Amyloidosis

Amyloidosis is caused by an accumulation of abnormal protein, called amyloid deposits. If these build up in your body they can cause problems, depending on where in your body they build up.

The heart and the kidneys are some of the organs which are most often affected. In severe cases it can lead to failure of the organ and death.

Cardiac amyloidosis results in a restrictive cardiomyopathy caused by extracellular deposition of proteins in the myocardium.

The proteins have an unstable structure that causes them to misfold, aggregate, and deposit as amyloid fibrils.

Possible findings on transthoracic echocardiography include:

• Thickening of the left ventricle.

• Granular or sparkling appearance of the myocardium secondary to amyloid deposition in the myocardium.

• Diastolic dysfunction

• Normal or reduced LV cavity size

• Pericardial effusion is often present but is usually small

• Right ventricular hypertrophy

• Left atrial enlargement

• Thickened AV valves

• Dissociation between short and long axis systolic function.