Amyloid Cardiomyopathy

 

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Amyloid cardiomyopathy is a restrictive infiltrative disease that results in severe LV diastolic dysfunction and moderate LV systolic dysfunction. Classically One may see is significant biatrial enlargement along with leaky valves and a pericardial effusion. Many of these features are shown in the off axis parasternal shot. The ventricles can both become quite thick, but the EKG will still show low volts. This individual had biopsy proven cardiac amyloid.

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The color Doppler shows there is both aortic and mitral valve regurgitation.

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Note how thick both ventricles are in this parasternal short axis at the level of the mid ventricle. There is a small pericardial effusion appreciated here as well. A similar Echo may be seen in someone with end stage renal disease on dialysis. Typically they will have significant LVH on EKG, however if their ventricle looks like this.

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This apical 4 chamber shows how massively dilated both atria are. They are similar in size to the ventricles. The RV is also enlarged and thickened. Both mitral and tricuspid annuli are enlarged. Look closely and you will see that in fact the tricuspid valve fails to properly coapt.
The mitral filling pattern is restrictive (highest grade of diastolic dysfunction) with a fast deceleration time and a small A wave.