99m）Tc-DPD 心肌摄取是 ATTR 中“心脏”结局的预后决定因素，无论是单独使用还是与 LV 壁厚度结合使用。
