Learning objectives
The purpose of this exhibit is
To demonstrate the characteristics and the various findings of cardiac magnetic resonance (CMR) images in patients with cardiac amyloidosis (CA).
To offer tips and state the pitfalls of CMR based on the patients with histo-pathologically proven CA in our institute between 2008 and 2012.
Background
General aspects about amyloidosis
Amyloidosis is a disease in which an insoluble amyloid protein fibril deposits mainly in the extracellular spaces of organs and tissues.
Amyloid fibril proteins are rigid,
non-branching fibrils approximately 10 nm in diameter.
The fibrils bind the dye Congo red and exhibit green birefringence when viewed by polarisation microscopy.
To date,
there are 27 known extracellular fibril proteins in humans (1).
There are two classifications of amyloidosis,
systemic and localized.
Definition of cardiac amyloidosis
Amyloid deposition in the myocardium,
conduction pathway,...
Imaging findings OR Procedure details
This educational exhibit shows
A review of typical CMR findings such as cine,
T2WI,
T2 map,
perfusion,
and late gadolinium enhancement (LGE).
This exhibit will especially focus on LGE.
Tips and pitfalls of CMR in acquisition and interpretation.
The techniques are described and illustrated.
Morphological finding
Concentric and symmetrical hypertrophy is frequent in CA in contrast to hypertrophic cardiomyopathy,
which presents asymmetrical hypertrophy.
Understanding morphological change helps to diagnose in CA when extra-cardiac histo-pathological evidence can be obtained by abdominal fat pad aspiration or duodenal...
Conclusion
LGE on CMR is a promising technique to diagnose CA because LGE is sensitive in detecting amyloid deposits within the myocardium.
In order to analyze and interpret the various CMR image findings,
radiologists must be able to precisely diagnose CA.
References
Sipe JD,
Benson MD,
Buxbaum JN,
Ikeda S,
Merlini G,
Saraiva MJ,
et al.
Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis.
Amyloid.
2010 Sep;17(3-4):101-4.
Gertz MA,
Comenzo R,
Falk RH,
Fermand JP,
Hazenberg BP,
Hawkins PN,
et al.
Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis,
Tours,
France,
18-22 April 2004.
Am J Hematol.
2005 Aug;79(4):319-28.
Syed IS,
Glockner...