Transthyretin Amyloidosis Cardiomyopathy in Greece: Clinical Insights from the National Referral Center.

Abstract

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:

One-hundred and nine 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/1.73m2, 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.

SHORT TITLE:

Transthyretin cardiomyopathy in Greece.

• Authors: Dimitrios Bampatsias, Foteini Theodorakakou, Alexandros Briasoulis, Georgios Georgiopoulos, Anna Dimoula, Vasileios Papantoniou, Ioannis Papantoniou, Chaido Skiadaresi, Pipitsa Valsamaki, Evangelos Repasos, Ioannis Petropoulos, Dimitrios Delialis, Alexandra Papathoma, Georgios Koutsis, Maria-Eirini Tselegkidi, Kimon Stamatelopoulos, Efstathios Kastritis
• Journal: Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese (Hellenic J Cardiol) (Oct 05 2023) ISSN: 2241-5955 [Electronic] Netherlands
• PMID: 37805173 (Publication Type: Journal Article)
• Copyright: Copyright © 2023 Hellenic Society of Cardiology. Published by Elsevier B.V. All rights reserved.