P347 SHORT–TERM EFFECTIVENESS OF TAFAMIDIS THERAPY IN PATIENTS WITH TRANSTYRETIN CARDIAC AMYLOIDOSIS: EVALUATION OF CLINICAL–INSTRUMENTAL AND BIOHUMORAL PARAMETERS

Abstract Background In the ATTRACT trial, Tafamidis has been shown to be effective in patients with transthyretin amyloidosis (ATTR) by improving quality of life and survival at 30–month follow–up. However, it‘s not clear which are the clinical–instrumental and bio–humoral parameters that change first and after how long time from the begging of treatment. Methods In our cardiomyopathy tertially referral center from January 2022 to October 2022, 47 patients with ATTR (73% males, 27% females, mean age 75±16 years) were admitted for the evaluation of the possible TTR treatment. Of these,30 received treatment with Tafamidis and 10 (M, mean age 77±11 years, 9ATTRwt, 1vATTR) completed a six months follow–up and constituted our patient population. The following parameters werecollected at baseline and after six–months follow–up: 6 minute walking test, Kansas score, NYHA class, NT–pro BNP,echocardiographic ejection fraction, 12 lead ECG with assessment of amplitude of QRS voltages, heart rhythm, presence of Q waves of pseudo necrosis, number of ventricular/supraventricular ectopic beats, presence of atrioventricular (AV) blocks, sinus blocks, hemiblocks, bundle branch blocks, QTc length, type and dose of additional medications. Results No patient changed the antiarrhythmic therapy during the six months follow–up, and no ventricular arrhythmias nor sinoatrial blocks were recorded. Two patients had pacemaker (PM) before the beginning of thetherapy and no PM was implanted during the follow–up. There was no significant difference in QRS voltage amplitude (p=0.157), atrial fibrillation (p=0.785), Q waves of pseudonecrosis (p= 1.000), ectopic beats (p= 1.000), AV blocks (p= 1.000), hemiblocks (p= 1.000), bundle branch blocks (p= 1.000), QTc (p= 0.813), ejection fraction (p= 0.317), NYHA class (p= 0.564), NTproBNP (p=0.273). Statistically significant differences were found in the Kansas score (p=0.042) and in the 6 minute walking test (p= 0.018). Conclusions Tafamidis is able to improve quality of life andfunctional capacity in ATTR patients early in the course of the treatment. The stability of the other parameters, that tend to fast worsen in the natural course of the disease, demonstrates the ability of Tafamidis to halt and possibly revert amyloidosis.