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Transthyretin Amyloid Cardiomyopathy: a 2-year single-centre experience
Session:
Comunicações Orais (Sessão 26) - Insuficiência Cardíaca 4 - Vários Tópicos
Speaker:
Bruno M. Rocha
Congress:
CPC 2022
Topic:
D. Heart Failure
Theme:
10. Chronic Heart Failure
Subtheme:
10.6 Chronic Heart Failure - Clinical
Session Type:
Comunicações Orais
FP Number:
---
Authors:
Bruno m. Rocha; Rita Amador; Sérgio Maltês; Andreia Marques; Catarina Oliveira; Pedro Lopes; Gonçalo Cunha; Mariana Paiva; Christopher Strong; Fernando Abreu; Sophia Pintão; Carlos Aguiar; Miguel Mendes
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,"sans-serif""><u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Background</span></span></u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">: Transthyretin Amyloid Cardiomyopathy (ATTR-CM) is an under-diagnosed condition often presenting with Heart Failure (HF). We aimed to assess a cohort of patients with ATTR-CM and HF, focusing</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">on the</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">centre</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">strategies</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">to identify new cases, prognosticate</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">and tailor treatment.</span></span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,"sans-serif""><u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Methods</span></span></u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">: We conducted an all-comers single-centre prospective registry of consecutive patients with HF due to ATTR-CM followed in our centre from November 2019 to 2021. As per site protocol, </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">diagnosis is established according to the algorithm by Gilmore et al. and </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">all patients are assessed in our HF outpatient clinic at least twice yearly with systematic electronic chart data collection. </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">We evaluated disease-modifying treatment and compliance with the current European Guidelines and CHAD-STOP management. A summary of this program is presented in the <strong><span style="color:#0070c0">central figure</span></strong>.</span></span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,"sans-serif""><u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Results</span></span></u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">: Overall, 60 patients were included (mean age 83 ± 7 years; 80% male). ATTR-CM was confirmed by the non-invasive algorithm in all but 8 patients, in whom endomyocardial biopsy was positive.</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Of those undergoing genetic testing (n=30),</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">7 (23%) presented with the hereditary form of ATTR-CM (4 Val50Met and 3 Val142Ile mutations). The initial presentation was</span></span> <span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">most often HF (n=43), atrial fibrillation (n=9), or “incidental” myocardial uptake on </span></span><sup><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">99m</span></span></sup><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Tc-HMDP bone scintigraphy </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">(</span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">grade </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">2-3) </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">performed for cancer staging (n=5). </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Beta-blockers were reduced or stopped in 40 (67%) patients, all of whom improved in NYHA class and/or NT-proBNP (>30% reduction) at 1-3 months. T</span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">afamidis 61mg was started in 22 patients and 15 more currently await approval. Those</span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif""> initiated on or referred to tafamidis 61mg (n=37) had less severe HF, as per NYHA (class I-II – 94 vs. 50%, p=0,033) and performance status (e.g. Karnofsky score 80-100 – 79 vs. 21%, p=0,010). Of those already on tafamidis (n=22), NYHA class remained stable or improved in all but 1 patient. In the year following vs. preceding treatment there was 2 vs. 3 total HF hospitalizations. No drug-related severe adverse events were reported. Over a 2-year follow-up, 14 (23.3%) patients died, of whom 1 was on tafamidis (compassionate use for 19 months). </span></span></span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,"sans-serif""><u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">Conclusions</span></span></u><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">: ATTR-CM recognition is improving in our </span></span><span style="font-size:10.0pt"><span style="font-family:"Arial","sans-serif"">dedicated rare disease program, possibly due to the implementation of several alert pathways. The identification of the disease at an earlier stage allows targeted treatment, compliant with the recommendations. Nonetheless, the rarity of this disease and the required expertise for its optimal management argues in favour of a national strategic plan based on referral centres for ATTR-CM.</span></span></span></span></p>
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