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.
| Original language | English |
|---|---|
| Pages (from-to) | 1076-1084 |
| Number of pages | 9 |
| Journal | Journal of the American College of Cardiology |
| Volume | 46 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Sep 20 2005 |
Fingerprint
ASJC Scopus subject areas
- Nursing(all)
Cite this
Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy. / Perugini, Enrica; Guidalotti, Pier Luigi; Salvi, Fabrizio; Cooke, Robin M T; Pettinato, Cinzia; Riva, Letizia; Leone, Ornella; Farsad, Mohsen; Ciliberti, Paolo; Bacchi-Reggiani, Letizia; Fallani, Francesco; Branzi, Angelo; Rapezzi, Claudio.
In: Journal of the American College of Cardiology, Vol. 46, No. 6, 20.09.2005, p. 1076-1084.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy
AU - Perugini, Enrica
AU - Guidalotti, Pier Luigi
AU - Salvi, Fabrizio
AU - Cooke, Robin M T
AU - Pettinato, Cinzia
AU - Riva, Letizia
AU - Leone, Ornella
AU - Farsad, Mohsen
AU - Ciliberti, Paolo
AU - Bacchi-Reggiani, Letizia
AU - Fallani, Francesco
AU - Branzi, Angelo
AU - Rapezzi, Claudio
PY - 2005/9/20
Y1 - 2005/9/20
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=24944518476&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=24944518476&partnerID=8YFLogxK
U2 - 10.1016/j.jacc.2005.05.073
DO - 10.1016/j.jacc.2005.05.073
M3 - Article
C2 - 16168294
AN - SCOPUS:24944518476
VL - 46
SP - 1076
EP - 1084
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
SN - 0735-1097
IS - 6
ER -