Quantitative imaging in cardiac transthyretin amyloidosis: rationale and design of the i-CARE study

Transthyretin (ATTR) amyloidosis is a progressive fatal disease caused by the deposition of transthyretin derived amyloid fibrils in various organs [1]. The disease can be classified into two forms: wild-type ATTR amyloidosis in the absence of predisposing transthyretin gene variants which predominantly affects the elderly, and hereditary ATTR amyloidosis caused by mutations in the transthyretin gene. In both forms, amyloid fibrils accumulate in the myocardial extracellular space, resulting in an infiltrative ATTR cardiomyopathy (ATTR-CM)