TY - JOUR
T1 - Usefulness and limitations of 99mTc-3, 3-diphosphono-1, 2-propanodicarboxylic acid scintigraphy in the aetiological diagnosis of amyloidotic cardiomyopathy
AU - Rapezzi, Claudio
AU - Quarta, Candida Cristina
AU - Guidalotti, Pier Luigi
AU - Longhi, Simone
AU - Pettinato, Cinzia
AU - Leone, Ornella
AU - Ferlini, Alessandra
AU - Salvi, Fabrizio
AU - Gallo, Pamela
AU - Gagliardi, Christian
AU - Branzi, Angelo
PY - 2011/3
Y1 - 2011/3
N2 - Purpose We previously reported in a small series of patients that 99mTc-3, 3-diphosphono-1, 2-propanodicarboxylic acid ( 99mTc-DPD) scintigraphy tested positive in transthyretin-related (TTR) (both mutant and wild-type) but not in primary (AL) amyloidotic cardiomyopathy (AC). We extended our study to a larger cohort of patients with AC. Methods We evaluated (1) 45 patients with TTR-related AC (28 mutant and 17 wild-type), (2) 34 with AL-related AC and (3) 15 non-affected controls. Myocardial uptake of 99mTc-DPD (740 MBq i.v.) was semiquantitatively and visually assessed at 5 min and at 3 h. Results Heart retention (HR) and heart to whole-body retention ratio (H/WB) of late 99mTc-DPD uptake were higher among TTR-related AC (HR 7.8%; H/WB 10.4) compared with both unaffected controls (HR 3.5%; H/WB 5.7; p<0.0001) and AL-related AC (HR 4.0%; H/WB 6.1; p<0.0001). For the diagnosis of TTR-related AC, positive and negative predictive accuracy of visual scoring of cardiac retention were: 80 and 100% (visual score ≥1); 88 and 100% (visual score ≥2); and 100 and 68% (visual score = 3). At adjusted linear regression analysis, TTR aetiology turned out to be the only positive predictor of increasing 99mTc-DPD uptake in terms of both HR [β 2.5, 95% confidence interval (CI) 1.5-3.5; p99mTc-DPD scintigraphy was confirmed to be useful for differentiating TTR from AL-related AC, diagnostic accuracy was lower than previously reported due to a mild degree of tracer uptake in about one third of AL patients. 99mTc-DPD scintigraphy can provide an accurate differential diagnosis in cases of absent or intense uptake evaluated by visual score.
AB - Purpose We previously reported in a small series of patients that 99mTc-3, 3-diphosphono-1, 2-propanodicarboxylic acid ( 99mTc-DPD) scintigraphy tested positive in transthyretin-related (TTR) (both mutant and wild-type) but not in primary (AL) amyloidotic cardiomyopathy (AC). We extended our study to a larger cohort of patients with AC. Methods We evaluated (1) 45 patients with TTR-related AC (28 mutant and 17 wild-type), (2) 34 with AL-related AC and (3) 15 non-affected controls. Myocardial uptake of 99mTc-DPD (740 MBq i.v.) was semiquantitatively and visually assessed at 5 min and at 3 h. Results Heart retention (HR) and heart to whole-body retention ratio (H/WB) of late 99mTc-DPD uptake were higher among TTR-related AC (HR 7.8%; H/WB 10.4) compared with both unaffected controls (HR 3.5%; H/WB 5.7; p<0.0001) and AL-related AC (HR 4.0%; H/WB 6.1; p<0.0001). For the diagnosis of TTR-related AC, positive and negative predictive accuracy of visual scoring of cardiac retention were: 80 and 100% (visual score ≥1); 88 and 100% (visual score ≥2); and 100 and 68% (visual score = 3). At adjusted linear regression analysis, TTR aetiology turned out to be the only positive predictor of increasing 99mTc-DPD uptake in terms of both HR [β 2.5, 95% confidence interval (CI) 1.5-3.5; p99mTc-DPD scintigraphy was confirmed to be useful for differentiating TTR from AL-related AC, diagnostic accuracy was lower than previously reported due to a mild degree of tracer uptake in about one third of AL patients. 99mTc-DPD scintigraphy can provide an accurate differential diagnosis in cases of absent or intense uptake evaluated by visual score.
KW - Tc-DPD scintigraphy
KW - Accuracy
KW - Amyloid
KW - Cardiomyopathy
KW - Differential diagnosis
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U2 - 10.1007/s00259-010-1642-7
DO - 10.1007/s00259-010-1642-7
M3 - Article
C2 - 21069320
AN - SCOPUS:79953733503
VL - 38
SP - 470
EP - 478
JO - European Journal of Pediatrics
JF - European Journal of Pediatrics
SN - 0340-6199
IS - 3
ER -