Effect of tafamidis on global longitudinal strain and myocardial work in transthyretin cardiac amyloidosis

Abstract

Aims In patients with transthyretin amyloid cardiomyopathy (ATTR-CM), the effect of tafamidis on myocardial function using serial speckle tracking echocardiography has not been reported. The purpose of this study was to describe the natural history of myocardial function in untreated ATTR-CM and determine the effect of tafamidis on myocardial functional parameters over 12 months of treatment. Methods and results A total of 45 subjects with ATTR-CM were retrospectively studied: 23 treated with tafamidis and 22 untreated. Two-dimensional speckle tracking echocardiography was analysed at baseline and 1 year. Serial longitudinal, circumferential, and radial strain, twist, torsion, and myocardial work were measured. Over 1 year, absolute global longitudinal strain (GLS) deteriorated more in the untreated group by a median of 1.1% [inter-quartile range (IQR) 0.95] compared with 0.3% (IQR 1) in the tafamidis group (P = 0.02). Myocardial work index and efficiency also deteriorated to a greater degree: 142.5 mmHg% (IQR 197) and 4% (IQR 8), respectively, in the untreated group compared with 61.5 mmHg% (IQR 210) and 1% (IQR 7) in the tafamidis group (P = 0.04). There were no significant between group differences in left ventricular ejection fraction (LVEF), tissue Doppler velocities, circumferential or radial strain, LV twist or torsion at 1 year. The stabilization effect of tafamidis on myocardial function at 1 year did not differ according to baseline GLS, LVEF, or National Amyloidosis Centre disease stage. Conclusions In ATTR-CM, tafamidis resulted in a lesser deterioration in GLS, myocardial work index, and efficiency over a 12-month period compared with a cohort not treated with tafamidis.

Changes in myocardial work associated with pediatric kidney transplantation: A pilot study of short‐term postoperative effect

“…Currently, MW has been used to assess the myocardial function in patients of hypertension, cardiomyopathy, and maintenance hemodialysis, 14 , 15 , 16 but studies about LV function evaluated by MW in children with KTx are few. Therefore, this study was mainly to evaluate the changes of LV MW in children with CKD stage 5 within 3 months after KTx.…”

Oral Therapy for the Treatment of Transthyretin-Related Amyloid Cardiomyopathy

“…A clinical benefit of tafamidis treatment, in terms of prolonged major cardiovascular outcome-free survival, lower deterioration of structural and functional changes of the left ventricle, and myocardial work, was also reported outside the frame of clinical trials, in retrospective series of real-life patients [119][120][121][122].…”

Reverse cardiac remodelling and dysfunction in A97S transthyretin cardiac amyloidosis after tafamidis treatment

“…Most reports on treatment groups have demonstrated less deteri-oration rate or stable clinical course compared with the non-treated group, whereas few demonstrated improvements in myocardial biomarkers. [3][4][5][6] In this case report, we present a case of hATTR-CM of A97S variant, an under-represented group in the current published data, with evidence of reverse cardiac remodelling demonstrated on multiple image modalities. This case report further demonstrated different treatment response of various variants of ATTR-CM.…”