Cardiac amyloidosis: can advances in diagnostics and treatments improve survival?

2009 
Amyloidosis is a generalised clinical disorder characterised by extracellular deposition of amyloid fibrils. Symptomatic cardiac amyloidosis is typically associated with light chain (AL) amyloidosis in the western world. Amyloid deposition in the myocardium causes restrictive cardiomyopathy. Cardiac manifestations affect patient’s performance status and limit therapeutic options. We present a case of rapidly progressing cardiac amyloid deposition due to AL amyloidosis presenting with typical features of restrictive cardiomyopathy. We also discuss the advances in non-invasive diagnostic techniques applied in this case and currently available novel treatments. Early diagnosis and better response to novel treatments could potentially improve the overall survival in this group of patients.
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