Journal Article Epublish
PubMed: 42938212 DOI: 10.3390/diagnostics16203373
Survival Outcomes in Transthyretin Cardiac Amyloidosis in the Tafamidis Era: Real-World Evidence from Greece.
Diagnostics (Basel) 16(20)
Authors: Freideriki Eleni Kourti, Alexios S Antonopoulos, Vasileios Kytinos, Theodoros Tsampras, Alexandrina Kouroutzoglou, Dimitrios Kyriakopoulos, Maria Eleni Koilakou, Pinelopi Karampina, Vasiliki Gardikioti, Dimitrios Terentes-Printzios, Alexandros Kasiakogias, Ioannis Leontsinis, Christina Chrysohoou, Maria Koutelou, Konstantinos Toutouzas, Konstantinos Tsioufis, Charalambos Vlachopoulos
Affiliations
- Cardiogenetics Unit, National Cardiac Amyloidosis Centre, 1st Cardiology Department, Hippokration Hospital, National and Kapodistrian University of Athens, 11527 Athens, Greece.
- Department of Nuclear Medicine, Kosmoiatriki, 11254 Athens, Greece.
- Department of Nuclear Medicine, Bioiatriki, 11526 Athens, Greece.
Abstract
Background: Diagnoses of transthyretin amyloid cardiomyopathy (ATTR-CM) have increased in recent years, with a growing proportion of patients being identified at earlier disease stages. However, temporal trends in ATTR-CM diagnosis and survival during the tafamidis era in the Greek population remain largely unknown. Methods: This retrospective study included 75 patients with ATTR-CM treated with tafamidis. Baseline demographic, clinical, laboratory, and echocardiographic characteristics were assessed, and survival outcomes were subsequently evaluated. Results: Among 75 tafamidis-treated patients, 14 deaths (19%) occurred during a median follow-up of 20 months [IQR 10.0-30.5] with an estimated 30-month survival of 75%. Kaplan-Meier analysis demonstrated significantly poorer survival among patients aged ≥80 years (log-rank p = 0.013), those with NYHA class III-IV (log-rank p = 0.018), and across progressively more advanced NAC stages I-III (global log-rank p < 0.001). In contrast, survival did not differ significantly according to LVEF <50% versus ≥50% (log-rank p = 0.863) or maximal wall thickness ≥15 mm versus <15 mm (log-rank p = 0.801). After Holm correction across five comparisons, only NAC stage remained statistically significant (adjusted p < 0.001). Conclusions: In this real-world Greek cohort of patients with ATTR-CM treated with tafamidis, the estimated 30-month all-cause mortality was 25% and more advanced NAC stage was associated with poorer survival.
MeSH
Prealbumin, Amyloidosis, Diagnosis, Transthyretin Amyloid Cardiomyopathy, Transthyretin Cardiac Amyloidosis