Title of article :
Noninvasive Etiologic Diagnosis of Cardiac Amyloidosis Using 99mTc-3,3-Diphosphono-1,2-Propanodicarboxylic Acid Scintigraphy Original Research Article
Author/Authors :
Enrica Perugini، نويسنده , , Pier Luigi Guidalotti، نويسنده , , Fabrizio Salvi، نويسنده , , Robin M.T. Cooke، نويسنده , , Cinzia Pettinato، نويسنده , , Letizia Riva، نويسنده , , Ornella Leone، نويسنده , , Mohsen Farsad، نويسنده , , Paolo Ciliberti، نويسنده , , Letizia Bacchi-Reggiani، نويسنده , , Francesco Fallani، نويسنده , , Angelo Branzi، نويسنده , , Claudio Rapezzi، نويسنده ,
Issue Information :
روزنامه با شماره پیاپی سال 2005
Abstract :
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.
Keywords :
AL , SPECT , Transthyretin , Ca , Single-photon emission computed tomography , TTR , ROI , Region of interest , LV , left ventricle/ventricular , monoclonal immunoglobulin light-chain , cardiac amyloidosis , OLT , orthotopic liver transplantation , 99mTc-DPD , 99mTc-3 , 3-diphosphono-1 , 2-propanodicarboxylic acid , 99mTc-MDP , 99mTc-methylene diphosphonate
Journal title :
JACC (Journal of the American College of Cardiology)
Journal title :
JACC (Journal of the American College of Cardiology)