PMC4246150_01
A four-chamber apical view echocardiogram showing bi-atrial dilatation, valve thickening, thick ventricular walls, and interventricular septum with speckled appearance.
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A recent echocardiogram demonstrated a drop in ejection fraction to 30% with global hypokinesis, with marked echogenicity of the LV myocardium (Fig. 1).
(A-D) Inversion sequence trying to determine optimal inversion time to assess for late gadolinium enhancement. Inversion time (TI)=97.5, 125, 152.5, and 180 ms, respectively. There is no optimal delineation of myocardial nulling relative to the myocardial blood pool. (C) TI of 152.5 demonstrate areas of increased signal within the myocardium which have increased relaxivity related to the blood pool which is opposite normal relaxation parameters and suggestive of diffuse infiltration of the myocardium, characteristic of amyloidosis.
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Congo red staining and the presence of apple green birefringence under polarized light. Cardiac MRI demonstrated diffuse LV hypertrophy and significant infiltrative process involving the entire myocardium (Figs. 2 and 3).
Inversion recovery images demonstrating late gadolinium enhancement throughout the myocardium. Diffuse infiltration of myocardium with amyloid results in technical difficulty in myocardial nulling. (A) In this case, abnormal enhancement of the papillary muscle (arrow) acts as a reference to normal myocardial relaxation time on magnitude images. (B) Subsequent phase sensitive inversion recovery images demonstrate the extent of myocardial abnormality.
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Congo red staining and the presence of apple green birefringence under polarized light. Cardiac MRI demonstrated diffuse LV hypertrophy and significant infiltrative process involving the entire myocardium (Figs. 2 and 3).