Transthyretin amyloidosis cardiomyopathy in Greece: Clinical insights from the National Referral Center.

Background: Clinical characteristics and outcomes of patients with transthyretin amyloidosis cardiomyopathy (ATTR-CM) vary by region, necessitating the acquisition of country-specific evidence for proper management.
Methods: This is an observational study including sequential patients presenting in the Amyloidosis Reference Center of Greece, from 01/2014 to 12/2022. ATTR-CM was diagnosed by positive scintigraphy and exclusion of light-chain amyloidosis or positive biopsy typing. Genetic testing was performed in all cases.
Results: In total, 109 ATTR-CM patients were included (median age, 81 years) of which 15 carried TTR mutations (27% Val30Met). Most patients (82%) presented with heart failure and 59% with atrial fibrillation, while 10% had aortic stenosis. Importantly, 78 (71.6%) had clinically significant extracardiac manifestations (45% musculoskeletal disorder, 40% peripheral neuropathy, and 33% gastrointestinal symptoms). Sixty-five (60%) received disease-specific treatment with tafamidis. Estimated median survival was 48 months; advanced NYHA class, National Amyloidosis Center stage, eGFR<45 ml/kg/1.73 m ² , NT-pro-BNP>5000 pg/mL were associated with worse survival, while tafamidis treatment was associated with improved survival in patients with IVS≥ 12 mm.
Discussion: These are the first data describing the characteristics, management, and outcomes of patients with ATTR-CM in Greece, which could influence local guidelines.
Competing Interests: Declaration of competing interest GG has received research support from Pfizer. KS has received research support from Pfizer. EK has received honoraria from Amgen, Genesis Pharma, Janssen, Takeda, Pfizer, GSK, and research support from Amgen, Janssen, and Pfizer. The rest of authors declare no conflict of interest.
(Copyright © 2023 Hellenic Society of Cardiology. Published by Elsevier Inc. All rights reserved.)