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Transthyretin Amyloid Cardiomyopathy Opportunistic Diagnosis: Where’s Wally?
Session:
Painel 10 - Doença Valvular 6
Speaker:
Bruno M. Rocha
Congress:
CPC 2020
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.3 Myocardial Disease – Diagnostic Methods
Session Type:
Posters
FP Number:
---
Authors:
Bruno M. Rocha; Andreia Marques; Gonçalo Lopes Da Cunha; Pedro Lopes ; Fernando Abreu; Catarina Brízido; Christopher Strong; Maria João Andrade; Carlos Aguiar; Sophia Pintão; Miguel Mendes
Abstract
<p><strong>Background: </strong>Transthyretin Amyloid Cardiomyopathy (ATTR-CM) is an underdiagnosed though treatable cause of Heart Failure (HF). We aimed to determine the prevalence of incidental <sup>99m</sup>Tc-HMDP myocardial uptake in patients undergoing bone scintigraphy for a non-cardiac reason.</p> <p><strong>Methods: </strong>We conducted a single-center prospective study enrolling patients without previously known HF who consecutively underwent <sup>99m</sup>Tc-HMDP bone scintigraphy for a non-cardiac reason, throughout the year 2019. Images were independently assessed by two experienced analysts blinded to patient data. Those who had myocardial radiotracer uptake Perugini grade 1-3 were identified and screened for ATTR-CM.</p> <p><strong>Results:</strong> A total of 877 patients underwent bone scintigraphy in our center, of whom 24 (2.74%) for suspected ATTR-CM (1 with grade 1 and 9 with grade 2-3 Perugini). The latter were excluded from further analysis. Thus, 853 patients (mean age 69 ± 13 years; 49.9% male; 91.7% referred for oncologic staging or surveillance) were included. There were 8 (0.94%) patients with the incidental finding of myocardial radiotracer uptake, 5 of whom with grade 1 and 3 with grade 2 Perugini. All had AL amyloidosis thoroughly excluded. Patients were ≥80 years of age, most (n=6) were male, with good functional status (e.g., n=6 had a Karnofsky Performance Status Scale ≥80), 3 with stage IV and 5 with stage I or cured cancer. Three patients had known lumbar stenosis and none had carpal tunnel syndrome. Three had NYHA ≥II HF symptoms and NT-proBNP >125pg/mL was identified in 7 cases (range: 334-4540pg/mL). All were in sinus rhythm, none had ECG criteria for left ventricular hypertrophy (i.e., Sokolow-Lyon index range: 10-22mm), and low-voltage (n=2) or 1<sup>st</sup> degree AV block (n=3) were additional ECG red flags. On echocardiography, interventricular basal septum was thickened in all cases (range: 13-19mm), while a few had apical sparing (n=3) or LVEF <55% (n=2). One has non-invasive ATTR-CM diagnostic criteria, 4 are undergoing additional assessment and 3 under Heart Team discussion.</p> <p><strong>Conclusions: </strong>Incidental myocardial <sup>99m</sup>Tc-HMDP uptake was observed in roughly 1% of the cohort referred for bone scintigraphy for a non-cardiac reason. Despite the low prevalence, our strategy opportunistically identified 8 ATTR-CM potential cases over 1 year. Given the competing oncologic prognosis, a case-by-case individualized decision for invasive testing and disease-modifying treatment should be carefully considered.</p>
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