- 作者：Claudio Rapezzi ; MD?/sup> ; claudio.rapezzi@unibo.it ; Candida C. Quarta ; MD?/sup> ; Pier Luigi Guidalotti ; MD&dagger ; ; Cinzia Pettinato ; MD&dagger ; ; Stefano Fanti ; MD&dagger ; ; Ornella Leone ; MD&Dagger ; ; Alessandra Ferlini ; MD§ ; ; Simone Longhi ; MD?/sup> ; Massimiliano Lorenzini ; MD?/sup> ; Letizia Bacchi Reggiani ; MSc ; MStat?/sup> ; Christian Gagliardi ; MD?/sup> ; Pamela Gallo ; MD?/sup> ; Caterina Villani ; MD?/sup> ; Fabrizio Salvi ; MD?/sup>
- 关键词：99mTc-DPD scintigraphy ; cardiac amyloidosis ; diagnosis ; hereditary transthyretin-related amyloidosis ; prognosis
- 刊名：JACC: Cardiovascular Imaging
- 出版年：2011
- 出版时间：June 2011
- 年：2011
- 卷：4
- 期：6
- 页码：659-670
- 全文大小：1503 K
Objectives
In a cohort of patients with hereditary transthyretin-related amyloidosis (ATTR), we aimed to assess the role of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) in detecting myocardial amyloid infiltration across a wide spectrum of cardiac involvement and in predicting major adverse cardiac events (MACE).Background
Hereditary transthyretin-related amyloidosis is a challenging and underdiagnosed condition where both early diagnosis and prognosis remain problematic.
Methods
We evaluated 63 patients with ATTR: 40 with and 23 without echocardiographically diagnosed amyloidotic cardiomyopathy (AC). Myocardial uptake of 99mTc-DPD scintigraphy was semiquantitatively and visually assessed at 5 min and 3 h.
Results
All patients with AC showed moderate-to-severe myocardial tracer uptake (i.e., visual score ?). 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 = ?.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.
Conclusions
In ATTR, 99mTc-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 99mTc-DPD myocardial uptake is a prognostic determinant of ¡°cardiac?outcome in ATTR, either alone or in combination with LV wall thickness.