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A. Basics
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07. Syncope and Bradycardia
08. Ventricular Arrhythmias and Sudden Cardiac Death (SCD)
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31. Pharmacology and Pharmacotherapy
32. Cardiovascular Nursing
33. e-Cardiology / Digital Health
34. Public Health and Health Economics
35. Research Methodology
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Transthyretin Cardiac Amyloidosis: insights from a tertiary hospital
Session:
Painel 9 - Doença Valvular 7
Speaker:
Pedro M. Lopes
Congress:
CPC 2020
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
17. Myocardial Disease
Subtheme:
17.6 Myocardial Disease – Clinical
Session Type:
Posters
FP Number:
---
Authors:
Pedro M. Lopes; Bruno ML Rocha; Gonçalo Lopes Da Cunha; Christopher Strong; Andreia Marques; Fernando Abreu; Sophia Pintão; Maria João Andrade; António Miguel Ferreira; Carlos Aguiar; Miguel Mendes
Abstract
<p><strong>Background: </strong>Transthyretin amyloid cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) in older adults. Advances in non-invasive diagnosis coupled with demonstration of efficacy of specific therapies has been shifting that paradigm. The aim of this study was to describe the main clinical characteristics of our cohort of patients with ATTR-CM.</p> <p> </p> <p><strong>Methods: </strong>Single-centre prospective registry enrolling consecutive patients with ATTR-CM diagnosed since 2019. Demographic, clinical, electrocardiographic (ECG), echocardiographic (TTE) and treatment data were assessed. ATTR-CM was diagnosed histologically or non-invasively (i.e., 99mTc-HMDP bone scan with Perugini grade 2 or 3). Patients with light-chain amyloidosis were excluded from analysis.</p> <p> </p> <p><strong>Results:</strong> Overall, 18 patients (mean age 81 ± 5.7 years-old, 77.8% males, 66.7% hypertensive; 44.4% NYHA II) were included. Of these, 13 patients were diagnosed non-invasively and 5 invasively. The clinical profile leading to ATTR-CM diagnosis was HF in most of the cases (n = 15, 83.3%). Nine patients were previously misdiagnosed (e.g., “hypertensive cardiopathy” in 6 cases). Severe aortic stenosis was concomitantly diagnosed in 2 patients. Carpal tunnel syndrome was the most common extra-cardiac associated feature (n=3, 16.7%). There were no cases of monoclonal gammopathy of undetermined significance. Atrial fibrillation (61.1%) and pseudo-infarct pattern (n=7, 38.9%) were the most common ECG findings. Six patients (33.3%) had low-voltage criteria, while one showed left ventricular hypertrophy criteria on ECG. Two patients developed symptomatic bradyarrhythmia, requiring permanent pacemaker implantation. On TTE, mean left ventricular ejection fraction was 45.2 ± 13.8% and global longitudinal strain -8.3 ± 1.9%, having observed an apical sparing pattern in 13 (72.2%) patients. Mean interventricular septum thickness was 20.9 ± 5.5mm. Pericardial effusion was present in 50%. CMR were performed in 6 patients, corroborating TTE and HDMP-scan findings in all cases. Furthermore, 7 patients were started on tafamidis 61mg as compassionate therapy (Table 1).</p> <p> </p> <p><strong>Conclusions:</strong> Over the course of one year, 18 patients were diagnosed with ATTR-CM patients in our center. A considerable number of cases were in the initial stages of the disease, and accordingly were started on disease-modifying treatment, as per ATTR-ACT trial criteria.</p>
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