Author + information
- Received March 25, 2011
- Accepted March 28, 2011
- Published online June 1, 2011.
- Claudio Rapezzi, MD⁎,⁎ (, )
- Candida C. Quarta, MD⁎,
- Pier Luigi Guidalotti, MD†,
- Cinzia Pettinato, MD†,
- Stefano Fanti, MD†,
- Ornella Leone, MD‡,
- Alessandra Ferlini, MD§,
- Simone Longhi, MD⁎,
- Massimiliano Lorenzini, MD⁎,
- Letizia Bacchi Reggiani, MSc, MStat⁎,
- Christian Gagliardi, MD⁎,
- Pamela Gallo, MD⁎,
- Caterina Villani, MD⁎ and
- Fabrizio Salvi, MD∥
- ↵⁎Reprint requests and correspondence:
Prof. Claudio Rapezzi, Istituto di Cardiologia, Policlinico S. Orsola-Malpighi, via Massarenti 9, 40138 Bologna, Italy
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 ≥2). Within the subgroup without AC, only 4 patients (with Ala36Pro, Gly47Ala, Thr49Ala, and Glu89Gln transthyretin mutations) showed myocardial tracer uptake and abnormal heart/whole body retention (H/WB) values: in all these cases endomyocardial biopsies showed amyloidotic infiltration. The H/WB was positively correlated with left ventricular (LV) mean wall thickness (Pearson's r = 0.695, p < 0.001) and negatively with LV ejection fraction (r = −0.368, p = 0.004). The H/WB was an unfavorable predictor of MACE-free survival at Cox univariate analysis and contributed to the multivariate model. Notably, LV wall thickness >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.
- 99mTc-DPD scintigraphy
- cardiac amyloidosis
- hereditary transthyretin-related amyloidosis
All authors have reported that they have no relationships to disclose.
- Received March 25, 2011.
- Accepted March 28, 2011.
- American College of Cardiology Foundation