Cardiac amyloidosis: Current and pipeline of therapies for the nuclear imager.

Cardiac amyloidosis (CA) is characterized by cardiac amyloid fibril deposition leading to organ dysfunction. The two most common forms are transthyretin (TTR) amyloid cardiomyopathy (ATTR-CM) and immunoglobulin light chain cardiomyopathy (AL-CM). Treatment of CA includes disease-modifying therapies and treating symptoms and cardiac co-morbidities. Patients with heart failure and CA are managed with diuretics for volume optimization, but other guideline-directed medical therapies should be used on a case-by-case basis. Current disease-modifying therapies for ATTR-CM include TTR tetramer stabilizers and gene silencers. Investigational therapies include Clustered Regularly Interspaced Short Palindromic Repeats and associated Cas9 endonuclease (CRISPR-Cas9) systems and amyloid depleters. Current disease-modifying therapies for AL-CM include anti-plasma cell dyscrasia therapies. Investigational therapies include stabilizers and depleters. Nuclear scintigraphy is used to differentiate CA sub-type with no established role for nuclear imaging in therapeutic monitoring to date. However, given nuclear imaging and radiotracer advances, further research is being conducted regarding a nuclear imaging-based approach to assess amyloid burden and organ response to therapy.