Cardiac Amyloidosis: Role of the Endomyocardial Biopsy.

Abstract

Amyloidosis is characterized by protein misfolding and extracellular deposition of insoluble beta-sheet fibrils. It represents a heterogeneous disease with different aetiologies and clinical manifestations. Cardiac involvement (ie, cardiac amyloidosis) has an adverse prognosis, early diagnosis and amyloid typing being crucial for treatment. Recently, the non-invasive diagnosis of CA has increased using imaging techniques, particularly in TTR forms with no monoclonal protein. In the other settings and with inconclusive peripheral biopsy, the EMB allows the demonstration of amyloid by Congo red staining under polarized light, whereas amyloid typing is performed by using immunohistochemistry, immune-electron microscopy and/or proteomics.