Cardiac amyloidosis

Discussion:

This case illustrates MR imaging features of cardiac amyloidosis in a patient with known AL amyloidosis.

Left and right ventricular hypertrophy, thickened atrial walls, abnormal blood-pool gadolinium kinetics and the non-coronary distribution of the findings support the diagnosis of cardiac amyloidosis 1-4 and speak against myocardial scar tissue or findings in the context of coronary artery disease.

The relatively mild increase in interventricular septum thickness, the still normal left ventricular mass, the extracellular volume of 46-52%, as opposed to higher values, and foremost the association with nephrotic syndrome are features that would favor AL over ATTR amyloidosis 5.

Evidence of myocardial edema, seen on T2 mapping and STIR images indicates a less favorable prognosis 6.

Immediately after the diagnosis of cardiac amyloidosis, the patient was also diagnosed with smoldering multiple myeloma after undergoing a bone marrow biopsy. He was treated accordingly with chemotherapy and immunotherapy.

A follow-up examination 8 months later shows mild improvement of the abnormal T1 and T2 values and extracellular volume under therapy.

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