Abstract
Abstract Introduction and aims Patients with amyloid cardiomyopathy (ATTR-CM) present with a restrictive phenotype characterized by decreased ventricular compliance and increased filling pressures, with progressive loss of atrial function and increased stiffness, which are associated with poor clinical outcomes. Tafamidis improves clinical outcomes and slows disease progression but its effects in left ventricle (LV) and atrium (LA) mechanics are still unclear, with recent studies (reference 1.) suggesting improvement of LA function (as assesses by LA strain [LAS]) in patients with sinus rythm (SR). Our study aimed to characterize the 1-year evolution of LV function and LA mechanics in ATTR-CM patients treated with tafamidis. Methods We prospectively evaluated a cohort of patients with ATTR-CM treated with tafamidis in a single-centre. Results Out of 41 patients treated, 13 were excluded due to incomplete follow-up or poor quality of echocardiographic images that precluded LAS analysis. A total of 28 patients (75% male) were included, with a mean age of 80 ± 10 years at the start of treatment and a median follow up of 14 months. 11 pts (39%) were in sinus rhythm (SR group) and 17 (61%) were not (non-SR group). At the start of the treatment with tafamidis, mean LV ejection fraction (LVEF) was 55.5 ± 11.4%, GLS was -10.7 ± 4.3%, E/e’ 17.2 ± 5.4 and indexed LA volume (LAVi) 47.4 ± 15.3 mL/m2. Median NTproBNP was 2031 (3143) pg/mL and pts walked 313 ± 121m on the 6-minute walk test (6MWT). Regarding LAS, there was significant reduction in all atrial functional components in both SR (LAS reservoir 13.1 ± 12, LAS conduit -6.5 ± 4.2, LA contraction -6.6 ± 3.6) and non-SR groups (LAS reservoir 5.1 ± 3.6) as well as significantly increased LA stiffness (SR – 1.4 ± 0.9, non-SR – 6.1 ±6.0). Our analysis showed stabilization, with no statistically significant differences in LVEF, GLS, diastolic function parameters, LAS or LA stiffness, as well as NTproBNP and 6MWT after 1-year treatment with tafamidis (tables 1 and 2). We found a moderate correlation between LAS contraction and NTproBNP in the SR group (r=0.650, p=0.031) – meaning that worse (less negative) LAS contraction associated with higher NTproBNP values – despite there being no correlation between other echocardiographic variables ante NTproBNP or 6MWT. Conclusions In our cohort of patients, patients presented with significantly reduced atrial function and increased stiffness. 12-month treatment with tafamidis allowed stabilization of LA and LV performance, as well as of NTproBNP and 6MWT. We found a moderate correlation between LA contraction objectively evaluated by strain analysis and NTproBNP in SR patients, highlighting the importance of LA mechanical contraction preservation in patients with a restrictive cardiomyopathy phenotype.
Published Version
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call