Amyloid disease of the heart

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Submitted by Dr T on August 14, 2011 – 3:57pm

Question: 
White 50 yo male.  6’2″ tall 174 lbs.  Currently experiencing ongoing shortness of breath and dizziness during exercise.  No previous history of heart disease, diabetes, cardiovascular disease. Summary from Echo:
1. Severe left atrial enlargement and mild to moderate right artial enlargement2. Normal LV size with mild concentric hypertrophy and normal systolic function EF 75%3. Normal diastolic function4.Mild upper normal RV size with preserved systolic function5. Trivial panvalvular regurgitation.6.  No indication of endocarditis prophylaxis.
Need to exclude amyloidosis. Performed a lung function test and it was normal. I am currently performing a 24 hour urine test to rule out amyloidosis. No other underlying causes have been discovered, such as sleep apnenia. What are other underlying factors would lead to this condition?

You are in the middle of a diagnostic evaluation, thus I can only comment on amyloidosis, an abnormal protein usually produced by cells in your bone marrow that can be deposited in any tissue or organ.

Cardiac amyloidosis (“stiff heart syndrome”) occurs when amyloid deposits take the place of normal heart muscle, and is a cause of what is called a restrictive cardiomyopathy. However, a number of tests are not consistent with that diagnosis at this point (unless this is very early in the disease process). Severe left atrial enlargement can occur in association with hypertensionaortic stenosis, mitral incompetence, mitral stenosis and hypertrophic cardiomyopathy.

Cardiac ECHO of a patient with Amyloid disease, showing a severe restrictive cardiomyopathy. LA, RA normal sizel LV, RV walls severely thickned.

An Echocardiogram of Severe left atrial enlargement and mitral stenosis:

 

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