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Abstract
Cardiomyopathy is a major cause of death in patients with systemic amyloidosis. There are several forms of systemic amyloidosis which cause cardiomyopathy and determination of the exact type of amyloid in each affected patient is essential for treatment and determination of prognosis. In this study, we tested the feasibility of determining the type of amyloidosis by biochemical analysis of endomyocardial biopsies.
Right ventricular endomyocardial biopsies were obtained from 10 patients with restrictive cardiomyopathy. Three patients had monoclonal protein demonstrated in serum or urine and all three had bone marrow findings consistent with monoclonal gammopathy. Seven patients had isolated cardiomyopathy without evidence of monoclonal gammopathy. A portion of each myocardial biopsy was submitted for histologic evaluation and all demonstrated amyloid by Congo red staining. Each biopsy was analysed biochemically by isolation of amyloid fibrils and the protein characterised by amino acid sequence analysis. Four amyloid isolates were characterised as immunoglobulin light chain proteins. Two specimens obtained from patients with transthyretin (TTR) DNA mutations contained TTR peptides proving the hereditary nature of the disease. Biopsies from four patients without a TTR mutation contained TTR and were consistent with the diagnosis of senile cardiac amyloidosis (SCA).
All endomyocardial biopsy specimens that were analysed had sufficient amyloid fibril subunit protein to allow characterisation by amino acid sequence analysis. This methodology is particularly useful in differentiating SCA with TTR amyloid fibrils from immunoglobulin light chain amyloidosis which also occurs in the elderly age group.
| Abbreviations | ||
| AL | = | amyloid light chain |
| ApoAI | = | apolipoprotein AI |
| ApoAII | = | apolipoprotein AII |
| CHF | = | congestive heart failure |
| IAPP | = | Islet associated poly peptide |
| NYHA | = | New York Heart Association |
| PCR | = | polymerase chain reaction |
| SCA | = | senile cardiac amyloidosis |
| TTR | = | transthyretin |
| Abbreviations | ||
| AL | = | amyloid light chain |
| ApoAI | = | apolipoprotein AI |
| ApoAII | = | apolipoprotein AII |
| CHF | = | congestive heart failure |
| IAPP | = | Islet associated poly peptide |
| NYHA | = | New York Heart Association |
| PCR | = | polymerase chain reaction |
| SCA | = | senile cardiac amyloidosis |
| TTR | = | transthyretin |