This case is similar to the first case published by Dr Gharzuddine in this website with emphasis on the use of strain imaging in making the diagnosis of cardiac amyloidosis.
An abdominal fat biopsy done confirmed this diagnosis of Amyloidosis.
The echocardiographic findings were well described in the case of Dr Gharzuddine, in his comments he added that “Recently an interesting finding has been noted on evaluation of longitudinal strain of the left ventricle with demonstration of a pattern of relative sparing of the apex from the decreased strain noted in basal segments. This appears to be highly specific for amyloidosis “.
The Bull’s eye of the longitudinal speckle tracking is a clear illustration of this statement (figure 1). It showed a high level of strain in the apex (> 20%) with a decrease of the strain in the basal segments.
To make the principle easier to understand another image of longidinal strain in graphic form is shown below (figure 2). This is from another patient with proven amyloidosis. In the right upper corner the curves of the basal egments (red and yellow) show a significantly decreased strain compared to the apical segments (magenta and green) with the mid-ventricular segment values in between.
The first description of this finding was done by the Cleveland clinic team (Phelan et al.). The conclusion of this study was: Cardiac Amyloidosis is characterized by regional variations in Longitudinal Strain from base to apex. A relative 'apical sparing' pattern of Longitudinal Strain is an easily recognizable, accurate and reproducible method of differentiating Cardiac Amyloidosis from other causes of LV hypertrophy.
The sensitivity and specificity are 93 % and 82% in differentiating Cardiac amyloidosis from other forms of left ventricular hypertrophy.
We recommend doing a longitudinal strain analysis by the speckle tracking technique in all patients with cardiac hypertrophy. It is easy to do; fast, reproducible and can help in the diagnosis of the etiology of the hypertrophy.
Reference: Phelan et al. Heart 2012; 98:1442e1448. Relative apical sparing of longitudinal strain in cardiac amyloidosis.