Evolution and prognostic value of deformations parameters and myocardial work in transthyretin amyloid cardiomyopathy

Abstract

Wild-type transthyretin amyloid cardiomyopathy (ATTR-CM) is an increasingly recognized condition. Prognosis is related to the early diagnosis and treatment. It remains clinically challenging to estimate the risk for many patients at the time of the diagnosis. Myocardial work is a novel echocardiographic tool, that improve characterization and prognostic value of the myocardial damages. We sought to analyze echocardiographic deformation parameters, their evolution, and to evaluate their incremental prognostic value, in ATTR-CM patients. We retrospectively analyzed clinical, electrocardiographic, biological, and echocardiographic characteristics in 113 consecutive patients (median age 82 [77–85], 90,4% men) diagnosed for a wild-type ATTR-CM based on international consensus. Baseline and 18 months follow-up evaluation were taken into account. All the data came from a single center and all the images were recorded on the same platform (Ve95, GE-healthcare). Eighty-six patients were treated with Tafamidis. Thirty-four patients died and twelve were hospitalized for heart failure at a median follow-up of 935 days (IQR 691–1159) days. Right ventricle strain (RVS), left atrial strain during contraction phase (LASct), global and basal longitudinal strain, Global Work Index (GWI) and TAPSE significantly decreased from baseline to 18 months follow-up. Left ventricular ejection fraction (LVEF), global wasted work (GWW) and global work efficiency (GWE) did not differ significantly. Using a multivariate analysis, two parameters could be highlighted for their prognostic value on baseline evaluation: GWI < 1347 mmHg and a RV end-diastole area of > 19 cm2 were significantly associated with the risk of death and/or hospitalization for heart failure. Treated patients with Tafamidis had a significantly better survival. Myocardial work, and particularly GWI might be consider at baseline as well as RV size to best assess the risk of major events in ATTR-CM patients.