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99mTC-DPD scintigraphy in the diagnosis and prognosis of transthyretin V30M cardiac amyloidosis
Session:
Comunicações Orais (Sessão 11) - Imagem 1 - TC e RM Cardíaca e Cardiologia Nuclear
Speaker:
Conceição Azevedo Coutinho
Congress:
CPC 2022
Topic:
B. Imaging
Theme:
03. Imaging
Subtheme:
03.4 Nuclear Imaging
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Conceição Azevedo Coutinho; Nuno Cortez-Dias; Guilhermina Cantinho; Susana Gonçalves; Nelson Cunha; Tiago Rodrigues; Laura Santos; Isabel Conceição; João Agostinho; Fausto Pinto
Abstract
<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><strong>Background:</strong> Early diagnosis and prognostic stratification of transthyretin amyloid cardiomyopathy (ATTR-CM) are crucial. Previous findings suggested that <sup>99m</sup>Tc-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scintigraphy has the potential to provide an earlier diagnosis of ATTR-CM but it seems to present suboptimal accuracy to detect ATTR-CM caused by V30M mutation, particularly in patients with onset of symptoms bellow 50 years of age. Furthermore, its prognostic value has never been evaluated in this mutation. </span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><strong>Objectives:</strong> To assess the diagnostic value of DPD scintigraphy to detect cardiomyopathy in a large cohort of patients with ATTR-V30M mutation and to explore its association with mortality. </span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><strong>Methods: </strong>Of the 305 ATTRV30M mutation carriers followed prospectively and who underwent DPD scintigraphy, 288 individuals [median age 46 (39-56); 49% male] without myocardial thickening attributable to other causes were enrolled in the study. ATTR-CM was defined by septal thickness ≥13mm plus at least one of the following criteria: (1) late heart-to-mediastinum (H/M) <sup>123</sup>I-metaiodobenzylguanidine (MIBG) ratio <1.60; (2) electrical heart disease (arrhythmia or cardiac conduction defect); or (3) amyloid infiltration documented in biopsy.</span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif"><strong>Results: </strong>ATTR-CM was identified in 41 (14.2%) patients and 34 (11.8%) individuals presented abnormal cardiac DPD uptake. A strong association was noted between cardiac DPD retention and the presence of amyloid cardiomyopathy. Cardiac DPD retention was associated with a 27-fold higher likelihood of having ATTR-CM (OR: 27.4; 95%CI 11.6-65.0; P <0.001). </span></span></p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:"Times New Roman",serif">During a mean follow-up of 33.6±1.2 months, 16 patients died (5.6%). Mortality was 14 times higher in patients with amyloid cardiomyopathy (HR: 14.1; 95%CI 4.9-40.7; P <0.001), 13 times higher in those with abnormal cardiac DPD uptake (HR: 12.59; 95% CI % 4.56-34.72; P <0.001) and 10 times higher in those with H/M MIBG ratio <1.60 (HR: 10.40; 95% CI 2.95-36.69; P <0.001). The prognostic value of ventricular thickness and cardiac DPD uptake was additive: patients without septal thickening and no cardiac DPD retention had excellent prognosis (5-year mortality of 0.75%), while those with septal thickening and/or abnormal DPD retention presented 5-year mortality rates ranging from 39.9 to 53.3%.</span></span></p> <p style="text-align:justify"><strong>Conclusions:</strong><span style="font-family:"Times New Roman",serif; font-size:12pt"> DPD scintigraphy is valuable in the evaluation of V30M mutation carriers, particularly for prognostic stratification purposes, identifying patients at higher risk of death.</span></p>
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