Value of Longitudinal Strain to Identify Wild-Type Transthyretin Amyloidosis in Patients With Aortic Stenosis

Abstract

Background: Wild-type transthyretin-related amyloidosis (ATTRwt) and degenerative aortic stenosis (AS) are both age-related. Diagnosis of cardiac amyloidosis (CA) among patients with AS may be difficult due to overlapping morphological and functional criteria. The aim of this study was to describe an echocardiographic longitudinal strain (LS) pattern among patients with AS with and without ATTRwt. Methods and Results:Patients who have AS with ATTRwt (n=30), AS without ATTRwt (n=50) and ATTRwt without AS (n=31) underwent two-dimensional speckle-tracking echocardiography. Transthyretin CA was based on positive bone scintigraphy without monoclonal gammopathy. All patients showed a gradual decrease in LS from the base to the apex resulting in a decrease of the global LS. A cut-off value of 1.0 for relative apical LS (average apical LS/[average basal LS+mid-LS]) was sensitive (88%) but less specific (68%) in differentiating ATTRwt among patients with severe AS. The best cut-off value for relative apical LS for identifying patients with ATTRwt among the whole population was 0.9 (sensitivity 74%, specificity 66%); however, 35%, 25% and 11% of patients who have ATTRwt without AS, with moderate AS and with severe AS, respectively, did not reach this threshold. Conclusions:A decrease of global and relative apical LS is common in patients with AS, even in the absence of ATTRwt. ATTRwt CA can be present even in the absence of relative apical sparing of LS.

Prevalence and significance of relative apical sparing in aortic stenosis: insights from an echo and cardiovascular magnetic resonance study of patients referred for surgical aortic valve replacement

“…Anyway, the association of RASP with indexes of more severe valvular disease, related LV dysfunction and global heart repercussion, is in line with previous published data. 9,13 We further demonstrated that RASP in these patients with severe AS occurs in the absence of CA, which is accordance with what was demonstrated by Robin et al 21 1 and 2. *P-values after adjustment for atrial fibrillation, using parametric and non-parametric ANCOVA.…”

Multimodality imaging in the diagnostic management of concomitant aortic stenosis and transthyretin-related wild-type cardiac amyloidosis

“…GLS impairment in ATTRwt-CA has a characteristic pattern called “apical sparing,” which refers to the relative reduction of the myocardial deformation of the basal segments compared with the apex ( 23 ). The coexistence of severe AS and ATTRwt-CA is associated with an early decrease of GLS: a value of −14% is sensitive but not specific for identifying ATTRwt-CA in patients with AS [area under the curve (AUC) 0.75] ( 24 ). Moreover, the apical sparing pattern is both sensitive and specific for the diagnosis of ATTRwt-CA in patients with increased left ventricular wall thickness ( 25 ).…”

“…The pattern apical sparing in patients with both AS and ATTRwt-CA has good accuracy (sensitivity 88%, specificity 68%, AUC 0.73) to diagnose ATTRwt-CA in patients with severe AS. Since the presence of apical sparing is common in AS even in the absence of ATTRwt-CA, it should be considered in combination with other echocardiographic parameters to raise the suspicion of ATTRwt-CA disease ( 24 ). On the other hand, AS is responsible for elevated wall stress and increased afterload that may mask the presence of apical sparing ( 10 , 26 ).…”

Current and novel echocardiographic assessment of left ventricular systolic function in aortic stenosis—A comprehensive review

“…73 The classical appearance of cardiac amyloidosis without AS is a reduction in strain values at the basal to mid segments consistent with the progressive myocardial infiltration with "apical sparing" which is a specific pattern associated with cardiac amyloidosis. 74 However, a recent study by Robin et al 75 evaluating a sub-type of cardiac amyloid determined that an increase in GLS with apical sparing is common in AS patients even without the presence of amyloidosis. It is suspected that afterload mismatch attributed to AS conceals the typical apical sparing pattern, reducing the clinical usefulness of GLS at identifying cardiac amyloidosis until afterload is removed following intervention.…”

“… 74 However, a recent study by Robin et al. 75 evaluating a sub‐type of cardiac amyloid determined that an increase in GLS with apical sparing is common in AS patients even without the presence of amyloidosis. It is suspected that afterload mismatch attributed to AS conceals the typical apical sparing pattern, reducing the clinical usefulness of GLS at identifying cardiac amyloidosis until afterload is removed following intervention.…”