Transthyretin Cardiac Amyloidoses in Older North Americans

Transthyretin cardiac amyloidoses in older North Americans.

The amyloidoses are a group of hereditary or acquired disorders caused by the extracellular deposition of insoluble protein fibrils that impair tissue structure and function. All amyloidoses result from protein misfolding, a common mechanism for disorders in older persons, including Alzheimer's disease and Parkinson's disease. Abnormalities in the protein transthyretin (TTR), a serum transporte...

Transthyretin Cardiac Amyloidosis in Black Americans.

Transthyretin-related cardiac amyloidosis is a progressive infiltrative cardiomyopathy that mimics hypertensive and hypertrophic heart disease and often goes undiagnosed. In the United States, the hereditary form disproportionately afflicts black Americans, who when compared with whites with wild-type transthyretin amyloidosis, a phenotypically similar condition, present with more advanced dise...

Transthyretin-Related Amyloidoses: A Structural and Thermodynamic Approach

(99m)Tc-pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses.

BACKGROUND Differentiating immunoglobulin light-chain (AL) from transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, therapy, and genetic counseling. We validated the discriminatory ability of (99m)Tc-pyrophosphate ((99m)Tc-PYP) scintigraphy in AL versus ATTR. METHODS AND RESULTS Forty-five subjects (12 AL, 16 ATTR wild type, and 17 ATTR mutants) un...

Tc-Pyrophosphate Scintigraphy for Differentiating Light-Chain Cardiac Amyloidosis from the Transthyretin-related Familial and Senile Cardiac Amyloidoses

Background—Differentiating immunoglobulin light-chain (AL) from transthyretin-related cardiac amyloidoses (ATTR) is imperative given implications for prognosis, therapy, and genetic counseling. We validated the discriminatory ability of Tc-pyrophosphate scintigraphy (TcPYP) in AL vs. TTR-related cardiac amyloidoses. Methods and Results—45 subjects (12 AL, 16 ATTR wild-type, and 17 ATTR mutants)...