- 作者：Enrica Perugini ; Pier Luigi Guidalotti ; Fabrizio Salvi ; Robin M.T. Cooke ; Cinzia Pettinato ; Letizia Riva ; Ornella Leone ; Mohsen Farsad ; Paolo Ciliberti ; Letizia Bacchi-Reggiani ; Francesco Fallani ; Ang
- 刊名：Journal of the American College of Cardiology
- 出版年：2005
- 出版时间：20 September 2005
- 年：2005
- 卷：46
- 期：6
- 页码：1076-1084
- 全文大小：389 K
Objectives
We investigated the diagnostic accuracy of 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid (99mTc-DPD) scintigraphy for differentiation of monoclonal immunoglobulin light-chain (AL) and transthyretin (TTR)-related cardiac amyloidosis.Background
Differential diagnosis between TTR-related and AL amyloidosis is often complex and time-consuming.
Methods
Patients under routine observation with TTR-related/AL systemic amyloidosis and echocardiographic evidence of cardiac involvement were studied with 99mTc-DPD scintigraphy.
Results
Patients with cardiac involvement of TTR-related (group A; n = 15) and AL (group B; n = 10) etiology were comparable for left ventricular mass and renal function. Heart and heart/whole-body tracer retention were significantly higher (p < 0.05) in group A as compared with group B and with 10 unaffected controls. At visual scoring, cardiac 99mTc-DPD uptake was present in all group A patients and absent in all group B patients; thus, using genotyping/immunohistochemistry as the reference technique, the accuracy of 99mTc-DPD scintigraphy for distinction of TTR-related and AL etiology was 100 % . Cardiac 99mTc-DPD uptake was also absent among unaffected controls. Using echocardiography as the reference standard for recognition of cardiac involvement, sensitivity and specificity of scintigraphy were both 100 % for group A patients; in group B, sensitivity was 0 % and specificity was 100 % (accuracy, 50 % ). Eleven patients with myocardial 99mTc-DPD uptake underwent 99mTc-methylene diphosphonate (99mTc-MDP) scintigraphy; all patients showed a 99mTc-MDP myocardial visual score of 0.
Conclusions
Etiology is a third major cause—in addition to type of organ-involved (soft-tissue/heart) and tracer type—of scintigraphic variability in cardiac amyloidosis. This is a highly relevant consideration for future studies. We conclude that 99mTc-DPD scintigraphy is a useful step in the workup of the differential diagnosis of TTR versus AL etiology in patients with documented cardiac amyloidosis.