Case Author(s): Thomas Vreeland, M.D. / Tom Miller, M.D., Ph.D. , 2/8/95 . Rating: #D2, #Q4

Diagnosis: Amyloidosis

Brief history:

78 year old man with heart failure

Images:

Anterior, left anterior oblique, and lateral views

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Full history/Diagnosis is available below


Diagnosis: Amyloidosis

Full history:

This 78 year-old man with congestive heart failure, atrial fibrillation and thrombocytopenia was referred for evaluation after an abnormal echocardiogram. Clinically, amyloidosis was suspected; however, the serum and urine electrophoresis were both negative for M-protein spikes.

Findings:

Diffuse myocardial uptake (greater than sternum) is seen on all three views on this Tc-99m pyrophosphate study.

Discussion:

Diffuse myocardial uptake (grade 3 or 4) will be seen in 50-90% of pyrophosphate scintigrams of the heart in patients with amyloidosis. (The grading system utilizes activity equal to the sternum as equal to 3.)

Classic Echocardiographic findings include

(1) "Speckling" of the thickened myocardial wall

(2) Hyperechoic valve leaflets

(3) Thickened atrial septa

Followup:

Multiple mucosal biopsies were negative for amyloidosis prior to the echocardiogram and pyrophosphate scintigram. Endomyocardial biopsy proved cardiac amyloidosis.

Major teaching point(s):

Myocardial infarction would be very unusual in this case because of the very large volume of involved myocardium.

Differential Diagnosis List

Myocarditis, pericarditis, metastatic calcification/hypercalcemia and diffuse infiltrative diseases (i.e. hemachromatosis)

ACR Codes and Keywords:

References and General Discussion of Myocardial Imaging (Anatomic field:Heart and Great Vessels, Category:Other generalized systemic disorder)

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Case number: mi005

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