Cardiac phenotype in hereditary transthyretin amyloidosis: correlations between fibril types and 99mTc-DPD uptake.

Abstract excerpt

Variant transthyretin amyloidosis is a systemic disease. In Sweden, the Val30Met variant is the most prevalent. Val30Met presents in two phenotypes: an early-onset form dominated by polyneuropathy and a late-onset form frequently accompanied by cardiomyopathy. These phenotypes are associated with two amyloid fibril types. Type A fibrils, contain both fragmented and full-length transthyretin, whereas type B...

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• Aged
• Amyloid
• Amyloid Neuropathies, Familial
• Cardiomyopathies
• Diphosphonates