Restrictive cardiomyopathy - cardiac amyloidosis

Case contributed by Dr Gregor Savli

Presentation

This patient was on treatment for multiple myeloma, when she started to suffer from progressive dyspnoea.

Patient Data

Age: 55
Gender: Female

All four cardiac chambers have thickened walls, including the atria and atrial septum, which alone is very suggestive of amyloidosis, even without other findings.

The hallmarks of restrictive cardiomyopathy are thickened cardiac walls (due to infiltration) and restricted diastolic filling of the ventricles (we usually look at left ventricle closer) with lowered EF (as opposed to unaltered or even increased EF with hypertrophic CMPs).

However, usually at least some late enhancement at cardiac amyloidosis can be seen, and this patient was no exception. Late enhancement was seen ranging from smaller sub-endocardial and mid-myocardial patches to a transmural larger patch of LE in the basal part of the heart.

Additional findigs are pericardial and pleural effusions.

Case Discussion

This patient had (almost?) exclusively cardiac AL amyloidosis and underwent further treatment with stem cell transplantation.

Eight months after this MR was performed, she is still alive and feeling quite well, considering everything the patient has endured.

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Case Information

rID: 25137
Case created: 7th Oct 2013
Last edited: 28th Nov 2015
Inclusion in quiz mode: Included

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