TY - JOUR
T1 - Role of 99mTc-DPD scintigraphy in diagnosis and prognosis of hereditary transthyretin-related cardiac amyloidosis
AU - Rapezzi, Claudio
AU - Quarta, Candida C.
AU - Guidalotti, Pier Luigi
AU - Pettinato, Cinzia
AU - Fanti, Stefano
AU - Leone, Ornella
AU - Ferlini, Alessandra
AU - Longhi, Simone
AU - Lorenzini, Massimiliano
AU - Reggiani, Letizia Bacchi
AU - Gagliardi, Christian
AU - Gallo, Pamela
AU - Villani, Caterina
AU - Salvi, Fabrizio
PY - 2011/6
Y1 - 2011/6
N2 - 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 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.
AB - 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 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.
KW - Tc-DPD scintigraphy
KW - cardiac amyloidosis
KW - diagnosis
KW - hereditary transthyretin-related amyloidosis
KW - prognosis
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U2 - 10.1016/j.jcmg.2011.03.016
DO - 10.1016/j.jcmg.2011.03.016
M3 - Article
C2 - 21679902
AN - SCOPUS:79958804438
VL - 4
SP - 659
EP - 670
JO - JACC: Cardiovascular Imaging
JF - JACC: Cardiovascular Imaging
SN - 1936-878X
IS - 6
ER -