Restrictive cardiomyopathy - cardiac amyloidosis
This patient was on treatment for multiple myeloma, when she started to suffer from progressive dyspnoea.
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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.
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.
- vanden Driesen RI, Slaughter RE, Strugnell WE. MR findings in cardiac amyloidosis. AJR Am J Roentgenol. 2006;186 (6): 1682-5. doi:10.2214/AJR.04.0871 - Pubmed citation
- Cummings KW, Bhalla S, Javidan-Nejad C et-al. A pattern-based approach to assessment of delayed enhancement in nonischemic cardiomyopathy at MR imaging. Radiographics. 2009;29 (1): 89-103. Radiographics (full text) - doi:10.1148/rg.291085052 - Pubmed citation
- Seeger A, Klumpp B, Kramer U et-al. MRI assessment of cardiac amyloidosis: experience of six cases with review of the current literature. Br J Radiol. 2009;82 (976): 337-42. Br J Radiol (full text) - doi:10.1259/bjr/76104551 - Pubmed citation