Echocardiographic changes after treatment with tafamidis in patients with TTR cardiac amyloidosis

Abstract Background Tafamidis is currently the only approved drug for the treatment of ATTR cardiomyopathy in order to reduce HF hospitalization and mortality. However, real world data on disease progression of patients undergoing this therapy are scarce. We sought to evaluate changes in echocardiographic parameters at 1—year follow-up. Methods We retrospectively evaluated consecutive patients diagnosed with ATTR-CA from 2018 to 2023 at 11 centers in Italy. Results The cohort included 520 patients from 11 centers, of whom 427 (82%) on tafamidis. The median age was 79 years (IQR 75 – 83), 464 (89%) were males and most of the patients presented with mild symptoms at baseline (14% NYHA I, 76% NYHA II). 1-year echocardiographic follow-up was available in 337 patients (278 on tafamidis and 59 not treated). Patients treated with tafamidis showed stable echocardiographic parameters, including left ventricular volumes, diameter, thickness and systolic (Baseline LVEF 51%, IQR 44 – 59; 1-year LVEF 52%, IQR 45 – 57) and diastolic function (Baseline E/e’ 15, IQR 11 – 20; 1-year E/e’ 15, IQR 12 – 19) as well as right ventricular dimension and function. Speckle tracking echocardiography showed stable left ventricular global longitudinal strain, right ventricular free wall longitudinal strain and left atrial strain. When compared with patients not treated, tafamidis treatment showed to slow significantly the decline of left ventricular global longitudinal strain (median change 0, IQR -1,7 - + 2,1), while the ones who did not receive the drug had a decline of the same index (median change +3,5, IQR 0,5 – 5,2, p-value